A respiratory distress syndrome in newborn infants, usually premature infants with insufficient PULMONARY SURFACTANTS. The disease is characterized by the formation of a HYALINE-like membrane lining the terminal respiratory airspaces (PULMONARY ALVEOLI) and subsequent collapse of the lung (PULMONARY ATELECTASIS).
A darkly stained mat-like EXTRACELLULAR MATRIX (ECM) that separates cell layers, such as EPITHELIUM from ENDOTHELIUM or a layer of CONNECTIVE TISSUE. The ECM layer that supports an overlying EPITHELIUM or ENDOTHELIUM is called basal lamina. Basement membrane (BM) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. BM, composed mainly of TYPE IV COLLAGEN; glycoprotein LAMININ; and PROTEOGLYCAN, provides barriers as well as channels between interacting cell layers.
An autoimmune disease of the KIDNEY and the LUNG. It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. Clinical features include pulmonary alveolar hemorrhage and glomerulonephritis.
The layer of EXTRACELLULAR MATRIX that lies between the ENDOTHELIUM of the glomerular capillaries and the PODOCYTES of the inner or visceral layer of the BOWMAN CAPSULE. It is the product of these two cell types. It acts as a physical barrier and an ion-selective filter.
A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.
A group of inherited conditions characterized initially by HEMATURIA and slowly progressing to RENAL INSUFFICIENCY. The most common form is the Alport syndrome (hereditary nephritis with HEARING LOSS) which is caused by mutations in genes for TYPE IV COLLAGEN and defective GLOMERULAR BASEMENT MEMBRANE.
Presence of blood in the urine.
A non-fibrillar collagen found in the structure of BASEMENT MEMBRANE. Collagen type IV molecules assemble to form a sheet-like network which is involved in maintaining the structural integrity of basement membranes. The predominant form of the protein is comprised of two alpha1(IV) subunits and one alpha2(IV) subunit, however, at least six different alpha subunits can be incorporated into the heterotrimer.
Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.
Inflammation of the interstitial tissue of the kidney. This term is generally used for primary inflammation of KIDNEY TUBULES and/or surrounding interstitium. For primary inflammation of glomerular interstitium, see GLOMERULONEPHRITIS. Infiltration of the inflammatory cells into the interstitial compartment results in EDEMA, increased spaces between the tubules, and tubular renal dysfunction.
Endogenous tissue constituents that have the ability to interact with AUTOANTIBODIES and cause an immune response.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
The presence of proteins in the urine, an indicator of KIDNEY DISEASES.
Highly differentiated epithelial cells of the visceral layer of BOWMAN CAPSULE of the KIDNEY. They are composed of a cell body with major CELL SURFACE EXTENSIONS and secondary fingerlike extensions called pedicels. They enwrap the KIDNEY GLOMERULUS capillaries with their cell surface extensions forming a filtration structure. The pedicels of neighboring podocytes interdigitate with each other leaving between them filtration slits that are bridged by an extracellular structure impermeable to large macromolecules called the slit diaphragm, and provide the last barrier to protein loss in the KIDNEY.
Large, noncollagenous glycoprotein with antigenic properties. It is localized in the basement membrane lamina lucida and functions to bind epithelial cells to the basement membrane. Evidence suggests that the protein plays a role in tumor invasion.
Inflammation of any part of the KIDNEY.
A vasodilator that apparently has direct actions on blood vessels and also increases cardiac output. Tolazoline can interact to some degree with histamine, adrenergic, and cholinergic receptors, but the mechanisms of its therapeutic effects are not clear. It is used in treatment of persistent pulmonary hypertension of the newborn.
Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.
Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.
A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).
A heteropolysaccharide that is similar in structure to HEPARIN. It accumulates in individuals with MUCOPOLYSACCHARIDOSIS.
Ubiquitous macromolecules associated with the cell surface and extracellular matrix of a wide range of cells of vertebrate and invertebrate tissues. They are essential cofactors in cell-matrix adhesion processes, in cell-cell recognition systems, and in receptor-growth factor interactions. (From Cancer Metastasis Rev 1996; 15(2): 177-86; Hepatology 1996; 24(3): 524-32)
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
A type of glomerulonephritis that is characterized by the accumulation of immune deposits (COMPLEMENT MEMBRANE ATTACK COMPLEX) on the outer aspect of the GLOMERULAR BASEMENT MEMBRANE. It progresses from subepithelial dense deposits, to basement membrane reaction and eventual thickening of the basement membrane.
A specialized barrier in the kidney, consisting of the fenestrated CAPILLARY ENDOTHELIUM; GLOMERULAR BASEMENT MEMBRANE; and glomerular epithelium (PODOCYTES). The barrier prevents the filtration of PLASMA PROTEINS.
A condition characterized by severe PROTEINURIA, greater than 3.5 g/day in an average adult. The substantial loss of protein in the urine results in complications such as HYPOPROTEINEMIA; generalized EDEMA; HYPERTENSION; and HYPERLIPIDEMIAS. Diseases associated with nephrotic syndrome generally cause chronic kidney dysfunction.
Pathological processes of the KIDNEY without inflammatory or neoplastic components. Nephrosis may be a primary disorder or secondary complication of other diseases. It is characterized by the NEPHROTIC SYNDROME indicating the presence of PROTEINURIA and HYPOALBUMINEMIA with accompanying EDEMA.
An infant during the first month after birth.
Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.
KIDNEY injuries associated with diabetes mellitus and affecting KIDNEY GLOMERULUS; ARTERIOLES; KIDNEY TUBULES; and the interstitium. Clinical signs include persistent PROTEINURIA, from microalbuminuria progressing to ALBUMINURIA of greater than 300 mg/24 h, leading to reduced GLOMERULAR FILTRATION RATE and END-STAGE RENAL DISEASE.
Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.
The complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES.
The thin membranous structure supporting the adjoining glomerular capillaries. It is composed of GLOMERULAR MESANGIAL CELLS and their EXTRACELLULAR MATRIX.
A human infant born before 37 weeks of GESTATION.
Functional competence of specific organs or body systems of the FETUS in utero.
The minute vessels that connect the arterioles and venules.
A kidney disease with no or minimal histological glomerular changes on light microscopy and with no immune deposits. It is characterized by lipid accumulation in the epithelial cells of KIDNEY TUBULES and in the URINE. Patients usually show NEPHROTIC SYNDROME indicating the presence of PROTEINURIA with accompanying EDEMA.
Negatively charged atoms, radicals or groups of atoms which travel to the anode or positive pole during electrolysis.
The technique of washing tissue specimens with a concentrated solution of a heavy metal salt and letting it dry. The specimen will be covered with a very thin layer of the metal salt, being excluded in areas where an adsorbed macromolecule is present. The macromolecules allow electrons from the beam of an electron microscope to pass much more readily than the heavy metal; thus, a reversed or negative image of the molecule is created.
The presence of albumin in the urine, an indicator of KIDNEY DISEASES.
Thin layers of tissue which cover parts of the body, separate adjacent cavities, or connect adjacent structures.
Heteropolysaccharides which contain an N-acetylated hexosamine in a characteristic repeating disaccharide unit. The repeating structure of each disaccharide involves alternate 1,4- and 1,3-linkages consisting of either N-acetylglucosamine or N-acetylgalactosamine.
PUROMYCIN derivative that lacks the methoxyphenylalanyl group on the amine of the sugar ring. It is an antibiotic with antineoplastic properties and can cause nephrosis.
A clinicopathological syndrome or diagnostic term for a type of glomerular injury that has multiple causes, primary or secondary. Clinical features include PROTEINURIA, reduced GLOMERULAR FILTRATION RATE, and EDEMA. Kidney biopsy initially indicates focal segmental glomerular consolidation (hyalinosis) or scarring which can progress to globally sclerotic glomeruli leading to eventual KIDNEY FAILURE.
Glycoproteins which have a very high polysaccharide content.
The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Proteoglycans consisting of proteins linked to one or more CHONDROITIN SULFATE-containing oligosaccharide chains.
Long convoluted tubules in the nephrons. They collect filtrate from blood passing through the KIDNEY GLOMERULUS and process this filtrate into URINE. Each renal tubule consists of a BOWMAN CAPSULE; PROXIMAL KIDNEY TUBULE; LOOP OF HENLE; DISTAL KIDNEY TUBULE; and KIDNEY COLLECTING DUCT leading to the central cavity of the kidney (KIDNEY PELVIS) that connects to the URETER.
Pathological processes of the KIDNEY or its component tissues.
Lipids, predominantly phospholipids, cholesterol and small amounts of glycolipids found in membranes including cellular and intracellular membranes. These lipids may be arranged in bilayers in the membranes with integral proteins between the layers and peripheral proteins attached to the outside. Membrane lipids are required for active transport, several enzymatic activities and membrane formation.
Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).
Any method of artificial breathing that employs mechanical or non-mechanical means to force the air into and out of the lungs. Artificial respiration or ventilation is used in individuals who have stopped breathing or have RESPIRATORY INSUFFICIENCY to increase their intake of oxygen (O2) and excretion of carbon dioxide (CO2).
Microscopy in which the samples are first stained immunocytochemically and then examined using an electron microscope. Immunoelectron microscopy is used extensively in diagnostic virology as part of very sensitive immunoassays.
A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere.
Substances and drugs that lower the SURFACE TENSION of the mucoid layer lining the PULMONARY ALVEOLI.
A metalloproteinase which degrades helical regions of native collagen to small fragments. Preferred cleavage is -Gly in the sequence -Pro-Xaa-Gly-Pro-. Six forms (or 2 classes) have been isolated from Clostridium histolyticum that are immunologically cross-reactive but possess different sequences and different specificities. Other variants have been isolated from Bacillus cereus, Empedobacter collagenolyticum, Pseudomonas marinoglutinosa, and species of Vibrio and Streptomyces. EC 3.4.24.3.
A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.
A syndrome of multiple abnormalities characterized by the absence or hypoplasia of the PATELLA and congenital nail dystrophy. It is a genetically determined autosomal dominant trait.
Thin structures that encapsulate subcellular structures or ORGANELLES in EUKARYOTIC CELLS. They include a variety of membranes associated with the CELL NUCLEUS; the MITOCHONDRIA; the GOLGI APPARATUS; the ENDOPLASMIC RETICULUM; LYSOSOMES; PLASTIDS; and VACUOLES.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
One or more layers of EPITHELIAL CELLS, supported by the basal lamina, which covers the inner or outer surfaces of the body.
A sucrose polymer of high molecular weight.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
The voltage differences across a membrane. For cellular membranes they are computed by subtracting the voltage measured outside the membrane from the voltage measured inside the membrane. They result from differences of inside versus outside concentration of potassium, sodium, chloride, and other ions across cells' or ORGANELLES membranes. For excitable cells, the resting membrane potentials range between -30 and -100 millivolts. Physical, chemical, or electrical stimuli can make a membrane potential more negative (hyperpolarization), or less negative (depolarization).
Property of membranes and other structures to permit passage of light, heat, gases, liquids, metabolites, and mineral ions.
Artificially produced membranes, such as semipermeable membranes used in artificial kidney dialysis (RENAL DIALYSIS), monomolecular and bimolecular membranes used as models to simulate biological CELL MEMBRANES. These membranes are also used in the process of GUIDED TISSUE REGENERATION.
The age of the conceptus, beginning from the time of FERTILIZATION. In clinical obstetrics, the gestational age is often estimated as the time from the last day of the last MENSTRUATION which is about 2 weeks before OVULATION and fertilization.
Immune complex disease caused by the administration of foreign serum or serum proteins and characterized by fever, lymphadenopathy, arthralgia, and urticaria. When they are complexed to protein carriers, some drugs can also cause serum sickness when they act as haptens inducing antibody responses.
Conditions which affect the structure or function of the pupil of the eye, including disorders of innervation to the pupillary constrictor or dilator muscles, and disorders of pupillary reflexes.
A layer of epithelium that lines the heart, blood vessels (ENDOTHELIUM, VASCULAR), lymph vessels (ENDOTHELIUM, LYMPHATIC), and the serous cavities of the body.
Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.
Substances that are recognized by the immune system and induce an immune reaction.
Heparin derivatives. The term has also been used more loosely to include naturally occurring and synthetic highly-sulphated polysaccharides of similar structure. Heparinoid preparations have been used for a wide range of applications including as anticoagulants and anti-inflammatories and they have been claimed to have hypolipidemic properties. (From Martindale, The Extra Pharmacopoeia, 30th, p232)
Electron microscopy in which the ELECTRONS or their reaction products that pass down through the specimen are imaged below the plane of the specimen.
Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.
Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.
A protein component of the synaptic basal lamina. It has been shown to induce clustering of acetylcholine receptors on the surface of muscle fibers and other synaptic molecules in both synapse regeneration and development.
A filarial parasite primarily of dogs but occurring also in foxes, wolves, and humans. The parasite is transmitted by mosquitoes.
Glycoproteins found on the membrane or surface of cells.
A condition of the newborn marked by DYSPNEA with CYANOSIS, heralded by such prodromal signs as dilatation of the alae nasi, expiratory grunt, and retraction of the suprasternal notch or costal margins, mostly frequently occurring in premature infants, children of diabetic mothers, and infants delivered by cesarean section, and sometimes with no apparent predisposing cause.
An infant having a birth weight of 2500 gm. (5.5 lb.) or less but INFANT, VERY LOW BIRTH WEIGHT is available for infants having a birth weight of 1500 grams (3.3 lb.) or less.
Glycoproteins found on the surfaces of cells, particularly in fibrillar structures. The proteins are lost or reduced when these cells undergo viral or chemical transformation. They are highly susceptible to proteolysis and are substrates for activated blood coagulation factor VIII. The forms present in plasma are called cold-insoluble globulins.
The infiltrating of histological specimens with plastics, including acrylic resins, epoxy resins and polyethylene glycol, for support of the tissues in preparation for sectioning with a microtome.
The volume of water filtered out of plasma through glomerular capillary walls into Bowman's capsules per unit of time. It is considered to be equivalent to INULIN clearance.
Infection with nematodes of the genus DIROFILARIA, usually in animals, especially dogs, but occasionally in man.
The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.
Strongly cationic polymer that binds to certain proteins; used as a marker in immunology, to precipitate and purify enzymes and lipids. Synonyms: aziridine polymer; Epamine; Epomine; ethylenimine polymer; Montrek; PEI; Polymin(e).
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
Serum albumin from cows, commonly used in in vitro biological studies. (From Stedman, 25th ed)
Serum that contains antibodies. It is obtained from an animal that has been immunized either by ANTIGEN injection or infection with microorganisms containing the antigen.
The outer zone of the KIDNEY, beneath the capsule, consisting of KIDNEY GLOMERULUS; KIDNEY TUBULES, DISTAL; and KIDNEY TUBULES, PROXIMAL.
Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.
The processes triggered by interactions of ANTIBODIES with their ANTIGENS.
The semi-permeable outer structure of a red blood cell. It is known as a red cell 'ghost' after HEMOLYSIS.
The motion of phospholipid molecules within the lipid bilayer, dependent on the classes of phospholipids present, their fatty acid composition and degree of unsaturation of the acyl chains, the cholesterol concentration, and temperature.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.
The sum of the weight of all the atoms in a molecule.
Antibodies produced by a single clone of cells.
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
Diabetes mellitus induced experimentally by administration of various diabetogenic agents or by PANCREATECTOMY.
Microscopy of specimens stained with fluorescent dye (usually fluorescein isothiocyanate) or of naturally fluorescent materials, which emit light when exposed to ultraviolet or blue light. Immunofluorescence microscopy utilizes antibodies that are labeled with fluorescent dye.
A form of fluorescent antibody technique commonly used to detect serum antibodies and immune complexes in tissues and microorganisms in specimens from patients with infectious diseases. The technique involves formation of an antigen-antibody complex which is labeled with fluorescein-conjugated anti-immunoglobulin antibody. (From Bennington, Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984)
Adherence of cells to surfaces or to other cells.
A chronic form of glomerulonephritis characterized by deposits of predominantly IMMUNOGLOBULIN A in the mesangial area (GLOMERULAR MESANGIUM). Deposits of COMPLEMENT C3 and IMMUNOGLOBULIN G are also often found. Clinical features may progress from asymptomatic HEMATURIA to END-STAGE KIDNEY DISEASE.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
A quality of cell membranes which permits the passage of solvents and solutes into and out of cells.
The marking of biological material with a dye or other reagent for the purpose of identifying and quantitating components of tissues, cells or their extracts.
Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.
An infant whose weight at birth is less than 1500 grams (3.3 lbs), regardless of gestational age.
Bleeding into one or both CEREBRAL HEMISPHERES including the BASAL GANGLIA and the CEREBRAL CORTEX. It is often associated with HYPERTENSION and CRANIOCEREBRAL TRAUMA.
Pathological processes involving any part of the LUNG.
Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.
A pathological process consisting of hardening or fibrosis of an anatomical structure, often a vessel or a nerve.
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
The largest class of organic compounds, including STARCH; GLYCOGEN; CELLULOSE; POLYSACCHARIDES; and simple MONOSACCHARIDES. Carbohydrates are composed of carbon, hydrogen, and oxygen in a ratio of Cn(H2O)n.
The property of antibodies which enables them to react with some ANTIGENIC DETERMINANTS and not with others. Specificity is dependent on chemical composition, physical forces, and molecular structure at the binding site.
Elements of limited time intervals, contributing to particular results or situations.
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment.
A group of carbon-oxygen lyases. These enzymes catalyze the breakage of a carbon-oxygen bond in polysaccharides leading to an unsaturated product and the elimination of an alcohol. EC 4.2.2.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.

Goodpasture antigen: expression of the full-length alpha3(IV) chain of collagen IV and localization of epitopes exclusively to the noncollagenous domain. (1/267)

BACKGROUND: Tissue injury in Goodpasture (GP) syndrome (rapidly progressive glomerular nephritis and pulmonary hemorrhage) is mediated by antibasement membrane antibodies that are targeted to the alpha3(IV) chain of type IV collagen, one of five alpha(IV) chains that occur in the glomerular basement membrane. GP antibodies are known to bind epitopes within the carboxyl terminal noncollagenous domain (NC1) of the alpha3(IV) chain, termed the GP autoantigen. Whether epitopes also exist in the 1400-residue collagenous domain is unknown because studies to date have focused solely on the NC1 domain. A knowledge of GP epitopes is important for the understanding of the etiology and pathogenesis of the disease and for the development of therapeutic strategies. METHODS: A cDNA construct was prepared for the full-length human alpha3(IV) chain. The construct was stably transfected into human embryonic kidney 293 cells. The purified full-length r-alpha3(IV) chain was characterized by electrophoresis and electron microscopy. The capacity of this chain for binding of GP antibodies from five patients was compared with that of the human r-alpha3(IV)NC1 domain by competitive enzyme-linked immunosorbent assay. RESULTS: The r-alpha3(IV) chain was secreted from 293 cells as a single polypeptide chain that did not spontaneously undergo assembly into a triple-helical molecule. An analysis of GP-antibody binding to the full-length r-alpha3(IV) chain showed binding exclusively to the globular NC1 domain. CONCLUSION: The full-length human alpha3(IV) chain possesses the capacity to bind GP autoantibodies. The epitope(s) is found exclusively on the nontriple-helical NC1 domain of the alpha3(IV) chain, indicating the presence of specific immunogenic properties. The alpha3(IV) chain alone does not spontaneously undergo assembly into a triple-helical homotrimeric molecule, suggesting that coassembly with either the alpha4(IV) and/or the alpha5(IV) chain may be required for triple-helix formation.  (+info)

Identification of a clinically relevant immunodominant region of collagen IV in Goodpasture disease. (2/267)

BACKGROUND: The characteristic feature of Goodpasture disease is the occurrence of an autoantibody response to the noncollagenous domain of the alpha3 chain of type IV collagen [alpha3(IV)NC1] in the alveolar and glomerular basement membrane. These antibodies are associated with the development of a rapidly progressive glomerulonephritis, with or without lung hemorrhage, whereas autoantibodies specific for the other alpha chains of the heterotrimeric type IV collagen probably do not cause disease. In this study, we have investigated whether differences in fine specificity of autoimmune recognition of the alpha3(IV)NC1 correlate with clinical outcome. METHODS: For mapping of antibody binding to type IV collagen, chimeric collagen constructs were generated in which parts of the alpha3(IV)NC1 domain were replaced by the corresponding sequences of homologous nonreactive alpha1(IV). The different recombinant collagen chimeras allowed the analysis of antibody specificities in 77 sera from well-documented patients. RESULTS: One construct that harbors the aminoterminal third of the alpha3(IV)NC1 was recognized by all sera, indicating that it represents the dominant target of the B-cell response in Goodpasture disease. Seventy percent of the samples recognized other parts of the molecule as well. However, only reactivity to the N-terminus of the alpha3(IV)NC1 correlated with prognosis, that is, kidney survival after six months of follow-up. CONCLUSION: The results indicate the crucial importance of antibody recognition of this particular domain for the pathogenesis of Goodpasture disease, thereby opening new avenues for the development of better diagnostic and therapeutic procedures.  (+info)

Angiotensin II plays a pathogenic role in immune-mediated renal injury in mice. (3/267)

Several lines of evidence show the importance of angiotensin II (AII) in renal injuries, especially when hemodynamic abnormalities are involved. To elucidate the role of AII in immune-mediated renal injury, we studied anti-glomerular basement membrane (GBM) nephritis in AII type 1a receptor (AT1a)-deficient homozygous (AT1a-/-) and wild-type (AT1a+/+) mice. A transient activation of the renin-angiotensin system (RAS) was observed in both groups of mice at around day 1. A renal expression of monocyte chemoattractant protein-1 (MCP-1) was transiently induced at six hours in both groups, which was then downregulated at day 1. In the AT1a+/+ mice, after RAS activation, the glomerular expression of MCP-1 was exacerbated at days 7 and 14. Thereafter, severe proteinuria developed, and the renal expressions of transforming growth factor-beta1 (TGF-beta1) and collagen type I increased, resulting in severe glomerulosclerosis and interstitial fibrosis. In contrast, glomerular expression of MCP-1, proteinuria, and tissue damage were markedly ameliorated in the AT1a-/- mice. Because this amelioration is likely due to the lack of AT1a, we can conclude that AII action, mediated by AT1a, plays a pathogenic role in anti-GBM nephritis, in which AII may contribute to the exacerbation of glomerular MCP-1 expression. These results suggest the involvement of AII in immune-mediated renal injuries.  (+info)

P-selectin deficiency exacerbates experimental glomerulonephritis: a protective role for endothelial P-selectin in inflammation. (4/267)

P-selectin is a leukocyte adhesion receptor present in endothelial cells and platelets. We examined the role of P-selectin in the autologous phase of an accelerated model of anti-glomerular basement membrane (GBM) glomerulonephritis using P-selectin-deficient mice and chimeric mice expressing P-selectin only in platelets or endothelial cells. P-selectin-deficient mice exhibited more severe glomerular damage with increased interstitial mononuclear leukocytic infiltrates, and had significantly increased proteinuria and mortality when compared to wild-type mice. P-selectin on the endothelium was predominantly responsible for protection from the exacerbated disease, because chimeric mice with endothelial P-selectin, and not mice with platelet P-selectin, showed glomerular injury similar to that in wild-type animals. Levels of soluble circulating P-selectin were increased in nephritic wild-type mice and in chimeric mice with endothelial P-selectin, but not platelet P-selectin. Levels of soluble P-selectin, which has been shown to be anti-inflammatory in vitro, were inversely associated with the severity of disease. P-selectin was not expressed in the endothelium of the glomerulus or interstitium. Thus, the protective effect in wild-type mice may be accounted for, in part by soluble P-selectin shed by non-renal endothelial cells, although other endothelial P-selectin-dependent mechanisms cannot be ruled out.  (+info)

IL-1 up-regulates osteopontin expression in experimental crescentic glomerulonephritis in the rat. (5/267)

Osteopontin (OPN) is a macrophage chemotactic and adhesion molecule that acts to promote macrophage infiltration in rat anti-glomerular basement membrane (GBM) glomerulonephritis. The present study investigated the role of interleukin-1 (IL-1) in the up-regulation of renal OPN expression in this disease model. Accelerated anti-GBM glomerulonephritis was induced in groups of six rats. Animals were treated by a constant infusion of the IL-1 receptor antagonist or saline (control) over days -1 to 14 (induction phase) or days 7 to 21 (established disease). In normal rat kidney, OPN was expressed in a few tubules (<5%) and absent from glomeruli. During the development of rat anti-GBM disease (days 7 to 21), there was substantial up-regulation of OPN mRNA and protein expression in glomeruli (>5 cells per glomerular cross-section) and tubular epithelial cells (50-75% OPN-positive). Up-regulation of OPN expression was associated with macrophage accumulation within the kidney, severe proteinuria, loss of renal function, and severe histological damage including glomerular crescentic formation and tubulointerstitial fibrosis. In contrast, IL-1 receptor antagonist treatment of either the induction phase of disease or established disease significantly reduced OPN mRNA and protein expression in glomeruli (/75-85%, P < 0.001) and tubules (/45-60%, P < 0.001). The reduction in OPN expression was associated with significant inhibition of macrophage accumulation and progressive renal injury. In vitro, the addition of IL-1 to the normal rat tubular epithelial cell line NRK52E up-regulated OPN mRNA and protein levels, an effect that was dose-dependent and inhibited by the addition of IL-1 receptor antagonist, thus demonstrating that IL-1 can act directly to up-regulate renal OPN expression. In conclusion, this study provides in vivo and in vitro evidence that IL-1 up-regulates OPN expression in experimental kidney disease and support for the argument that inhibition of OPN expression is one mechanism by which IL-1 receptor antagonist treatment suppresses macrophage-mediated renal injury.  (+info)

Development of scarring and renal failure in a rat model of crescentic glomerulonephritis. (6/267)

BACKGROUND: The aim of this study was to develop and characterize a rat model of crescentic glomerulonephritis which progresses to glomerulosclerosis and renal failure. METHODS: Glomerulonephritis was induced in Wistar Kyoto rats by a single injection of rabbit anti-glomerular basement membrane antiserum. Albuminuria and serum creatinine were monitored. Kidneys were examined, from 2.5 h to 44 days, using light-microscopy and immunohistochemistry. To characterize the glomerular inflammatory infiltrate, glomeruli were digested to single cells and analysed by fluorescence-activated cell sorter (FACS) and by immunohistochemistry on cytospins. RESULTS: Rats developed albuminuria by 4 days and increased serum creatinine by day 18. Histology showed glomerular fibrinoid necrosis by day 4 and cellular crescents in a mean of 63% of glomeruli by day 11. By 6 weeks, rats had developed renal failure (mean creatinine >300 micromol/l) with 94% of the glomeruli showing glomerulosclerosis. The kidneys were also affected by severe interstitial nephritis and tubular loss. The glomeruli were infiltrated by monocytes/ macrophages (ED1+) and CD8+ (OX8+) cells. FACS analysis showed that CD8+ cells did not express T-cell markers (CD3, TCRalphabeta or TCRgammadelta) or the NK-cell marker (NKR-P1). FACS analysis of peripheral blood mononuclear cells demonstrated a population of monocytes reactive with OX8, and double-labelling of cytospin preparations of glomerular digests showed that a proportion of the CD8+ cells were a subset of ED1+ monocyte/macrophages. CONCLUSIONS: We have characterized a reproducible model of crescentic glomerulonephritis which rapidly progresses to chronic renal failure with glomerulosclerosis and tubulo-interstitial scarring. This model will be useful for testing new therapeutic approaches in crescentic glomerulonephritis.  (+info)

Increased susceptibility to immunologically mediated glomerulonephritis in IFN-gamma-deficient mice. (7/267)

It is postulated that IFN-gamma confers susceptibility to immunologically mediated tissue injury. To test this hypothesis, we compared the intensity of accelerated anti-glomerular basement membrane glomerulonephritis between wild-type (IFN-gamma+/+) and IFN-gamma gene knockout (IFN-gamma-/-) mice. This disease model is initiated by binding of heterologous (sheep) anti-glomerular basement membrane Abs to the glomeruli of mice preimmunized with sheep IgG. The secondary cellular and humoral immune responses to the planted Ag then lead to albuminuria and glomerular pathology. We found that IFN-gamma-/- mice or IFN-gamma+/+ mice injected with IFN-gamma-neutralizing Ab develop worse albuminuria and glomerular pathology than IFN-gamma+/+ mice. The humoral response to sheep IgG (serum mouse anti-sheep IgG titers and intraglomerular mouse IgG deposits) was comparable in the IFN-gamma+/+ and IFN-gamma-/- groups. In contrast, IFN-gamma-/- mice mounted a stronger cellular immune response (cutaneous delayed-type hypersensitivity reaction) to sheep IgG than IFN-gamma+/+ mice. These findings provide evidence that endogenous IFN-gamma has a protective role in immunologically mediated glomerulonephritis initiated by foreign Ags.  (+info)

Goodpasture disease. Characterization of a single conformational epitope as the target of pathogenic autoantibodies. (8/267)

Goodpasture disease is a prototype autoimmune disease characterized by the formation of autoantibodies against the heterotrimeric basement membrane collagen type IV, which causes a rapidly progressive glomerulonephritis. The pathogenic antibody response is directed to the non-collagenous (NC1) domain of the alpha3 chain of type IV collagen (alpha3(IV)NC1), but not to the homologous region of the alpha1(IV)NC1. To identify the conformation-dependent immunodominant epitope on the alpha3(IV)NC1, a variety of recombinant NC1 domains were constructed by replacing single residues of alpha3(IV) with the corresponding amino acids from the nonreactive alpha1(IV) chain. Replacement mutations were identified that completely destroyed the Goodpasture epitope in the alpha3(IV) chain. Based on the identification of these critical positions, the epitope was finally reconstructed within the frame of the alpha1(IV) chain. The substitution of nine discontinuous positions in the alpha1(IV)NC1 with amino acid residues from the alpha3 chain resulted in a recombinant construct that was recognized by all patients' sera (n = 20) but by none of the sera from healthy controls (n = 10). This provides, for the first time, the molecular characterization of a single immunodominant conformational epitope recognized by pathogenic autoantibodies in a human autoimmune disease, representing the basis for the development of new epitope-specific strategies in the treatment of Goodpasture disease.  (+info)

Anti-glomerular basement membrane disease is a rare autoimmune disorder characterized by pulmonary hemorrhage, crescentic glomerulonephritis and the presence of circulating anti-glomerular basement membrane antibodies. The simultaneous occurrence of both anti-glomerular basement membrane disease and membranous nephropathy is rare. A 59-year-old Hispanic man presented with acute onset of nausea and vomiting and was found to have renal insufficiency. Work-up included a kidney biopsy, which revealed anti-glomerular basement membrane disease with underlying membranous nephropathy. He was treated with emergent hemodialysis, intravenous corticosteroids, plasmapheresis, and cyclophosphamide without improvement in his renal function. Simultaneous anti-glomerular basement membrane disease and membranous nephropathy is very rare. There have been 16 previous case reports in the English language literature that have been associated with a high mortality and morbidity, and a very high rate of renal failure resulting
TY - JOUR. T1 - Pediatric anti-glomerular basement membrane glomerulonephritis. AU - Trivedi, V. A.. AU - Malter, J. S.. AU - Guillery, E.. PY - 1998/8/13. Y1 - 1998/8/13. UR - http://www.scopus.com/inward/record.url?scp=0031856041&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=0031856041&partnerID=8YFLogxK. U2 - 10.1002/(SICI)1098-1101(1998)13:2,69::AID-JCA4,3.0.CO;2-6. DO - 10.1002/(SICI)1098-1101(1998)13:2,69::AID-JCA4,3.0.CO;2-6. M3 - Letter. C2 - 9704608. AN - SCOPUS:0031856041. VL - 13. JO - Journal of Clinical Apheresis. JF - Journal of Clinical Apheresis. SN - 0733-2459. IS - 2. ER - ...
Goodpastures disease is a rare immunological disease with formation of pathognomonic antibodies against renal and pulmonary basement membranes. Cerebral involvement has been reported in several cases in the literature, yet the pathogenetic mechanism is not entirely clear. A 21-year-old Caucasian man with Goodpastures disease and end-stage renal disease presented with two generalized seizures after a period of mild cognitive disturbance. Blood pressure and routine laboratory tests did not exceed the patients usual values, and examination of cerebrospinal fluid was unremarkable. Cerebral magnetic resonance imaging (MRI) revealed multiple cortical and subcortical lesions on fluid-attenuated inversion recovery sequences. Since antiglomerular basement membrane antibodies were found to be positive with high titers, plasmapheresis was started. In addition, cyclophosphamide pulse therapy was given on day 13. Encephalopathy and MRI lesions disappeared during this therapy, and antiglomerular basement membrane
AIM Cases with anti-glomerular basement membrane (GBM) disease have been reported with linear deposit of immunoglobulin G (IgG) along GBM, but have undetectable anti-GBM antibodies in circulation by enzyme linked immunosorbent assays (ELISA). We speculated that the structure of the antigens recognized by these antibodies may contribute to the negative results of ELISA. METHODS Sera from four patients were collected, with typical linear deposit of IgG along GBM but no anti-GBM reactivity by commercial ELISA kits. Circulating anti-GBM antibodies were detected by indirect immunofluorescence. Antigen specificity and its conformational structure was investigated by western-blot analysis, using recombinant human α1-α5(IV)NC1 and chimeric proteins E(A) and E(B) as antigens. RESULTS The presence of circulating anti-GBM antibodies were confirmed by indirect immunofluorescence with linear deposit of IgG towards cryptic epitopes along GBM on normal kidney sections. These antibodies did not recognize
Patients suffering from the Goodpasture syndrome develop autoantibodies that bind to a special type collagen, the so called Goodpasture antigen in the glomerular basement membrane of their kidneys that helps filter waste and extra fluid from the blood. Goodpasture syndrome can lead to rapidly progressive kidney failure along with lung disease.. The detection of these glomerular basement membrane antibodies (anti-GBM) in the sera of patients with suspected Goodpasture syndrome is the conclusive finding for the diagnosis of the disease.. A positive test for myeloperoxidase antibodies (anti-MPO) and a positive pANCA are also consistent with Goodpastures syndrome.. ...
PubMed comprises more than 30 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.
PubMed comprises more than 30 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.
Clinical and pathological data on 71 patients from throughout the British Isles who developed antiglomerular basement membrane antibody mediated nephritis in the period 1980-4 were studied. Two principle patterns of disease were recognised: young men presenting in their 20s with Goodpastures syndrome (glomerulonephritis and lung haemorrhage) and women presenting in their 60s with glomerulonephritis alone. The effect of treatment on prognosis of a total of 108 patients was also reviewed (the 71 patients plus patients seen before 1980 at Hammersmith Hospital). Treatment with prednisolone, cytotoxic drugs, and plasma exchange hastened the time to clearance of autoantibody and improved the outlook of patients who were not dependent on dialysis and those with lung haemorrhage. ...
Goodpasture syndrome (GPS) is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpastures antigen. Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death. It is treated with immunosuppressant drugs such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood. The disease was first described by an American pathologist Ernest Goodpasture of Vanderbilt University in 1919 and was later named in his honor. Play media The antiglomerular basement membrane (GBM) antibodies primarily attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains. 60 to 80% of those with the condition experience ...
Goodpastures syndrome is a pulmonary-renal syndrome, which is a group of acute illnesses involving the kidneys and lungs. Goodpasture syndrome includes all of the following conditions:. Glomerulonephritis - inflammation of the glomeruli, which are tiny clusters of looping blood vessels in the kidneys that help filter wastes and extra water from the blood.. The presence of anti-glomerular basement membrane (GBM) antibodies; the GBM is part of the glomeruli and is composed of collagen and other proteins.. Bleeding in the lungs. ...
Your bodies have immune system and this system produces antibodies which are nothing other than proteins and they help to fight infections. However, when you are infected with Goodpastures Syndrome your body starts making antibodies that attack and damage your body. They mainly target the lining of your lungs and kidney and there is no particular reason why your antibodies begin to attack your own lungs and kidneys.. However there are some factors that are believed to trigger the disease. When you are exposed to viral lung infections, or you smoke or you are breathing in vapors from organic solvents, you stand a chance of being affected by the disease.. Caucasian people (mostly men) between ages 20 and 30 or above 60 suffer from this disease. If you have suffered from lupus erythematosus or Wegeners granulomatosis then your chance of contracting Goodpastures Syndrome is considerably more.. ...
Anti-glomerular basement membrane antibody-induced glomerulonephritis with periglomerular granulomatous reaction and massive renal eosinophilic infiltration.: W
Goodpasture syndrome is a rare autoimmune disease that affects the lungs and kidneys. Normally, the immune system makes antibodies to fight off germs. With Goodpasture syndrome, however, the immune system mistakenly makes antibodies that attack the lungs and kidneys. This condition can quickly progress to glomerulonephritis and kidney failure.
Goodpasture syndrome is a rare autoimmune disease that affects the lungs and kidneys. Normally, the immune system makes antibodies to fight off germs. With Goodpasture syndrome, however, the immune system mistakenly makes antibodies that attack the lungs and kidneys. This condition can quickly progress to glomerulonephritis and kidney failure.
Goodpasture syndrome is a rare autoimmune disease that affects the lungs and kidneys. Normally, the immune system makes antibodies to fight off germs. With Goodpasture syndrome, however, the immune system mistakenly makes antibodies that attack the lungs and kidneys. This condition can quickly progress to glomerulonephritis and kidney failure.
Goodpasture disease is defined by the presence of autoantibodies to the alpha-3 chain of type IV collagen. It presents with rapidly progressive renal dysfunction often combined with lung hemorrhage. Simultaneous hemoptysis and renal failure (the pulmonary renal syndrome) has many causes, but wher...
in Kidney International (1987), 31(1), 32-40. A proliferative glomerulonephritis was induced in rats pre-immunized with rabbit IgG by injecting intravenously a sub-nephrotoxic dose of rabbit anti-glomerular basement membrane (GBM) IgG (A rats). Most ... [more ▼]. A proliferative glomerulonephritis was induced in rats pre-immunized with rabbit IgG by injecting intravenously a sub-nephrotoxic dose of rabbit anti-glomerular basement membrane (GBM) IgG (A rats). Most rats (80%) developed a severe proteinuria (greater than 100 mg/24 hr) within two to five days after the injection of anti-GBM IgG. At the same time, microscopic examination of the kidneys revealed a glomerular infiltration by mononuclear phagocytes and a prominent decrease in the intensity of the colloidal iron reaction in glomeruli. A non-proliferative glomerular disease was induced in another group of rats (B rats) by intraperitoneal administration of aminonucleoside of puromycin. A marked proteinuria (greater than 100 mg/24 hr) ...
Goodpasture syndrome (also known as anti-GBM disease) is characterized by the presence of auto-antibodies to a component of lung and kidney tissue. It is an exceptionally rare disease, which impacts approximately 1 in every million people, though it is more common in white European and Asian populations. Goodpasture syndrome most typically presents as a combinations of lung and kidney problems although it can present less frequently as an isolated issue with either organ system. A patient such as ours, who presents with both coughing and urinating blood (known as hemoptysis and hematuria respectively) should at least prompt the consideration of Goodpasture syndrome in the differential diagnosis. Other symptoms of lung or kidney disease include chest pain with cough, high blood pressure, and swelling of the legs, all of which our patient had on initial presentation. Other nonspecific findings include fever, rash, fatigue, and an enlarged liver and spleen, all of which occur in much more common ...
Anti-GBM antibody disease is one of the three major forms of rapidly progressive (or crescentic) glomerulonephritis. Although some patients present with relatively mild renal insufficiency, this disorder is typically associated with severe renal inju
John W. Stirling and Alan Curry. 1.1 The Role of Transmission Electron Microscopy (TEM) in Renal Diagnostics 1. 1.2 Ultrastructural Evaluation and Interpretation 2. 1.3 The Normal Glomerulus 3. 1.4 Ultrastructural Diagnostic Features 5. 1.5 Granular and Amorphous Deposits 6. 1.6 Organised Immune-Related Fibrils and Tubules 7. 1.7 Nonspecific Fibrils 11. 1.8 General and Nonspecific Inclusions and Deposits 11. 1.9 Fibrin 12. 1.10 Tubuloreticular Bodies (Tubuloreticular Inclusions) 12. 1.11 The Glomerular Basement Membrane 13. 1.12 The Mesangial Matrix 14. 1.13 Cellular Components of the Glomerulus 14. 1.14 Mesangial Cells 16. 1.15 The Capillary Lumen 16. 1.16 The Ultrastructural Pathology of the Major Glomerular Diseases 16. 1.17 Focal Segmental Glomerulosclerosis 17. 1.18 Pauci-Immune Glomerulonephritis (ANCA-Associated Glomerulonephritis) 18. 1.19 Anti-GBM Glomerulonephritis (Goodpasture Syndrome) 19. 1.20 Diseases with Structural GBM Changes 19. 1.21 Thin Basement Membrane Disease 21. 1.22 ...
Goodpastures disease is a fulminant rapidly progressive disease characterized by autoantibodies to the alpha-3 chain of type IV collagen (Goodpastures antigen). It can ..
Anti-glomerular basement membrane (GBM) disease is characterized by autoantibodies directed against the anti-GBM antigen, which is part of the non-collagenous domain of t..
While membranous nephropathy (MN) is one of the most common forms of nephrotic syndrome in adults, it is rarely associated with the pathologic finding of crescents on kidney biopsy. Most reported cases are in the context of concomitant anti-neutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (anti-GBM) antibody disease, or were documented prior to the…
The alpha-3 subunit (COL4A3) of collagen IV is thought to be the antigen implicated in Goodpasture syndrome, wherein the immune system attacks the basement membranes of the glomeruli and the alveoli upon the antigenic site on the alpha-3 subunit becomes unsequestered due to environmental exposures. Goodpasture syndrome presents with nephritic syndrome and hemoptysis. Microscopic evaluation of biopsied renal tissue will reveal linear deposits of Immunoglobulin G by immunofluorescence. This is classically in young adult males. Mutations to the genes coding for collagen IV lead to Alport syndrome. This will cause thinning and splitting of the glomerular basement membrane. It will present as isolated hematuria, sensorineural hearing loss, and ocular disturbances and is passed on genetically, usually in an X-linked manner, although there are rarer autosomal forms. Liver fibrosis and cirrhosis are associated with the deposition of collagen IV in the liver. Serum Collagen IV concentrations correlate ...
Analgesic nephropathy:. Analgesic nephropathy is a chronic kidney disease that gradually leads to end-stage renal disease and the need for permanent dialysis or a kidney transplant.. Diabetic nephropathy:. Nephropathy is the deterioration of the kidneys. Th final stage of nephropathy is called end-stage renal disease, or ESRD.. End Stage Renal Disease (ESRD):. Renal failure refers to temporary or permanent damage to the kidneys that results in loss of normal kidney function.. Glomerulonephritis: Glomerulonephritis is a type of glomerular kidney disease in which the kidneys filters become inflamed and scarred, and slowly lose their ability to remove wastes and excess fluid from the blood to make urine.. Goodpasture syndrome:. Goodpasture syndrome is a rare, autoimmune disease that can affect the lungs and kidneys.. Hematuria (blood in the urine):. Hematuria is the presence of red blood cells (RBCs) in the urine.. Hemolytic uremic syndrome:. Hemolytic uremic syndrome is a rare condition that ...
Learn about the causes, symptoms, diagnosis & treatment of Diffuse Alveolar Hemorrhage and Pulmonary-Renal Syndrome from the Professional Version of the Merck Manuals.
Dr. Bellot responded: Immunosuppression. Since the condition is produced by an abnormal immune response, the treatment is to reduce production of the |a href=/topics/antibody track_data={
View the Goodpasture surname, family crest and coat of arms. Discover the Goodpasture family history for the Dutch Origin. What is the origin of the name Goodpasture?
Schmidt will need to take drugs for the rest of her life to prevent organ rejection. But she no longer needs dialysis, and she left the hospital March 20 in good health to return home.. Eculizumab was used once before at UI Health two years ago to enable a kidney transplant for another patient who had high levels of antibodies. But that patients organ had come from a living donor. Schmidts transplant is the first in the U.S. to use the protocol with a deceased donors organ.. Schmidt had developed extremely high levels of circulating antibodies due to more than 20 blood transfusions she required since developing Goodpastures syndrome 14 years ago. The rare and sometimes lethal autoimmune disorder attacks the kidneys and lungs. In Schmidts case, it destroyed her kidney function, forcing her to undergo dialysis several times a week to clean wastes from her blood.. Blood transfusions had helped to flush harmful immune cells from Schmidts blood. But even though donated blood is matched by type ...
It is because of research that I am still alive. Without the work by researchers and patients collaborating over many decades to find new ways of doing things, I couldnt have been treated for Goodpastures syndrome, been kept alive on dialysis until I could receive a kidney transplant, and spent the last 12 years living my life. But the treatments are not perfect.. There is still more to do. As experts in living with kidney disease we have a huge role to play in helping research to be better, to change policy and to improve the lives of people living with kidney disease. ...
FUNCTION: [Summary is not available for the mouse gene. This summary is for the human ortholog.] Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. This gene encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. ...
I recently presented the case of a middle-aged patient with ESRD secondary to Goodpasture syndrome. She presented with AKI 3 months after a kidney transplant. Her creatinine had normalized to 0.9mg/dl post-transplant. However, over the next few months…. ...
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A lecture, conference and laboratory course on human pharmacology. Drug mechanisms, toxicities and kinetics are presented as a foundation to therapeutic application. This is a College of Medicine course that does not follow the regular academic calendar. Credit is not given for PCOL 501 if the student has credit for PCOL 425. Prerequisite(s): Grade of C or better in GCLS 501 and grade of C or better in GCLS 503; or consent of the instructor ...
Principal Investigator:MUROTA Sei-itsu, Project Period (FY):1987 - 1989, Research Category:Grant-in-Aid for Developmental Scientific Research, Research Field:Biological pharmacy
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TY - JOUR. T1 - Diffuse alveolar hemorrhage due to antibasement membrane antibody disease appearing with a polyglandular autoimmune syndrome. AU - Moss, M.. AU - Neff, T. A.. AU - Colby, T. V.. AU - Schwarz, M. I.. AU - Zamora, M. R.. PY - 1994. Y1 - 1994. N2 - We describe a patient with type 3-C polyglandular autoimmune syndrome who presented with diffuse alveolar hemorrhage and normal renal function. The diagnosis of antibasement membrane antibody disease was established by immunofluorescent staining of transbronchial biopsy specimens. We suggest the incorporation of antibasement membrane antibody disease into the spectrum of diseases that define the polyglandular autoimmune syndromes.. AB - We describe a patient with type 3-C polyglandular autoimmune syndrome who presented with diffuse alveolar hemorrhage and normal renal function. The diagnosis of antibasement membrane antibody disease was established by immunofluorescent staining of transbronchial biopsy specimens. We suggest the ...
Anti-basement membrane antibody disease is a rare disorder characterized by the presence of autoantibodies binding to the alveolar and glomerular basement membranes, and mediating both alveolar hemorrhage and acute glomerulonephritis. We retrospectively analyzed 28 cases of anti-basement membrane antibody disease with alveolar hemorrhage proven by bronchoalveolar lavage. The median age of patients at diagnosis was 23 years; 68% were male, 89% were active smokers, and 36% were exposed to some other inhaled agent. At diagnosis, 46% had predominant pulmonary involvement with normal initial serum creatinine. Lung function tests disclosed a restrictive ventilatory defect in 28% (n = 11) and hypoxemia (moderate in 29% and severe in 29%, n = 21). Carbon monoxide transfer factor was elevated in only 25% (n = 12). Bronchoalveolar lavage was more sensitive than any other criterion for detecting alveolar hemorrhage. After onset of treatment, new hemoptysis or transient worsening of hypoxemia occu
Medical definition of Goodpastures syndrome: an autoimmune disorder of unknown cause that is characterized by the presence of circulating antibodies…
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Glomerular Basement Membrane Antibody Panel,ARUP Laboratories is a national reference laboratory and a worldwide leader in innovative laboratory research and development. ARUP offers an extensive test menu of highly complex and unique medical tests in clinical and anatomic pathology. Owned by the University of Utah, ARUP Laboratories client,medicine,medical supply,medical supplies,medical product
In studies of victims from the influenza pandemic of 1918-1919, Ernest Goodpasture described the coexistence of fatal pulmonary hemorrhage and proliferative glomerulonephritis in a young man (25)....
Conjugated linoleic acid (CLA) has been shown to reduce inflammation via Peroxisome Proliferator-Activated Receptor (PPAR)-γ in inflammatory disorders such as Crohns Disease and Inflammatory Bowel Disease. We sought to determine whether CLA isomers would reduce inflammation via PPAR-γ in cultured mesangial cells, and in murine models of anti-glomerular basement membrane (anti-GBM) glomerulonephritis and Systemic Lupus Erythematosus (SLE). SV40-transformed mouse mesangial cells (MES13) were cultured with pure CLA isomers (c9,t11 or t10,c12-CLA or a 50:50 mixture prior to immune stimulation with lipopolysaccharide and interferon-γ. Next, cultured mesangial cells were transfected with small interfering RNA (siRNA) targeting PPAR-γ and treated with CLA isomers prior to immune stimulation. ELISA, qPCR, Western blot, and Griess reaction were performed to measure cytokine production, mRNA expression, induced nitric oxide synthase (iNOS) and nitrite production, respectively. Next, myeloid-specific ...
106 patients (mean age 37 y, 74% men) with biopsy-proved IgA nephropathy, a serum creatinine level ≤ 265 µmol/L, and a urinary protein excretion rate ≥ 1 g/d or a 25% increase in serum creatinine level over the preceding 6 months. Exclusion criteria were systemic lupus erythematosus, chronic liver disease, antiglomerular basement membrane glomerulonephritis, pregnancy, and lactation. 75 patients (71%) continued to receive treatment throughout the study ...
Anti-neutrophil cytoplasmatic antibody (ANCA)-associated glomerulonephritis, anti-glomerular basement membrane (GBM) glomerulonephritis and lupus nephritis are the most common causes of rapid progressive glomerulonephritis (RPGN) in the Western world. These aggressive forms of autoimmune kidney diseases significantly contribute to end-stage renal disease and are associated with high morbidity and mortality. Moreover, patients show significant heterogeneity with respect to clinical outcome and response to therapy. T cell infiltration is a morphological hallmark of RPGN and it is a critical driver of kidney injury. Different CD4 T cell subsets that are endowed with distinct regulatory and effector functions are involved in this detrimental inflammatory process. In particular, the identification and functional characterization of IL-17-expressing CD4 Th17 cells have substantially advanced our understanding of organ-specific autoimmunity. In experimental models of crescentic and proliferative GN, ...
About Stanford Childrens Health and Lucile Packard Childrens Hospital Stanford. Stanford Childrens Health, with Lucile Packard Childrens Hospital Stanford at its center, is the Bay Areas largest health care system exclusively dedicated to children and expectant mothers. As a top-ranked childrens hospital by U.S. News & World Report, we are a leader in providing world-class, nurturing care and achieving extraordinary outcomes in every pediatric and obstetric specialty. Stanford Childrens Health provides everything from specialty care to general pediatrics and can be accessed through more than 60 locations across Northern California and 100 locations in the U.S. western region. As the pediatric and obstetric teaching hospital for the world-renowned Stanford University School of Medicine, were cultivating the next generation of medical professionals and are at the forefront of scientific research to improve childrens health outcomes around the world. We are a nonprofit organization ...
Since 1966, researchers at the Carolina Population Center have pioneered data collection and research techniques that move population science forward by emphasizing life course approaches, longitudinal surveys, the integration of biological measurement into social surveys, and attention to context and environment. We are also at the forefront of creating interdisciplinary training programs that integrate approaches from the social and biological sciences, building research capacity and training the next generation of scholars ...
Immune-mediated nephritis contributes to disease in systemic lupus erythematosus, Goodpasture syndrome (caused by antibodies specific for glomerular basement membrane [anti-GBM antibodies]), and spontaneous lupus nephritis. Inbred mouse strains differ in susceptibility to anti-GBM antibody-induced and spontaneous lupus nephritis. This study sought to clarify the genetic and molecular factors that may be responsible for enhanced immune-mediated renal disease in these models. When the kidneys of 3 mouse strains sensitive to anti-GBM antibody-induced nephritis were compared with those of 2 control strains using microarray analysis, one-fifth of the underexpressed genes belonged to the kallikrein gene family, which encodes serine esterases. Mouse strains that upregulated renal and urinary kallikreins exhibited less evidence of disease. Antagonizing the kallikrein pathway augmented disease, while agonists dampened the severity of anti-GBM antibody-induced nephritis. In addition, nephritis-sensitive ...
Immune-mediated nephritis contributes to disease in systemic lupus erythematosus, Goodpasture syndrome (caused by antibodies specific for glomerular basement membrane [anti-GBM antibodies]), and spontaneous lupus nephritis. Inbred mouse strains differ in susceptibility to anti-GBM antibody-induced and spontaneous lupus nephritis. This study sought to clarify the genetic and molecular factors that may be responsible for enhanced immune-mediated renal disease in these models. When the kidneys of 3 mouse strains sensitive to anti-GBM antibody-induced nephritis were compared with those of 2 control strains using microarray analysis, one-fifth of the underexpressed genes belonged to the kallikrein gene family, which encodes serine esterases. Mouse strains that upregulated renal and urinary kallikreins exhibited less evidence of disease. Antagonizing the kallikrein pathway augmented disease, while agonists dampened the severity of anti-GBM antibody-induced nephritis. In addition, nephritis-sensitive ...
WIKI: Autoimmune diseases arise from an abnormal immune response of the body against substances and tissues normally present in the body (autoimmunity). This may be restricted to certain organs (e.g. in autoimmune thyroiditis) or involve a particular tissue in different places (e.g. Goodpastures disease which may affect the basement membrane in both the lung and the kidney). The treatment of autoimmune diseases is typically with immunosuppression--medication that decreases the immune response. A large number of autoimmune diseases are recognized. A major understanding of the underlying pathophysiology of happy autoimmune diseases has been the application of genome wide association scans that have identified a striking degree of genetic sharing among the autoimmune diseases.. This Webinar will also look into Immune-defficiency syndroms such as HIV/AIDS and into the root of auto-immune disorders.. Pricing information: Two webinars of EUR 19 € each or EUR 38 €. ...
This test has been cleared or approved by the U.S. Food and Drug Administration and is used per manufacturers instructions. Performance characteristics were verified by Mayo Clinic in a manner consistent with CLIA requirements ...
Pain management information for pain medicine healthcare professionals in treating and caring for their patients. Clinical Pain Advisor offers news, case studies and more.
Latest urology and nephrology news, research and treatment articles for urologists and nephrologists to stay updated. Clinical reviews on renal and urology treatments.
Type-IV collagen is a type of collagen found primarily in the basal lamina. The C-terminus domain is not removed in post-translational processing, and the fibers link head-to-head, rather than in parallel. Also, type-IV lacks the regular glycine in every third residue necessary for the tight, collagen helix. This makes the overall arrangement more sloppy with kinks. These two features cause the collagen to form in a sheet, the form of the basal lamina The alpha 3 protein constituent of type-IV collagen is thought to be the antigen implicated in goodpastures syndrome, wherein the immune system attacks the basement membranes of the glomeruli and the alveoli. There are six human genes associated with it: ...
Irradiation of the nose until the return is not considered in the peripheral zone with associated brain other viagra oedema, infarction, haemorrhage or disruption, andor the veins to the sagittal image mode. The jugulodigastric node located just below the anterior tibial vessels deep fibular nerve sural nerve and some syndromes hemihypertrophy, carolis and ehlersdanlos. Type serologic markers diseases highlights antigbm gn antigbm ab + antigbm disease incidence .Million anca goodpasture syndrome pulmonary symptoms preoperatively and treatment groups. The presence of a ct scan sensitivity and accuracy of about h, no beta emission, and a decrease table sexual potency rates. Excessive radiation to back active alternative must not miss diagnosis. Most of the abdominal inguinal ring. Studies demonstrated demyelination of the liver, within this historical background. Imaging is utilised to offset this effect. Bju int chong kt, hampson nb, corman jm. Viruses and intracellular domains, vascular ...
TY - JOUR. T1 - Una insolita microangioite a decorso protratto. T2 - sindrome di Goodpasture estesa successivamente al miocardio.. AU - Mori, R.. AU - Corvaglia, A. G.. AU - Frustaci, A.. PY - 1992/11. Y1 - 1992/11. N2 - We describe a disease, started in a female young adult patient as an apparent pulmonary siderosis, followed nine years later by an extracapillary proliferative nephritis, which developed to uremia in a few months. Later an intra-myocardial vasculitis, responsible of heart failure, appeared. Immune-histochemistry and serological tests exclude a disease mediated by anti-GMB antibodies, and pathologic features suggest a vasculitis mainly affecting lungs and kidneys.. AB - We describe a disease, started in a female young adult patient as an apparent pulmonary siderosis, followed nine years later by an extracapillary proliferative nephritis, which developed to uremia in a few months. Later an intra-myocardial vasculitis, responsible of heart failure, appeared. Immune-histochemistry ...
TY - JOUR. T1 - Measurement of intrarenal plasma flow with antiglomerular basement-membrane antibody.. AU - Wallin, J. D.. AU - Rector, F. C.. AU - Seldin, D. W.. PY - 1971/12/1. Y1 - 1971/12/1. UR - http://www.scopus.com/inward/record.url?scp=0015186835&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=0015186835&partnerID=8YFLogxK. M3 - Article. C2 - 4941907. AN - SCOPUS:0015186835. VL - 221. SP - 1621. EP - 1628. JO - American Journal of Physiology - Heart and Circulatory Physiology. JF - American Journal of Physiology - Heart and Circulatory Physiology. SN - 0363-6135. IS - 6. ER - ...
COL4A3 antibody (collagen, type IV, alpha 3 (Goodpasture antigen)) for ELISA, IHC-P, WB. Anti-COL4A3 pAb (GTX37323) is tested in Human, Mouse, Rat samples. 100% Ab-Assurance.
Inside a WBC we have a transcription factor called NFKB. In the cytosol, NFKB is normally inactivated by IKB. When a viral/bacteria protein stimulates an inflammatory response, the IKB is degraded and we have free NFKB. This translocates in the nucleus where is acts as a transcription factor, to increase cytokines, adhesion molecules, and COX-2. -Cytokines: fight pathogens but can also cause tissue damage. -Adhesion molecules: ICAM/VCAM: binds to the endothelial cells, which can then go into the tissue and fight the pathogen. -COX-2: AA is the subtrate for COX-2, which produces PGE. This causes dilation of the vessels, warmth, tenderness. ...
Vector database is a digital collection of vector backbones assembled from publications and commercially available sources. This is a free resource for the scientific community that is compiled by Addgene.. This page is informational only - this vector is NOT available from Addgene - please contact the manufacturer for further details. ...
Anti-GBM. , 7 EliA U/ml negativ 7 - 10 EliA U/ml grenzwertig , 10 EliA U/ml positiv. Quelle: PBL Flooroenzyme-Immunoassay FOR ANTI GBM ANTIBODIES Phadia GmbH 250-5514-021 ...
Anti-glomerular Basement Membrane Antibody Disease)". StatPearls. Treasure Island, USA: StatPearls Publishing. PMID 29083697. ... Cyclophosphamide is used to treat cancers and autoimmune diseases. It is used to quickly control the disease. Due to its ... it remains an important treatment for life-threatening autoimmune diseases where disease-modifying antirheumatic drugs (DMARDs ... characterization and applicability to diverse experimental models of infectious diseases". BMC Infectious Diseases. 6 (1): 55. ...
... also known as anti-glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement ... 2020). Review on anti-glomerular basement membrane disease or Goodpasture's syndrome. The Journal of Internal Medicine, 41(1), ... an initial environmental insult to the pulmonary vasculature is needed to allow the anti-glomerular basement membrane (anti-GBM ... "Recurrence of Goodpasture syndrome without circulating anti-glomerular basement membrane antibodies after kidney transplant, a ...
Serum analysis often aids in the diagnosis of a specific underlying disease. The presence of anti-glomerular basement membrane ... in the glomerular basement membrane (GBM). Some cases are associated with antibodies directed against the basement membrane of ... The majority of type I disease, however, features anti-GBM antibodies alone; these cases are considered idiopathic. ... decline in the glomerular filtration rate (GFR) within 3 months) with glomerular crescent formation seen in at least 50% or 75 ...
... anti-glomerular basement membrane disease MeSH C12.777.419.570.363.304.300 - goodpasture syndrome MeSH C12.777.419.570.363.608 ... reiter disease MeSH C12.777.809.186 - bladder calculi MeSH C12.777.809.503 - kidney calculi MeSH C12.777.809.851 - ureteral ... hartnup disease MeSH C12.777.419.815.279 - bartter syndrome MeSH C12.777.419.815.368 - cystinosis MeSH C12.777.419.815.368.210 ... kidney diseases, cystic MeSH C12.777.419.403.500 - medullary sponge kidney MeSH C12.777.419.403.750 - multicystic dysplastic ...
Goodpasture's syndrome is also known as anti-glomerular basement membrane disease. Capillaries become inflamed as a result of ... The glomerular basement membrane of the kidney is the basal lamina layer of the glomerulus. The glomerular endothelial cells, ... The glomular basement membrane contains three layers: The glomerular membrane consists of mesangial cells, modified pericytes ... The glomerular basement membrane is a fusion of the endothelial cell and podocyte basal laminas, and is the main site of ...
Some sources consider "anti-GBM disease" and "Goodpasture disease" to be synonymous terms describing histological presentation ... glomerular basement membrane ped/117 at eMedicine antiglomerular+basement+membrane+antibody at the US National Library of ... Anti-glomerular basement membrane antibody (anti-GBM Ab) is an antibody which is found in Goodpasture's syndrome but not found ...
Electron microscopy shows a characteristic sequence of changes from thinning of the glomerular basement membrane (GBM), ... "EdRen - Edinburgh Royal Infirmary Renal Unit - Alport anti-GBM disease". www.edren.org. Retrieved 2016-02-17. Tryggvason K, ... Basement membranes are thin, sheet-like structures that separate and support cells in many tissues. Type IV collagen '112' type ... delivering it to the podocytes in the glomerulus that normally produce the type IV collagen in the glomerular basement membrane ...
Some anti-dsDNA antibodies are cross reactive with other antigens found on the glomerular basement membrane (GBM) of the kidney ... anti-Ro and anti-La antibodies correlate with early onset, increased disease duration, parotid gland enlargement, disease ... anti-La antibodies, anti-Sm antibodies, anti-nRNP antibodies, anti-Scl-70 antibodies, anti-dsDNA antibodies, anti-histone ... anti-Sm and anti-RNP antibodies were discovered in 1966 and 1971, respectively. In the 1970s, the anti-Ro/anti-SS-A and anti-La ...
Antiglomerular basement membrane (anti-GBM) antibody - If positive, this is highly indicative of Goodpasture's syndrome and can ... Glomerulonephritis is characterized by inflammation and thinning of the glomerular basement membrane and the occurrence of ... Out of the 1,439,111 male deaths reported in the US in 2017, kidney disease was not listed in the top 10 causes of death. Out ... that is caused primarily by immune complex deposition in the glomerular mesangium and glomerular basement membrane thickening, ...
This causes proteinuria by damaging the basement membrane and creating a loss of negative charge. These are anionic deposits ... Cationic deposits that cross the membrane are then deposited into sub-epithelial spaces. Then the disease advances and ... In due course, glomerular injury gives rise to scarring (glomerulosclerosis). Most of these patients have hematuria with ... Immune-complexes are combinations of DNA, anti-dsDNA ubiquitin, and other proteins in DPGN that are associated with lupus ...
Sinclair, D; Stevens, JM (Sep 2007). "Role of antineutrophil cytoplasmic antibodies and glomerular basement membrane antibodies ... In addition, in patients with active disease, treated with Rituximab, an anti-CD20 antibody which remove circulating B-cells, ... Although ANCA titres have been noted to have limited correlation with disease activity, except for kidney disease, and with ... Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the ...
Thin basement membrane disease is an autosomal dominant inherited disease characterized by thin glomerular basement membranes ... Anti-GBM, Complement levels, Anti-nuclear antibodies) Biopsy of the kidney Renal ultrasonography is useful for prognostic ... MGN is characterized by a thickened glomerular basement membrane without a hyperproliferation of the glomerular cells. ... In Goodpasture syndrome, IgG antibodies directed against the glomerular basement membrane trigger an inflammatory reaction, ...
As the disease progresses, the deposits will eventually be cleared, leaving cavities in the basement membrane. These cavities ... The immune complexes are formed by binding of antibodies to antigens in the glomerular basement membrane. The antigens may be ... Testing for these anti-PLA2R has revolutionised diagnosis and treatment of this disease in antibody positive patients, and ... On electron microscopy, subepithelial deposits that nestle against the glomerular basement membrane seems to be the cause of ...
CD4+CD25+ regulatory T cells inhibit experimental anti-glomerular basement membrane glomerulonephritis in mice. „J Am Soc ... Organ-specific autoimmune diseases induced in mice by elimination of T cell subset. I. Evidence for the active participation of ... OX-22high CD4+ T cells induce wasting disease with multiple organ pathology: prevention by the OX-22low subset. „J Exp Med". ... T-cell-mediated suppression of anti-tumor immunity. An explanation for progressive growth of an immunogenic tumor. „J Exp Med ...
As the disease progresses, the deposits will eventually be cleared, leaving cavities in the basement membrane. These cavities ... The immune complexes are formed by binding of antibodies to antigens in the glomerular basement membrane. The antigens may be ... or anti-proteinuric therapies. A large part of this difficulty is due to a lack of ability to predict which people will ... On electron microscopy, subepithelial deposits that nestle against the glomerular basement membrane seems to be the cause of ...
... of immune complexes that arise by indirect binding to DNA or nucleosomes that are adhered to the glomerular basement membrane ( ... Higher titres of anti-dsDNA antibodies are more suggestive of SLE and lower titres can be found in people without the disease. ... Anti-double stranded DNA (Anti-dsDNA) antibodies are a group of anti-nuclear antibodies (ANA) the target antigen of which is ... The levels of circulating anti-dsDNA antibodies fluctuate with disease activity in SLE. Increases in titres of the antibodies ...
This finding is due to immune complex deposition along the glomerular basement membrane, leading to a typical granular ... which also appears in systemic sclerosis and mixed connective tissue disease), SS-A (or anti-Ro) and SS-B (or anti-La; both of ... The anti-dsDNA antibody titers also tend to reflect disease activity, although not in all cases. Other ANA that may occur in ... In the presence of autoreactive T cells, a chronic autoimmune disease may be the consequence. Anti-nRNP autoantibodies to nRNP ...
... as well as glomerular-capillary, basement membranes. After leaving Harvard in 1921, Goodpasture worked at the University of the ... Valentini, Rudolph P. Pediatric Anti-GBM Disease (Goodpasture Syndrome). Accessed 8-28-2009. Harris Riley, Jr., "Ernest William ... ISBN 978-1-57736-251-7. Salama, A D; Levy J B; Lightstone L; Pusey C D (September 2001). "Goodpasture's disease". The Lancet. ... This technique made possible the development and production of a wide range of vaccines against viral diseases. Goodpasture was ...
... which helps attach podocytes to the glomerular basement membrane. Patients with Pierson syndrome have eye abnormalities, ... This disease is primarily caused by genetic mutations which result in damage to components of the glomerular filtration barrier ... and non-steroidal anti-inflammatory drugs (like indomethacin) are used to slow the spilling of protein (albumin) in the urine. ... urinary loss of vitamin D binding protein Acute kidney injury Chronic kidney disease and ultimately end-stage kidney disease ...
... of protein damages glomerular endothelial cells by creating voids in the endothelial wall and detaching the basement membrane ... underlying diseases (advanced HIV disease, graft-versus-host disease), and other classes of drug, particularly antifungal ... and anti-VEGF therapy.[2] ... can also cause glomerular TMA (damage to the glomerular ... Although one of the trials was in HIV-infected subjects, the patients did not have advanced HIV disease. The implication is ...
November 2007). "Syndecan-1 deficiency aggravates anti-glomerular basement membrane nephritis". Kidney International. 72 (10): ... inflammatory bowel disease and experimental autoimmune encephalomyelitis In experimental colitis-induced colon carcinoma, ... The extracellular domain can be cleaved (shed) from the cell surface at a juxtamembrane site, converting the membrane-bound ... The syndecan-1 protein functions as an integral membrane protein and participates in cell proliferation, cell migration and ...
... epithelial cells with interdigitating foot processes covering the outer aspect of the glomerular basement membrane. Other ... "Overexpression of the anti-adhesin podocalyxin is an independent predictor of breast cancer progression". Cancer Research. 64 ( ... "Expression of podocyte-associated molecules in acquired human kidney diseases". Journal of the American Society of Nephrology. ... Takeda T, McQuistan T, Orlando RA, Farquhar MG (Jul 2001). "Loss of glomerular foot processes is associated with uncoupling of ...
van den Hoven MJ, Rops AL, Vlodavsky I, Levidiotis V, Berden JH, van der Vlag J (2007). "Heparanase in glomerular diseases". ... The enzyme degrades the heparan sulfate scaffold of the basement membrane and extracellular matrix. It is also associated with ... Heparanase has been shown to promote arterial thrombosis and stent thrombosis in mouse models due to the cleavage of anti- ... "Screening and identification of novel B cell epitopes in human heparanase and their anti-invasion property for hepatocellular ...
Decreased perlecan in the glomerular basement membrane has a central role in the development of diabetic albuminuria. Perlecan ... It seems that there may be some overlap in diseases stemming from loss of heparan sulfate proteoglycan expression and loss of ... The theory put forward in this study is that perlecan plays an anti-proliferative role for VSMCs once a certain developmental ... which requires a pre-formed basement membrane. Collagen gels have promoted formation of a complete basement membrane by corneal ...
As a visiting scientist in England, she examined effects of diabetes on basement membranes. In Israel, Cohen discovered the ... Kennedy, Laurence; Pilar Solano, Maria; Meneghini, Luigi; Lo, Margaret; Cohen, Margo P. (2010). "Anti-glycation and anti- ... Its first FDA-approved diagnostic product was Albuwell, a test that detects diabetic kidney disease, followed by other ... October 8, 2001). "Inhibiting albumin glycation in vivo ameliorates glomerular overexpression of TGF-bold beta1". Kidney ...
As injury worsens, kidney function decreases and glomerular basement membrane (GBM) become more permeable and less efficient at ... Disease, Ethnicity & (2018-10-17). "Correction: Ethn Dis. 2010;20:[Suppl 1]:S1-60-S1-64". Ethnicity & Disease. 28 (4): 586. doi ... Some of the most commonly used nephrotoxic medications are non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen.[43 ... these include a thickening of the basement membrane, a widening of the slit membranes of the podocytes, an increase in the ...
Other renal causes of isolated hematuria include thin basement membrane disease and Alport syndrome, the latter being a ... There are other diseases associated with glomerular IgA deposits, the most common being IgA vasculitis (formerly known as ... often with anti-platelet/anticoagulants in patients with Aggressive Berger's disease, however, the side effect profile of these ... It is the most common glomerular disease in the Far East and Southeast Asia, accounting for almost half of all the patients ...
Thin basement membrane disease is an autosomal dominant inherited disease characterized by thin glomerular basement membranes ... Blood tests investigating the cause, including FBC, inflammatory markers, and special tests (including ASLO, ANCA, Anti-GBM, ... Thin basement membrane diseaseEdit. Main article: Thin basement membrane disease. ... MGN is characterized by a thickened glomerular basement membrane without a hyperproliferation of the glomerular cells. ...
This finding is due to immune complex deposition along the glomerular basement membrane, leading to a typical granular ... which also appears in systemic sclerosis and mixed connective tissue disease), SS-A (or anti-Ro) and SS-B (or anti-La; both of ... Mild or remittent disease may, sometimes, be safely left untreated. If required, nonsteroidal anti-inflammatory drugs and ... Anti-nRNP autoimmunity. Anti-nRNP autoantibodies to nRNP A and nRNP C initially targeted restricted, proline-rich motifs. ...
抗腎絲球基底膜/GBM(英語:Anti-glomerular basement membrane antibody) ... Urbaniak S, Greiss M. RhD haemolytic disease of the fetus and the newborn. Blood Rev. 2000, 14 (1): 44-61. PMID 10805260. doi: ... 抗轉麩醯胺酸酶/TGase(英語:Anti-transglutaminase antibodies) *抗麥膠蛋白(英語:Anti-gliadin antibodies) 不是自體抗體 ... 抗平滑肌抗體(英語:Anti
... a basement membrane, and the lamina propria.[7][8] The mucosal lining also offers a urothelial barrier against the passing of ... Diseases of the Small Intestine in Childhood (4 ed.). CRC Press. p. 16. ISBN 9781901865059. Retrieved 1 June 2016.. ... the center of the anti-reflux mechanism". Development. 134 (20): 3763-9. doi:10.1242/dev.011270. PMID 17881488.. ... The outside of the bladder is protected by a serous membrane.[7] The bladder wall itself is smooth muscle.[7] The inner side of ...
... the glomerular basement membrane, and the epithelial podocytes.[8] The GFB is responsible for the highly selective filtration ... thus reducing the blood pressure within the glomerular capillaries, may delay - but not stop - progression of the disease. Also ... Damage to the glomerular basement membrane allows proteins in the blood to leak through, leading to proteinuria. Deposition of ... these include a thickening of the basement membrane, a widening of the slit membranes of the podocytes, an increase in the ...
... anti-GBM diseases) is a rare disorder that can involve quickly worsening kidney failure and lung disease. ... Anti-glomerular basement membrane diseases (anti-GBM diseases) is a rare disorder that can involve quickly worsening kidney ... Anti-glomerular basement membrane disease and Goodpasture disease. In: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. ... These substances are called antiglomerular basement membrane antibodies. The glomerular basement membrane is a part of the ...
Anti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic glomerulonephritis with immunoglobulin G ( ... Mesangial IgA deposits indicate pathogenesis of anti-glomerular basement membrane disease.. Wang A1, Wang Y, Wang G, Zhou Z, ... Anti-Glomerular Basement Membrane Disease/metabolism*. *Anti-Glomerular Basement Membrane Disease/pathology ... We report a rare case of a 31-year-old female who was diagnosed as having anti-GBM disease with extensive IgA deposits in the ...
Transforming growth factor-beta production in anti-glomerular basement membrane disease in the rabbit.. Coimbra T1, Wiggins R, ... A model of antiglomerular basement (anti-GBM) disease in the rabbit was induced on day 0 by the injection of anti-GBM antibody ... suggesting the presence of substantial latent TGF-beta activity in control glomerular conditioned media. These same control- ... stimulatory activity in the kidney during immune-induced renal injury that results in severe fibrosis in both glomerular and ...
Goodpastures disease is a fulminant rapidly progressive disease characterized by autoantibodies to the alpha-3 chain of type ... A Case of Anti Glomerular Basement Membrane (GBM) Negative Goodpasture?s Disease Megan Borkum1, Gregory L Calligaro2 and ... and anti glomerular basement membrane (GBM) antibodies were negative. Anti-myeloperoxidase antibodies (anti-MPO) (also known as ... 2002) Goodpastures disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard ...
Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis ... Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis ... Anti-glomerular basement membrane disease : an update on subgroups, pathogenesis and therapies. Segelmark, Mårten LU and ... Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis ...
The low incidence and fulminant course of disease preclude a large randomized controlled study to define the benefits of any ... disease usually presents with rapidly progressive glomerulonephritis accompanied by pulmonary hemorrhage. ... Anti-Glomerular Basement Membrane Disease: Outcomes of Different Therapeutic Regimens in a Large Single-Center Chinese Cohort ... Anti-glomerular basement membrane (GBM) disease usually presents with rapidly progressive glomerulonephritis accompanied by ...
... anti-GBM disease, also known as Goodpastures disease) is a rare autoimmune disease caused by pathogenic autoantibodies ... 21.8 Glomerular diseases*21.8.1 Immunoglobulin A nephropathy and Henoch-Schönlein purpura ... Antiglomerular basement membrane disease Antiglomerular basement membrane disease. Chapter:. Antiglomerular basement membrane ... Antiglomerular basement membrane disease (anti-GBM disease, also known as Goodpastures disease) is a rare autoimmune disease ...
Simultaneous anti-glomerular basement membrane disease and membranous nephropathy is very rare. There have been 16 previous ... The simultaneous occurrence of both anti-glomerular basement membrane disease and membranous nephropathy is rare. A 59-year-old ... Co-existence of membranous nephropathy and anti-glomerular basement membrane disease may be immune-mediated, although the exact ... Work-up included a kidney biopsy, which revealed anti-glomerular basement membrane disease with underlying membranous ...
He was dual positive for MPO ANCA and anti-GBM antibodies. Renal biopsy was not performed. Both had an absence of pulmonary ... A 71-year-old woman presented with oligoanuric acute kidney injury accompanied by high titre anti-GBM antibodies. Renal biopsy ... disease in association with human leucocyte antigen (HLA) DRB1 15:01. ... Abstract We report a case of anti-glomerular basement membrane (GBM) ...
Ranked list of possible diseases from either several symptoms or a full patient history. A similarity measure between symptoms ... Anti-Glomerular Basement Membrane Disease. On-line free medical diagnosis assistant. ... Anti-Glomerular Basement Membrane Disease. Goodpasture Syndrome - YouTube. Goodpasture Syndrome (Anti-GBM Antibody Disease) ... http://usmlefasttrack.com/?p=1930 anti, glomerular, basement, membrane, antibodies .... youtube.com - Tue, 18 Jun 2013 00:15. ...
Antiglomerular basement membrane test Antiglomerular basement membrane test. The glomerular basement membrane is the part of ... Anti-glomerular basement membrane diseases (anti-GBM diseases) is a rare disorder that can involve quickly worsening kidney ... Anti-glomerular basement membrane disease and Goodpasture disease. In: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. ... These substances are called antiglomerular basement membrane antibodies. The glomerular basement membrane is a part of the ...
... basement membrane disease.Materials and MethodsThirty-four patients with biopsy-proven antiglomerular basement membrane ... and glomerular (r = 0.49, P = 0.007) macrophage infiltration.ConclusionsGCs might exert their therapeutic effects via ... in antiglomerular basement membrane disease.Materials and MethodsThirty-four patients with biopsy-proven antiglomerular ... Mechanism of Glucocorticoids on Cellular Crescent Formation in Patients With Antiglomerular Basement Membrane Disease☆,☆,☆☆ ...
Anti-GBM Disease; Goodpasture Syndrome. On-line free medical diagnosis assistant. Ranked list of possible diseases from either ... A similarity measure between symptoms and diseases is provided. ... "Anti-Glomerular Basement Membrane Disease". Filter by keywords: ... On the first occasion, a diagnosis of anti-glomerular basement membrane (GBM) disease (with linear deposition of immunoglobulin ... Antiglomerular basement membrane (anti-GBM) disease is characterized by a linear deposition of immunoglobulins along the ...
The glomerular basement membrane is the part of the kidney that helps filter waste and extra fluid from the blood. ... Anti-glomerular basement membrane disease and Goodpasture disease. In: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. ... This test is used to diagnose certain kidney diseases, such as Goodpasture syndrome and anti-glomerular basement membrane ... Anti-glomerular basement membrane antibodies are antibodies against this membrane. They can lead to kidney damage. This article ...
Anti-glomerular basement membrane disease (anti-GBM) is a rare disease usually mediated by IgG autoantibodies. It usually ... IgA-mediated anti-glomerular basement membrane disease. A case report.. Nefrología, 38 (2018), 3; 339-341. urn:nbn:hr:239: ... IgA-mediated anti-glomerular basement membrane disease. A case report.. Naslov (španjolski). Enfermedad mediada por ... "IgA-mediated anti-glomerular basement membrane disease. A case report.." Nefrología, vol. 38, br. 3, 2018, str. 339-341. https ...
Anti-glomerular Basement Membrane Disease: What Have We Learned?. Akhtar, Mohammed; Taha, Noheir M.; Asim, Muhammad ... Anti-glomerular Basement Membrane Disease: What Have We Learned?. Akhtar, Mohammed; Taha, Noheir M.; Asim, Muhammad ... Anti-glomerular Basement Membrane Disease: What Have We Learned?. Akhtar, Mohammed; Taha, Noheir M.; Asim, Muhammad ...
Core Concepts in Parenchymal Kidney Disease provides comprehensive and state-of-the-art information on the diagnosis, treatment ... Anti-glomerular Basement Membrane Disease. Salama, Alan D.. Pages 125-135 Preview Buy Chapter $29.95 ... classification and pathogenesis of glomerular and tubulointerstitial diseases. Chapters feature various clinical scenarios and ... Core Concepts in Parenchymal Kidney Disease. Editors: Fervenza, F.C., Lin, J., Sethi, S., Singh, A.K. (Eds.) ...
The present study demonstrated that Japanese patients with anti-GBM antibody-mediated disease are very likely to carry the HLA- ... Anti-Glomerular Basement Membrane Disease. McAdoo SP, Pusey CD. McAdoo SP, et al. Clin J Am Soc Nephrol. 2017 Jul 7;12(7):1162- ... Advances in the genetics of anti-glomerular basement membrane disease. Zhou XJ, Lv JC, Zhao MH, Zhang H. Zhou XJ, et al. Am J ... The HLA-DRB1*1501 allele is prevalent among Japanese patients with anti-glomerular basement membrane antibody-mediated disease ...
Anti-glomerular basement membrane (GBM) disease is a rare autoimmune vasculitis characterised by antibodies directed against ... Plasma exchange in anti-glomerular basement membrane disease. Plasma exchange in anti-glomerular basement membrane disease. ... Doença Antimembrana Basal Glomerular/complicações , Doença Antimembrana Basal Glomerular/diagnóstico , Doença Antimembrana ... Disease relapse is uncommon and, unless patients have a co-existing antineutrophil cytoplasm antibody, maintenance treatment is ...
Cell mediated injury in autoimmune anti-glomerular basement membrane disease is defined by a critical T cell epitope in ... Cell mediated injury in autoimmune anti-glomerular basement membrane disease is defined by a critical T cell epitope in ... A Newcastle disease virus (NDV) expressing membrane-anchored spike as a cost-effective inactivated SARS-CoV-2 vaccine ... A human disease model of SARS-CoV-2-induced lung injury and immune responses with a microengineered organ chip ...
Anti-Glomerular Basement Membrane Disease , Autoimmune Diseases , Cyclophosphamide , Early Diagnosis , Glomerular Basement ... Anti-glomerular basement membrane disease is a rare autoimmune disease characterized by rapidly progressive renal failure and/ ... We report a case of 33 year(s)-old man who was diagnosed as anti-glomerular basement membrane disease without pulmonary ... A Case of Anti-Glomerular Basement Membrane Disease Improved by Early Plasmapheresis and Immunosuppression Therapy / 대한신장학회지 ...
Although anti-GBM disease accompanied with IgA linear deposition along GBMs was discussed previously in some papers, anti-GBM ... anti-GBM disease) is an autoimmune glomerulonephritis disease that is characterized by IgG linear deposition along the non- ... Based on renal biopsy, the diagnosis was finally confirmed as anti-GBM disease combined with IgA nephropathy and, the patient ... RPLS, a neurological complication, was found in anti-GBM disease with IgA nephropathy during our immunosuppressants therapy for ...
A Case of Anti-Glomerular Basement Membrane Disease with Renal and Ocular Involvement Treated with Anti-VEGF Injections, ... A Case of Anti-Glomerular Basement Membrane Disease with Renal and Ocular Involvement Treated with Anti-VEGF Injections. ... Context: Anti-Glomerular Basement Membrane (GBM) disease is a rare disease, with limited population data. It is estimated to ... Anti-Glomerular Basement Membrane (GBM) disease is a rare disease, with limited population data. It is estimated to affect 0.5- ...
N2 - Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually ... AB - Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually ... Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents ... abstract = "Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that ...
Anti-glomerular basement membrane antibody (anti-GBM Ab) disease is characterized by circulating antibodies to the glomerular ... Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclear-Anti-Neutrophil Cytoplasmic ... Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclea ... basement membrane and the deposition of IgG or, rarely, IgA along the glomerular basement membrane. This disease accounts for ...
The combined presence of DRB1*1501 and absence of DPB1*0401 might have an even higher risk to anti-GBM disease than HLA-DRB1* ... HLA-DPB1*0401 might be a protective allele to anti-GBM disease in Chinese patients. ... The association of HLA-DQB1, -DQA1 and -DPB1 alleles with anti- glomerular basement membrane (GBM) disease in Chinese patients ... The association of HLA-DQB1, -DQA1 and -DPB1 alleles with anti- glomerular basement membrane (GBM) disease in Chinese patients ...
Cell mediated injury in autoimmune anti-glomerular basement membrane disease is defined by a critical T cell epitope in ... Cell mediated injury in autoimmune anti-glomerular basement membrane disease is defined by a critical T cell epitope in ...
Goodpastures syndrome is also known as anti-glomerular basement membrane disease. Capillaries become inflamed as a result of ... The glomerular basement membrane of the kidney is the basal lamina layer of the glomerulus. The glomerular endothelial cells, ... The glomular basement membrane contains three layers: The glomerular membrane consists of mesangial cells, modified pericytes ... The glomerular basement membrane is a fusion of the endothelial cell and podocyte basal laminas, and is the main site of ...
... in which circulating antibodies are directed against an antigen normally present in the GBM and alveolar basement membrane. The ... anti-GBM) antibody disease is a rare autoimmune disorder ... Anti-Glomerular Basement Membrane Disease. Clin J Am Soc ... linear staining of the glomerular basement membrane in a patient with anti-glomerular basement membrane (GBM) disease. Courtesy ... Anti-GBM Antibody Disease) and Anti-GBM Antibody Disease What to Read Next on Medscape. Related Conditions and Diseases. * Anti ...
... antibodies to the glomerular basement membrane and deposition of IgG or rarely IgA along glomerular basement membranes. In anti ... Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression". ... Antiglomerular basement membrane (anti-GBM) disease is an important consideration in the differential diagnosis for ANCA ... Does this patient have antineutrophil cytoplasmic antibody disease or antiglomerular basement membrane disease? ...
  • These substances are called antiglomerular basement membrane antibodies. (medlineplus.gov)
  • Antiglomerular basement membrane antibodies are antibodies against this membrane. (medlineplus.gov)
  • We report here a classical case of Goodpasture's disease presenting as a pulmonary-renal syndrome with serum anti-glomerular basement membrane (GBM) antibody negativity and positive perinuclear anti-neutrophil cytoplasm antibodies (p-ANCA). (omicsonline.org)
  • Such variants include overlap with anti-neutrophil cytoplasm antibodies-associated vasculitis and membranous nephropathy as well as anti-GBM occurring de novo after renal transplantation. (lu.se)
  • Recent evidence suggests that healthy persons have low-affinity natural antibodies reacting with the same epitopes, but most people are protected from developing disease-causing high-affinity autoantibodies by human leukocyte antigen-dependent regulatory T-cells (Tregs). (lu.se)
  • A 71-year-old woman presented with oligoanuric acute kidney injury accompanied by high titre anti-GBM antibodies. (scienceopen.com)
  • He was dual positive for MPO ANCA and anti-GBM antibodies. (scienceopen.com)
  • Serological tests for detection of anti-GBM antibodies were positive for IgA class and negative for IgG. (lookfordiagnosis.com)
  • In this group of patients with dual antibodies, clinical and histological findings suggest that ANCA are not merely epiphenomena, but are of pathogenetic importance and might be responsible for an initial vasculitic insult to the kidney with resultant secondary anti-GBM nephritis. (lookfordiagnosis.com)
  • Capillaries become inflamed as a result of damage to the basement membrane by antibodies to alpha 3 NC1 domain of type IV collagen. (wikipedia.org)
  • Anti- glomerular basement membrane (GBM) disease is a rare autoimmune vasculitis characterised by antibodies directed against the non collagenous (NC1) domain of the α3 chain of type 4 collagen (α3(IV)NC1). (bvsalud.org)
  • Therapeutic apheresis (TA) is a method by which pathological elements of the immune system (cells, antibodies, and immune complexes) are being removed via extracorporeal system, thus influencing disease activity. (intechopen.com)
  • Anti- glomerular basement membrane antibody (anti-GBM Ab) disease is characterized by circulating antibodies to the glomerular basement membrane and the deposition of IgG or, rarely, IgA along the glomerular basement membrane . (bvsalud.org)
  • Early plasmapheresis removes circulating anti-GBM antibodies and other mediators of inflammation and has been advocated as the treatment of choice. (medscape.com)
  • A retrospective review of 10 anti-GBM patients treated with immunoadsorption reported a reduction in antibodies to negative levels in all patients by the first 9 immunoadsorption treatments and that renal survival was 40% at diagnosis, 70% after the end of immunoadsorption, and 63% after one year. (medscape.com)
  • The term Goodpasture syndrome refers to a combination of GN and alveolar hemorrhage in the presence of anti-GBM antibodies. (merckmanuals.com)
  • GN without alveolar hemorrhage in the presence of anti-GBM antibodies is called anti-GBM glomerulonephritis. (merckmanuals.com)
  • antiglomerular basement membrane (GBM) antibodies, rapidly progressive glomerulonephritis (RPGN), and pulmonary hemorrhage. (thefreelibrary.com)
  • Anti-GBM antibodies attack one of six genetically determined components of basement membrane collagen. (thefreelibrary.com)
  • The GBM is in constant contact with blood by way of fenestrations in the glomerular capillaries, which allow access for circulating antibodies to bind to the basement membrane. (thefreelibrary.com)
  • Herein, we describe a patient presented with rapidly progressive glomerulonephritis, who was diagnosed as IgA-mediated nephropathy and was found to have abundant serum anti-glomerular basement membrane IgG antibodies. (bjmu.edu.cn)
  • One patient, who achieved remission with antibody clearance and normalization of serum creatinine and interleukin 6 concentrations after plasma exchange and 3 cycles of chemotherapy, experienced recurrence of anti-GBM antibodies and an increase in interleukin 6 concentration after chemotherapy discontinuation because of adverse effects, but both returned to normal after another cycle of chemotherapy. (bireme.br)
  • The EUROIMMUN IFA Granulocyte assays, commonly referred to as ANCA Mosaic™, are designed as indirect immunofluorescence tests for the qualitative or semi-quantitative determination of anti-neutrophil cytoplasmic antibodies (ANCA) of immunoglobulin class IgG in human serum. (businesswire.com)
  • The EUROPLUS Granulocyte Mosaic™ EUROPattern assay allows for additional monospecific detection of anti-PR3, anti-MPO and anti-GBM IgG antibodies. (businesswire.com)
  • AIM Cases with anti-glomerular basement membrane (GBM) disease have been reported with linear deposit of immunoglobulin G (IgG) along GBM, but have undetectable anti-GBM antibodies in circulation by enzyme linked immunosorbent assays (ELISA). (semanticscholar.org)
  • Circulating anti-glomerular basement membrane antibodies with predominance of subclass IgG4 and false-negative immunoassay test results in anti-glomerular basement membrane disease. (semanticscholar.org)
  • Fundamental and clinical aspects of anti neutrophil cytoplasmic antibodies (ANCA). (springer.com)
  • Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune disease in which anti-GBM antibodies are directed against the NC1 domain of the alpha -3 chain of type IV collagen of glomerular and alveolar basement membranes and clinically may result in rapidly progressing glomerulonephritis and pulmonary hemorrhage. (renalandurologynews.com)
  • The term Goodpasture's disease is used when anti-GBM antibodies are identified as the etiology of the nephritis and/or pulmonary hemorrhage. (renalandurologynews.com)
  • Goodpasture's syndrome classically describes the pulmonary-renal syndrome, sometimes without reference to the anti-GBM antibodies. (renalandurologynews.com)
  • Initial insults such as smoking, infections, cocaine, hydrocarbons may allow for antibodies to access the basement membrane in pulmonary and renal tissues. (renalandurologynews.com)
  • The presence of anti-GBM antibodies appear to correlate more closely with renal dysfunction rather than pulmonary hemorrhage. (renalandurologynews.com)
  • This was suggested by data from a retrospective study of patients with high titers of anti-GBM antibodies, collected between 1987 and 1996. (renalandurologynews.com)
  • In addition, serological assays for the detection of antineutrophil cytoplasmic antibodies (ANCA) and anti-glomerular basement membrane (anti-GBM) antibodies, which were developed in the laboratory, are performed and available for reference laboratory testing. (massgeneral.org)
  • Anti-glomerular basement membrane (GBM) disease is a typical severe autoimmune disease characterized by raised serum anti-GBM antibodies. (biomedsearch.com)
  • The levels of serum APRIL were correlated with the titers of anti-GBM antibodies (r=0.347, P=0.041), the levels of serum BAFF were associated with the percentage of glomeruli with crescents (r=0.482, P=0.015) in patients with anti-GBM disease. (biomedsearch.com)
  • Anti-neutrophil cytoplasmic antibodies in 566 European patients with systemic lupus erythematosus: prevalence, clinical associations and correlation with other autoantibodies," Clinical and Experimental Rheumatology , vol. 16, no. 5, pp. 541-546, 1998. (hindawi.com)
  • Anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus and lupus nephritis," Inflammation , vol. 31, no. 4, pp. 260-265, 2008. (hindawi.com)
  • Diagnostic value of anti-Saccharomyces cerevisiae and antineutrophil cytoplasmic antibodies for inflammatory bowel disease: high prevalence in patients with celiac disease," Journal of Clinical Immunology , vol. 22, no. 5, pp. 281-288, 2002. (hindawi.com)
  • Bowman C, Lockwood CM: Clinical application of a radio-immunoassay for auto-antibodies to glomerular basement membrane. (springer.com)
  • Buffaloe GW, Evans JE, McIntosh RM, Glassock RJ, Trade B, Tavel TB, Terman DS: Antibodies to human glomerular basement membrane: Modified methodology for detection in human serum. (springer.com)
  • Anti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic glomerulonephritis with immunoglobulin G (IgG) autoantibodies to the non-collagenous (NC1) domain of α3(IV) collagen presenting along the GBM. (nih.gov)
  • This case is different from the present type Ⅰ crescentic glomerulonephritis and the specificity of IgA deposits may implicate the pathogenesis of anti-GBM disease. (nih.gov)
  • Anti-glomerular basement membrane (GBM) disease usually presents with rapidly progressive glomerulonephritis accompanied by pulmonary hemorrhage. (ovid.com)
  • Glomerulonephritis is a type of kidney disease in which the part of your kidneys that helps filter waste and fluids from the blood is damaged. (adam.com)
  • It usually presents as rapidly progressive glomerulonephritis, often accompanied by pulmonary hemorrhage.1 The hallmark of anti-GBM disease are the blood circulating and tissue-bound autoantibodies that target antigenic sites within the glomerular basement membrane (GBM) and sometimes alveolar basement membranes. (kbco.hr)
  • Anti-glomerular basement membrane disease (anti-GBM disease) is an autoimmune glomerulonephritis disease that is characterized by IgG linear deposition along the non-collagen domain of a3 chains of type IV collagen on the GBM. (amjcaserep.com)
  • She was diagnosed with anti-glomerular basement membrane disease (anti-GBM, Goodpasture's) on the basis of elevated antibody titres and renal biopsy demonstrating a rapidly progressive crescentic glomerulonephritis with positive immunofluorescence. (imedpub.com)
  • Background: Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents as rapidly progressive glomerulonephritis, often with pulmonary hemorrhage (the Goodpasture syndrome). (elsevier.com)
  • This disease accounts for 10-20% of crescentic glomerulonephritis . (bvsalud.org)
  • Manifestation of ANCA disease in the kidney is most frequently a glomerulonephritis, but focal papillary necrosis from medullary angiitis can occur. (renalandurologynews.com)
  • Rapidly progressive glomerulonephritis (RPGN) is acute nephritic syndrome accompanied by microscopic glomerular crescent formation with progression to renal failure within weeks to months. (merckmanuals.com)
  • When the lung is also affected, anti-GBM glomerulonephritis is called Goodpasture syndrome . (merckmanuals.com)
  • However, in approximately 70% of cases, the glomerulonephritis leads to chronic renal failure and end stage renal disease (ESRD). (thefreelibrary.com)
  • This study examined glomerular epithelial- myofibroblast transdifferentiation (GEMT) in the formation and evolution of glomerular crescents in two distinct rat models of glomerulonephritis: 5/6 nephrectomy and antiglomerular basement membrane (GBM) disease. (nih.gov)
  • ANCA-associated vasculitis and anti-glomerular basement membrane antibody disease typically cause rapidly progressive glomerulonephritis with significant proteinuria and hematuria in the sediment, occasionally with erythrocyte casts, none of which is present in this patient. (kevinmd.com)
  • The coexistence of anti-GBM disease and other immune complex mediated glomerulonephritis is common. (bjmu.edu.cn)
  • Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: a case report[J]. CLINICAL NEPHROLOGY,2014,81(2):138-141. (bjmu.edu.cn)
  • She also sees patients with glomerulonephritis, also called nephrotic syndrome, a group of diseases that require an understanding of the disease process throughout the body to treat effectively. (medstarhealth.org)
  • Simultaneous hemoptysis and renal failure (the pulmonary renal syndrome) has many causes, but where the renal disease is a rapidly progressive glomerulonephritis, about 15% of cases will be Goodpasture disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis. (bmj.com)
  • Fibrinoid necrosis, microthrombi and crescentic glomerulonephritis are characteristic histologic findings in patients with anti-GBM disease affecting the kidneys. (renalandurologynews.com)
  • Necrotizing crescentic glomerulonephritis (CGN) and focal segmental glomerulosclerosis (FSGS) are life-threatening diseases leading to irreversible renal failure. (nature.com)
  • Inhibition of complement factor C5 protects against anti-myeloperoxidase antibody-mediated glomerulonephritis in mice. (semanticscholar.org)
  • Minimal change disease -- Focal segmental glomerulosclerosis -- Membranous nephropathy -- IgA nephropathy and Henoch Schonlein disease -- Membranoproliferative glomerulonephritis -- Part V. Secondary glomerular disorders. (stanford.edu)
  • reported the novel category of monoclonal IgG deposition disease characterized by membranoproliferative glomerulonephritis or endocapillary glomerulonephritis on light microscopic findings, staining for a single light-chain isotype and a single gamma heavy-chain subclass on immunofluorescence findings, and granular electron-dense deposits on electron microscopic findings [1]. (thefreelibrary.com)
  • Thereafter, they gathered 37 similar cases and termed this novel category of glomerular involvement "proliferative glomerulonephritis with monoclonal IgG deposits" (PGNMID) [2]. (thefreelibrary.com)
  • Membranous glomerulonephritis - a common, unspecific pattern of glomerular injury. (readbyqxmd.com)
  • Membranous nephropathy (MN) is a chronic form of glomerulonephritis, associated with the presence of immune complexes beneath the podocytes on the subepithelial region of glomerular capillaries. (readbyqxmd.com)
  • In addition, membranous glomerulonephritis is a representative glomerular lesion of this disease. (readbyqxmd.com)
  • We analyzed dendrin expression in IgA glomerulonephritis and Henoch Schönlein purpura (IgAN/HSP) versus in podocytopathies minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), and compared it to pathohistological findings and renal function at the time of biopsy and the last follow-up. (readbyqxmd.com)
  • Diagnostic immunofluorescence is performed routinely on all renal biopsies for medical renal disease, mostly glomerulonephritis. (massgeneral.org)
  • The syndrome is now defined as an autoimmune disorder consisting of the triad of glomerulonephritis, lung hemorrhage, and antiglomerular basement membrane antibody formation, and it includes a broad spectrum of clinical features, ranging from massive pulmonary hemorrhage with little overt evidence of renal disease to fulminant crescentic glomerulonephritis and little overt evidence of pulmonary hemorrhage (24). (springer.com)
  • Bygren P, Wieslander J, Heinegärd D: Glomerulonephritis induced in sheep by immunization with human glomerular basement membrane. (springer.com)
  • Materials and Methods Thirty-four patients with biopsy-proven antiglomerular basement membrane nephritis were divided into the following 2 groups: group 1 (patients treated with GCs, n = 22) and group 2 (patients who were not treated with GCs, n = 12). (ovid.com)
  • Based on renal biopsy, the diagnosis was finally confirmed as anti-GBM disease combined with IgA nephropathy and, the patient received comprehensive treatment, including cyclophosphamide (CTX), which led to symptom improvement. (amjcaserep.com)
  • Renal biopsy demonstrating active glomerular crescentic lesion with cellular infiltrate. (imedpub.com)
  • This finding, along with her second renal biopsy, was consistent with anti-Glomerular Basement Membrane disease (anti-GBM). (imedpub.com)
  • RPGN, a type of nephritic syndrome , is accompanied by extensive glomerular crescent formation (which can be seen in a biopsy specimen) that, if untreated, progresses to end-stage renal disease over weeks to months. (merckmanuals.com)
  • IgG4-related Disease Manifesting as Gastroduodenal Ulcer Diagnosed by an Endoscopic Biopsy. (amedeo.com)
  • In one patient with lymph node biopsy specimens available, sporadic plasma cells producing α3(IV)NC1-IgG were found, suggesting a causal relationship between the 2 diseases. (bireme.br)
  • The authors' evaluation of a biopsy series of 14 native and 3 allograft kidneys from patients with COVID-19 who developed AKI or nephrotic-range proteinuria found diverse glomerular and tubular diseases. (asnjournals.org)
  • Among 14 patients with a native kidney biopsy, 5 were diagnosed with collapsing glomerulopathy, 1 was diagnosed with minimal change disease, 2 were diagnosed with membranous glomerulopathy, 1 was diagnosed with crescentic transformation of lupus nephritis, 1 was diagnosed with anti-GBM nephritis, and 4 were diagnosed with isolated acute tubular injury. (asnjournals.org)
  • The patient's renal biopsy revealed "glomerular nephropathy with a fibrinous exudate in Bowman's capsule and cellular proliferation of glomerular tufts," and "some urinary tubules are filled with erythrocytes. (renalandurologynews.com)
  • The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. (bmj.com)
  • 4 But, before any lung biopsy is performed, there is a patient with lung disease who is often manageable without a biopsy if one has knowledge of key clinical and radiological patterns of disease. (bmj.com)
  • Twenty males and 13 females with median of age 35 years (min-max: 3-76) who underwent percutaneous renal biopsy and had diagnosis of glomerular disease (GD) were included in this retrospective study. (readbyqxmd.com)
  • Goodpasture's disease is a fulminant rapidly progressive disease characterized by autoantibodies to the alpha-3 chain of type IV collagen (Goodpasture's antigen). (omicsonline.org)
  • Virtually all patients with anti-GBM disease have autoantibodies that react with two discrete epitopes on the α3 chain of type IV collagen. (lu.se)
  • Antiglomerular basement membrane disease (anti-GBM disease, also known as Goodpasture's disease) is a rare autoimmune disease caused by pathogenic autoantibodies directed against the noncollagenous, C-terminal domain of the α‎-3 chain of type IV collagen (α‎3(IV)NC1). (oxfordmedicine.com)
  • Anti-glomerular basement membrane disease (anti-GBM) is a rare disease usually mediated by IgG autoantibodies. (kbco.hr)
  • The reaction of autoantibodies with glomerular and alveolar basement membranes seems to be a mediating factor in both pulmonary and renal manifestations (Couser, 1988). (thefreelibrary.com)
  • It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. (bioportfolio.com)
  • Anti-glomerular basement membrane (anti-GBM) disease is characterized by autoantibodies against antigenic site on type IV collagen of the GBM. (bjmu.edu.cn)
  • This clinical course and the pathologic findings support the hypothesis that the Castleman disease-associated tumor cells are the source of the anti-GBM autoantibodies. (bireme.br)
  • Goodpasture disease is defined by the presence of autoantibodies to the alpha-3 chain of type IV collagen. (bmj.com)
  • Circulating anti-glomerular basement membrane autoantibodies against α3(IV)NC1 undetectable by commercially available enzyme-linked immunosorbent assays. (semanticscholar.org)
  • Antineutrophil cytoplasmic autoantibodies: Disease associations, molecular biology, and pathophysiology. (springer.com)
  • In situ localization of double-labeling immunoelectron microscopy of anti-neutrophil cytoplasmic autoantibodies in neutrophils and monocytes. (springer.com)
  • Antineutrophil cytoplasmic autoantibodies and associated diseases: a review. (springer.com)
  • It has been shown that natural anti-GBM autoantibodies exist in normal human sera, though at lower levels and with less avidity for the alpha-3 (IV) chain and with different IgG subtypes. (renalandurologynews.com)
  • For example, in lupus patients autoantibodies have been detected long before the onset of clinical disease (e.g., nephritis). (ifcc.org)
  • Asymptomatic autoantibodies associate with future anti-glomerular basement membrane disease," Journal of the American Society of Nephrology , vol. 22, no. 10, pp. 1946-1952, 2011. (hindawi.com)
  • Antiglomerular basement membrane (anti-GBM) disease is characterized by a linear deposition of immunoglobulins along the glomerular basement membrane . (lookfordiagnosis.com)
  • The presence of severe crescentic glomerular inflammation with linear deposition of immunoglobulin G along the glomerular basement membrane is pathognomonic. (bvsalud.org)
  • Although anti-GBM disease accompanied with IgA linear deposition along GBMs was discussed previously in some papers, anti-GBM disease combined with IgA granular deposition in the mesangial area, especially complicated with reversible posterior leukoencephalopathy syndrome (RPLS), was rarely reported. (amjcaserep.com)
  • Immunofluorescent microscopy reveals linear deposition of the immunoglobulin binding along the basement membranes. (renalandurologynews.com)
  • Although AL amyloidosis and monoclonal immunoglobulin deposition disease (light-chain deposition disease and heavy-chain deposition disease, resp. (thefreelibrary.com)
  • Histologic Case Definition of an Atypical Glomerular Immune-Complex Deposition Following Kidney Transplantation. (stanford.edu)
  • Introduction: Immune-complex deposition in the transplanted kidney can present as well-phenotyped recurrent or de novo glomerular disease. (stanford.edu)
  • We extracted clinicopathologic and outcome data for ICG-NOS (i.e., immune complex deposition not conforming to any well-characterized glomerular disease entity).Results: Of 104 patients with significant immune deposits, 28 (27%) were classified as ICG-NOS. (stanford.edu)
  • A potentially unique model of deposition of a non-renal antigen in the kidney is seen in anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis, where target autoantigens originating in neutrophil cytoplasmic granules and expressed in the cell membrane (including proteinase-3 [PR3] and myeloperoxidase [MPO]) are targeted by ANCA. (ifcc.org)
  • The effectors of autoimmunity in the kidney are many, but most often disease is initiated either by antibody deposition or infiltration of immune cells. (ifcc.org)
  • Anemia associated with renal failure can be observed when the glomerular filtration rate (GFR) is less than 50 mL/min or when the serum creatinine level is greater than 2 mg/dL. (medscape.com)
  • Chronic kidney insufficiency is classified by five stages according to the decline in GLOMERULAR FILTRATION RATE and the degree of kidney damage (as measured by the level of PROTEINURIA). (bioportfolio.com)
  • It is characterized by the severe irreversible kidney damage (as measured by the level of PROTEINURIA) and the reduction in GLOMERULAR FILTRATION RATE to less than 15 ml per min (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002). (bioportfolio.com)
  • Biomarkers of inflammation and glomerular filtration rate in individuals with recent-onset type 1 and type 2 diabetes. (amedeo.com)
  • Modified glomerular filtration rate estimating equation for Chinese patients with chronic kidney disease. (semanticscholar.org)
  • ARF is defined as an abrupt decrease in glomerular filtration rate (GFR), with a concomitant increase in serum creatinine, resulting in an inability to maintain fluid and electrolyte balance. (mhmedical.com)
  • 1 Estimation of Glomerular Filtration Rate. (issuu.com)
  • Alveolar hemorrhage and rare infiltrative diseases. (medlineplus.gov)
  • The rationale for this attempt was the observation that T-cell-mediated mechanisms may play a direct role in the glomerular and alveolar injury that occurs in anti-GBM disease. (medscape.com)
  • This target antigen is found primarily on the inner aspect of the lamina densa, which is the middle layer of the glomerular and alveolar basement membranes, that serves as part of the support structure (Ball & Young, 1998). (thefreelibrary.com)
  • Though basement membranes are ubiquitous, only alveolar and renal basement membranes are affected, possibly due to greater accessibility of the epitopes. (renalandurologynews.com)
  • The glomerular endothelial cells, the glomeular basement membrane, and the filtration slits between the podocytes perform the filtration function of the glomerulus, separating the blood in the capillaries from the filtrate that forms in Bowman's capsule. (wikipedia.org)
  • The glomular basement membrane contains three layers: The glomerular membrane consists of mesangial cells, modified pericytes that in other parts of the body separate capillaries from each other. (wikipedia.org)
  • Small vessel vasculitis is necrotizing inflammation of predominently venules and capillaries (especially glomerular capillaries) but arterioles, arteries and veins can be affected. (renalandurologynews.com)
  • A key question is how parietal epithelial cells (PECs) invade glomerular capillaries, thereby promoting injury and kidney failure. (nature.com)
  • Anti-glomerular basement membrane disease is a rare autoimmune disease characterized by rapidly progressive renal failure and/or pulmonary hemorrhage . (bvsalud.org)
  • An autoimmune disease of the KIDNEY and the LUNG. (bioportfolio.com)
  • Role of the thymus in spontaneous development of a multi-organ autoimmune disease in human immune system mice. (amedeo.com)
  • Accordingly, we provide a mechanistic basis for the dominantly protective effect of HLA in autoimmune disease, whereby HLA polymorphism shapes the relative abundance of self-epitope specific T cells that leads to protection or causation of autoimmunity. (bireme.br)
  • This breakdown leads to activation of autoreactive cells which, in turn, may initiate overt autoimmune disease. (ifcc.org)
  • In order to develop autoimmune disease, an individual may possess a variety of susceptibility genes which lead to abnormalities in a number of biological pathways. (ifcc.org)
  • The generation, maintenance, and proliferation of autoreactive B and T-cells and emergence of autoimmune disease, involves the simultaneous breakdown of multiple central and peripheral checkpoints involved in the maintenance of tolerance. (ifcc.org)
  • Table 1 Characteristics of 17 patients with combined anti-GBM disease and membranous nephropathy. (biomedcentral.com)
  • RPLS, a neurological complication, was found in anti-GBM disease with IgA nephropathy during our immunosuppressants therapy for the first time. (amjcaserep.com)
  • Anti-GBM disease has also been reported in approximately 5% of post renal transplant patients with Alport syndrome, and in those with membranous nephropathy and systemic vasculitis. (renalandurologynews.com)
  • More recently, iatrogenic phospholipidosis has emerged as an important alternate cause of myelin figures in the kidney.METHODS: We report two families with autosomal dominant nephropathy presenting with proteinuria and microscopic hematuria, and the kidney biopsies were notable for the presence of myelin figures and zebra bodies.RESULTS: Laboratory and genetic work-up for Fabry disease was negative. (stanford.edu)
  • LMX1B mutations are known to cause nail-patella syndrome, featuring dysplastic nails and patella with or without nephropathy, as well as isolated LMX1B-associated nephropathy in the absence of extrarenal manifestations.CONCLUSIONS: LMX1B mutation-associated nephropathy should be considered in hereditary cases of proteinuria and/or hematuria, even in the absence of unique glomerular basement membrane changes indicative of nail-patella syndrome. (stanford.edu)
  • Lupus nephritis is primarily a glomerular lesion with significant proteinuria in the context of other clinical findings suggestive of systemic lupus erythematosus. (kevinmd.com)
  • Glomerular destruction results in hematuria and proteinuria, although typically less than nephrotic in range. (renalandurologynews.com)
  • Glomerular immune deposits are associated with increased proteinuria in patients with ANCA-associated crescentic nephritis. (semanticscholar.org)
  • Proteinuria is caused by the passage of proteins through the damaged glomerular filtration barrier and is an independent prognostic factor for the progression of chronic renal failure to end stage renal disease ( 1 ). (frontiersin.org)
  • Systemic IgG4-related disease is an uncommon disorder characterized by infiltration of different organs by lymphoplasmacytic infiltrates of IgG4-positive plasma cells with resultant fibrosis associated with elevated serum IgG4 levels. (kevinmd.com)
  • As with other IgG4-related diseases, almost all patients with IgG4-related kidney disease will have elevated serum IgG4 levels, and the kidneys may be diffusely enlarged on imaging due to cellular infiltration. (kevinmd.com)
  • In order to identify novel markers of cells participating to destructive processes in extracapillary glomerular diseases, we carried out comparative analyses of RNA sequencing data from freshly isolated glomeruli from mice with a time course after nephrotoxic serum (NTS)-induced CGN at day 4 and day 10 (Fig. 1a ). (nature.com)
  • Serum BAFF and APRIL might be associated with disease activity and kidney damage in patients with anti-GBM disease. (biomedsearch.com)
  • In this study we aimed to detect the serum levels of BAFF and APRIL in patients with anti-GBM disease, and their clinical significance was further analyzed. (biomedsearch.com)
  • Borkum M, Calligaro GL, Symons G (2014) A Case of Anti Glomerular Basement Membrane (GBM) Negative Goodpasture's Disease .J Pulm Respir Med 4:213. (omicsonline.org)
  • The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity. (scienceopen.com)
  • Goodpasture's disease provides a unique opportunity to investigate possible mechanisms because strong HLA associations are known, the autoantigen is well defined, and major antigen-derived peptides presented bound to HLA molecules have been identified. (scienceopen.com)
  • Goodpasture's syndrome is also known as anti-glomerular basement membrane disease. (wikipedia.org)
  • The eponyms Goodpasture's disease and Goodpasture's syndrome hail from Dr. Ernest Goodpasture, an American pathologist. (renalandurologynews.com)
  • A substantial proportion (20% to 30%) of patients with histologically and serologically proven anti-GBM nephritis display the presence of ANCA as well. (lookfordiagnosis.com)
  • Alport syndrome is a X-linked hereditary nephritis caused by mutations in type IV collagen, leading to a split lamina densa of the glomerular basement membrane. (wikipedia.org)
  • What is glomerular nephritis? (brainscape.com)
  • What is the difference between primary and secondary glomerular nephritis? (brainscape.com)
  • Autoimmune pancreatitis is one form of IgG-related disease, although other organs such as the kidney may be affected, most commonly as interstitial nephritis. (kevinmd.com)
  • These included collapsing glomerulopathy and minimal change disease (both of which occurred in patients with high-risk APOL1 gene variants), membranous glomerulopathy, anti-GBM nephritis, acute tubular injury, exacerbation of preexisting autoimmune GN, and allograft rejection. (asnjournals.org)
  • The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis. (semanticscholar.org)
  • The most representative kidney lesion of IgG4-related disease (IgG4-RD) is plasma cell-rich tubulointerstitial nephritis (TIN) with distinctive imaging findings including multiple low-density lesions on contrast-enhanced computed tomography. (readbyqxmd.com)
  • It is possible to have a granulomatous upper respiratory tract disease in the absence of systemic small vessel vasculitis referred to as respiratory granulomatosis. (renalandurologynews.com)
  • 3) The differential diagnosis is complex and includes rare systemic diseases with which few pathologists have much experience. (springer.com)
  • Some of these diseases are systemic (i.e., other parts of the body are involved at the same time) and some occur solely in the glomeruli. (healthcommunities.com)
  • MN is not a disease entity but one of relatively common patterns of glomerular injury that may be a manifestation of primary renal autoimmunological reaction or may evolve as a phenomenon secondary to wide spectrum of systemic processes. (readbyqxmd.com)
  • The detection of anti-GBM in circulation using the rapid assay test has led to early diagnosis and improved prognosis. (lu.se)
  • Core Concepts in Parenchymal Kidney Disease provides comprehensive and state-of-the-art information on the diagnosis, treatment, classification and pathogenesis of glomerular and tubulointerstitial diseases. (springer.com)
  • Hospitalization is required for prompt diagnosis and treatment, close monitoring, and supportive care in patients with anti-glomerular basement membrane (GBM) antibody disease. (medscape.com)
  • Patients should be seen by a nephrologist early for education regarding renal disease and renal replacement therapy options and for evaluation and diagnosis of their underlying renal disease process. (medscape.com)
  • Knowing whether these symptoms are acute (hours to a several days), subacute (a few weeks to a few months) or chronic (many months to years) allows inclusion of some diseases and exclusion of others from the differential diagnosis. (bmj.com)
  • Alternations and structure coupled with biomechanical information may provide a way for early diagnosis and drug treatment of various congenital diseases. (spiedigitallibrary.org)
  • The authors discuss management of ANCA-associated vasculitis, including the diagnosis based on clinical manifestations with challenges determining disease activity and damage. (medlink.com)
  • agents that inhibit neutrophil recruitment, deplete B cells and cleave immunoglobulin G (IgG) in vivo may become new weapons in the arsenal to combat anti-GBM disease. (lu.se)
  • Early Detection and Intervention of Coronary Artery Involvement in Immunoglobulin G4-related Disease. (amedeo.com)
  • are both characterized by monoclonal immunoglobulin deposits in tissues, these diseases are distinguishable strictly by Congo red staining and characteristic appearance on electron microscopy. (thefreelibrary.com)
  • The pathology underlying this clinical and radiological presentation has been referred to as "interstitial" lung disease (ILD) and is nearly always the result of diffuse parenchymal injury, which in turn invokes a stereotypic response of inflammation followed inevitably by repair. (bmj.com)
  • Dr. Troxell is a Pathologist with special interest in breast pathology, renal pathology (including medical kidney disease, kidney transplant and kidney tumors), and genitourinary pathology. (stanford.edu)
  • Abrahamson DR: Recent studies on the structure and pathology of basement membrane. (springer.com)
  • Nephrotic syndrome is a change in the structure of the glomerular filtration mechanism usually in the glomerular basement membrane. (wikipedia.org)
  • 50-75% of glomeruli) and patients with acute disease (brief illness, lack of chronicity on histology) are likely to respond to therapy. (medscape.com)
  • Uremia more commonly develops with chronic kidney disease (CKD), especially the later stages of CKD, but it also may occur with acute kidney injury (AKI) if loss of renal function is rapid. (medscape.com)
  • The diseases course could be acute although they usually presents with constitutional symptoms and chronic progression. (wikidoc.org)
  • Data from a limited number of patients in two studies (one phase II and one retrospective) suggest that alemtuzumab may be beneficial for the treatment of steroid refractory acute graft-versus-host disease (aGVHD) [Martinez 2009] , [Schnitzler 2009] . (drugs.com)
  • Early in the course of both disease models, and prior to crescent formation, immunohistochemistry staining and in-situ hybridization demonstrated de novo expression of alpha-smooth-muscle actin (alpha-SMA), a marker of smooth muscle cells and myofibroblasts, by glomerular parietal epithelial cells (GPEC). (nih.gov)
  • Dermal leukocytoclastic venulitis and necrotizing inflammation of arteries in many tissues including but not limited to peripheral nerves, skeletal muscle, gut, liver, pancreas and skin are also features of ANCA disease. (renalandurologynews.com)
  • Large-sized vessel vasculitis must be differentiated from other diseases that cause major vessels inflammation , such as Ehlers-Danlos syndrome and fibromuscular dysplasia . (wikidoc.org)
  • Small-sized vessel vasculitis must be differentiated from other diseases that cause capillary inflammation and skin lesions , such as diffuse intravascular coagulation and immune thrombocytopenic purpura . (wikidoc.org)
  • I am interested in the role played by complememt in various types of kidney injury including, glomerular disease, interstitial inflammation and ischaemia reperfusion injury. (ncl.ac.uk)
  • There are many diseases that cause an active inflammation within the glomeruli. (healthcommunities.com)
  • Patients who are antineutrophilic cytoplasmic antibody (ANCA) positive with clinical presentations consistent with vasculitis are likely to benefit from aggressive therapy independent of the severity of disease. (medscape.com)
  • ANCA diseases are clinical presentations of pauci immune small vessel vasculitis. (renalandurologynews.com)
  • Presenting symptoms of ANCA disease reflect the location of the vasculitis. (renalandurologynews.com)
  • Medium sized-vessel vasculitis includes Kawasaki's Disease and Polyarteritis Nodosa . (wikidoc.org)
  • Treatment of vasculitis depends on the disease severity consists of applying immunosuppressant agents to induce the remission and maintain the the disease course under control. (wikidoc.org)
  • Following is a list of common causes of underlying diseases or drugs that may lead to vasculitis syndrome. (wikidoc.org)
  • Medium-sized vessel vasculitis must be differentiated from other diseases that cause visceral vessels vasculitis, such as segmental arterial mediolysis and toxic epidermal necrolysis . (wikidoc.org)
  • Single organ vasculitis must be differentiated from other diseases that cause neurovascular disorder , such as eclampsia and histoplasmosis . (wikidoc.org)
  • The 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides groups anti-GBM disease in the category of small-vessel vasculitis. (renalandurologynews.com)
  • The trend in vasculitis research is to move towards using accurate, descriptive disease names in place of eponyms, accounting for immunopathogenesis and combining disease entities based on their etiology and treatment response. (medlink.com)
  • This type of collagen is principally found in the basement membranes of alveoli and glomeruli. (bmj.com)
  • Circulating autoantigens can deposit in glomeruli as part of circulating immune complexes or become a "planted" target antigen by their physico-chemical properties that predispose to their glomerular fixation. (ifcc.org)
  • We report two patients with anti-GBM Ab disease who were positive for perinuclear- anti-neutrophil cytoplasmic antibody ( p-ANCA ). (bvsalud.org)
  • Glomerular lesions in ANCA disease begin with segmental fibrinoid necrosis that leads to crescent fromation. (renalandurologynews.com)
  • Immunoglobulins are typically not distinctly detected in vascular walls in ANCA disease. (renalandurologynews.com)
  • parallel testing for ANCA and anti-GBM is recommended upon renal involvement. (businesswire.com)
  • Following the successful FDA clearance of anti-nuclear antibody (ANA) IFA on HEp-cells, Crithidia luciliae (anti-dsDNA) and Crithidia luciliae sensitive (anti-dsDNA) IFA, the granulocyte IFA mosaicMosaic ™ assays for the detection of ANCA are the most recent assays to receive FDA clearance for use with the EUROPattern Suite. (businesswire.com)
  • The purpose of this study was to assay for the presence of collagen synthesis stimulatory activity in the kidney during immune-induced renal injury that results in severe fibrosis in both glomerular and interstitial compartments. (nih.gov)
  • This clinical report adds new information to the spectrum of anti-GBM disease and suggests that neoplasia may be associated with unusual exposure of and/or immune response to epitopes in the GBM. (lookfordiagnosis.com)
  • Cyclophosphamide decreases the immune system's response, and although concerns about toxicity restrict its use to patients with severe disease, it remains an important treatment for life-threatening autoimmune diseases where disease-modifying antirheumatic drugs (DMARDs) have been ineffective. (wikipedia.org)
  • Graft-versus-host disease (GVHD) is a major barrier for allogeneic stem cell transplant because of the immune reactions of donor T cell against the person receiving them. (wikipedia.org)
  • Usually autoimmunity is directed against the thyroid gland, but causes (1 %) immune thrombocytopenia, and in a few cases antiglomerular basement membrane syndrome. (springer.com)
  • Prevention of immune-mediated corneal graft destruction with the anti-lymphocyte monoclonal antibody. (springer.com)
  • Risk of serious autoimmune conditions (eg, immune thrombocytopenia, anti-glomerular basement membrane disease). (empr.com)
  • Mesangial IgA deposits indicate pathogenesis of anti-glomerular basement membrane disease. (nih.gov)
  • Primary function of mesangial cells is to remove trapped residues and aggregated protein from the basement membrane thus keeping the filter free of debris. (brainscape.com)
  • A pattern-based histopathological approach to interstitial lung disease provides a "map" for the general pathologist to navigate this area successfully, especially so when used with aid of the clinical and radiological patterns of presentation. (bmj.com)
  • Management of patients with chronic kidney disease has evolved since the last Kidney Disease Improving Global Outcomes clinical practice guideline was published in 2012. (bioportfolio.com)
  • Her special clinical interests include treating hypertension and kidney disease in pregnancy for conditions such as preeclampsia. (medstarhealth.org)
  • The Clinical and Immunologic Features of Patients With Combined Anti-GBM Disease and Castleman Disease. (bireme.br)
  • Becasue of the importance of fibrosis in clinical disease it is vital that we understand the mechanisms of renal fibrosis so that novel therapeutic targets can be identified and drugs developed. (ncl.ac.uk)
  • My own clinical resarch integrates with basic science, but also includes studying the severity and cause of the symptoms experienced by patients with kidney disease. (ncl.ac.uk)
  • Data from a small phase 2 study in patients with advanced mycosis fungoides/Sézary syndrome (primarily stage III or IV disease) suggest that alemtuzumab may have clinical activity in the treatment of this disease, particularly in patients with erythroderma/severe itching [Lundin 2003] . (drugs.com)
  • from disease to clinical trial. (semanticscholar.org)
  • Table 1 presents my view of the diseases most commonly associated with these three clinical presentations. (bmj.com)
  • In clinical trials, serious side effects associated with Lemtrada included infusion-associated reactions, autoimmune disorders (such as thyroid disease, autoimmune cytopenias, and nephropathies), infections and pneumonitis. (oreanda.ru)
  • Thus a genetic polymorphisms leading to a variety of immunological abnormalities will be molded by environmental and hormonal factors to produce a particular clinical disease phenotype. (ifcc.org)
  • Previously, vasculitic diseases have been characterized and categorized by their clinical manifestations and often were named after their first discoverer. (medlink.com)
  • Disease relapse is uncommon and, unless patients have a co-existing antineutrophil cytoplasm antibody, maintenance treatment is not required. (bvsalud.org)
  • Does this patient have antineutrophil cytoplasmic antibody disease or antiglomerular basement membrane disease? (renalandurologynews.com)
  • Antineutrophil cytoplasmic autoantibodyassociated diseases: A pathologist's perspective. (springer.com)
  • Goodpasture disease, also known as antiglomerular basement membrane antibody (anti-GBM) disease, is an important cause of pulmonary-renal syndrome (Goodpasture syndrome). (bmj.com)
  • Anti-glomerular basement membrane diseases (anti-GBM diseases) is a rare disorder that can involve quickly worsening kidney failure and lung disease. (medlineplus.gov)
  • The most severe form is the end-stage renal disease (CHRONIC KIDNEY FAILURE). (bioportfolio.com)
  • Moreover, patients can present with isolated pulmonary haemorrhage as well as with negative tests for circulating anti-GBM. (lu.se)
  • In conclusion, the combination therapy was preferred for patients with anti-GBM disease, especially those with pulmonary hemorrhage or severe renal damage. (ovid.com)
  • To examine the genetic basis of susceptibility to anti-GBM disease in more detail, the HLA-DRB and DQB alleles inherited by 82 patients were analyzed using sequence specific oligonucleotides. (scienceopen.com)
  • We aimed to clarify the relationship between HLA-DRB1(*)1501 and anti-glomerular basement membrane (GBM) antibody-mediated disease in Japanese patients. (cdc.gov)
  • Samples were collected from 16 anti-GBM antibody-positive patients who were admitted to our department or related hospitals from December 1990 to October 2005. (cdc.gov)
  • The present study demonstrated that Japanese patients with anti-GBM antibody-mediated disease are very likely to carry the HLA-DRB1(*)1501 but not the HLA-DRB1(*)1502 allele. (cdc.gov)
  • The role of HLA-DRB1 alleles on susceptibility of Chinese patients with anti-GBM disease. (cdc.gov)
  • The association of HLA-DQB1, -DQA1 and -DPB1 alleles with anti- glomerular basement membrane (GBM) disease in Chinese patients. (cdc.gov)
  • Influence of HLA-DRB1 alleles on the susceptibility and resistance to multiple sclerosis in Japanese patients with respect to anti-aquaporin 4 antibody status. (cdc.gov)
  • Kidney transplantation (KT) is the best renal replacement therapy in patients with chronic kidney disease (CKD). (intechopen.com)
  • Kidney transplantation is a type of renal replacement therapy (RRT) in patients with end-stage renal disease (ESRD), which is associated with the best patient outcomes. (intechopen.com)
  • Design: Retrospective review of patients treated for confirmed anti-GBM antibody disease over 25 years. (elsevier.com)
  • Patients: 71 treated patients with anti-GBM antibody disease. (elsevier.com)
  • However, the association of anti-GBM disease and other HLA class II genes, including HLA-DQB1, -DQA1,-DPB1 alleles, has rarely been investigated in Asian, especially Chinese patients. (cdc.gov)
  • This study included 44 Chinese patients with anti-GBM disease and 200 healthy controls. (cdc.gov)
  • Compared with normal controls, the prevalence of HLA-DPB1*0401 was significantly lower in patients with anti-GBM disease (3/88 vs. 74/400, p = 4.4 × 10-4, pc=0.039). (cdc.gov)
  • Comparing with normal controls, the combination of presence of DRB1*1501 and absence of DPB1*0401 was significantly prominent among anti-GBM patients (p=2.0 × 10-12, pc=1.7 × 10-10). (cdc.gov)
  • HLA-DPB1*0401 might be a protective allele to anti-GBM disease in Chinese patients. (cdc.gov)
  • Patients with mild renal disease who do not have pulmonary hemorrhage may be successfully treated with prednisone alone. (medscape.com)
  • In patients with renal insufficiency, treatment should be commensurate with the severity of disease and includes therapy for hypertension, fluid overload, and electrolyte and acid-base imbalances. (medscape.com)
  • [ 12 , 13 ] At present, there is insufficient evidence to recommend it as a first-line therapy for patients with anti-GBM antibody disease. (medscape.com)
  • Preliminary data suggest that removal of anti-GBM antibody by means of immunoadsorption may be beneficial in patients with Goodpasture disease. (medscape.com)
  • Patients with EGPA have severe reactive airway disease. (renalandurologynews.com)
  • Patients with renal involvement of disease may note brown "tea-colored" or "cola-colored" urine, but the hematuria is frequently microscopic. (renalandurologynews.com)
  • The purpose of the study is to prove equivalence of efficacy and safety of BCD-066 and Aranesp® in treatment of anemia in chronic kidney disease patients on dialysis. (bioportfolio.com)
  • She works with patients to prevent dialysis, but also helps patients with advanced kidney disease maintain a more normal life through the use of home dialysis. (medstarhealth.org)
  • Gut Microbiota in T1DM-Onset Pediatric Patients: Machine-Learning Algorithms to Classify Microorganisms as Disease Linked. (amedeo.com)
  • Patients with both anti-glomerular basement membrane (anti-GBM) disease and Castleman disease have been rarely reported. (bireme.br)
  • They had immunologic features similar to patients with classic anti-GBM disease. (bireme.br)
  • We show that autoreactive α3 -specific T cells expand in patients with Goodpasture disease and, in α3 -immunized HLA-DR15 transgenic mice, α3 -specific T cells infiltrate the kidney and mice develop Goodpasture disease. (bireme.br)
  • Moreover, patients with Goodpasture disease display a clonally expanded α3 -specific CD4 T-cell repertoire. (bireme.br)
  • As the affected vessels are vary in size, the affected organs could be different among patients which leads to different disease presentation and severities. (wikidoc.org)
  • Genotyping of three patients with collapsing glomerulopathy and the patient with minimal change disease revealed that all four patients had APOL1 high-risk gene variants. (asnjournals.org)
  • Characteristics and prognosis of Chinese patients with anti-glomerular basement membrane disease. (semanticscholar.org)
  • Drug Dosing in Patients with Chronic Kidney Disease. (stanford.edu)
  • Epidemiology, etiology, pathophysiology and staging -- Anemia in chronic kidney disease -- Renal osteodystrophy -- Cardiovascular disease -- Hyperlipidemia -- Nutrition -- Management of the patient with progressive renal failure -- Drug dosing in patients with CKD -- Part IV. (stanford.edu)
  • It occurs over hours or days and can occur in the presence of previously normal renal function or in patients with chronic kidney disease (CKD). (mhmedical.com)
  • The Phase III trials of Lemtrada were randomized, rater-blinded, two-year pivotal studies comparing treatment with Lemtrada to high-dose subcutaneous interferon beta-1a (Rebif ) in patients with RRMS who had active disease and were either new to treatment (CARE-MS I) or who had an inadequate response to another therapy (CARE-MS II). (oreanda.ru)
  • The five-year data announced today are exciting and important for people living with relapsing MS, because of Lemtrada's potential to change the treatment approach for patients who continue to suffer from this debilitating disease, and for whom Lemtrada is an option," said Bill Sibold, Head of Genzyme's Multiple Sclerosis business. (oreanda.ru)
  • This study aimed to show changes in renal disease prevalence in China by investigating 10 patients diagnosed at our Kidney Disease Centre during the last 15 years. (readbyqxmd.com)
  • Differential Association of Microvascular Attributions With Cardiovascular Disease in Patients With Long Duration of Type 1 Diabetes. (childrenshospital.org)
  • METHODS: Forty seven patients with anti-GBM disease were enrolled in this study. (biomedsearch.com)
  • There are substances known to occur in patients with cns disease above the prepregnancy rate, and blood glucose, urine output, drug peak, and trough concentration range dose related and include nausea, vomiting, and hypersensitivity reactions with patch c. (roanokechowan.edu)
  • Treatment with immunosuppressants is required to induce remission of disease activity, after which patients are transitioned to more gentle maintenance immunosuppression. (medlink.com)
  • The RAVE trial also showed superiority of rituximab for patients presenting with a severe disease relapse. (medlink.com)
  • Moreover, only IgG1 among the four subclasses (BindingSite, Birmingham, UK) and only lambda light chains were detected in the glomerular capillary walls (Figures 2 and 3). (thefreelibrary.com)
  • Abrahamson DR: Structure and development of the glomerular capillary wall and basement membrane. (springer.com)
  • Aumailley M, Timpl R: Attachment of cells to basement membrane collagen type IV. (springer.com)
  • Brazel D, Pollner R, Oberbäumer I, Kühn K: Human basement membrane collagen (type IV): The amino acid sequence of the α2(IV) chain and its comparison with the α1(IV) chain reveals deletions in the α1(IV) chain. (springer.com)
  • Butkowski RJ, Langeveld JPM, Wieslander J, Hamilton J, Hudson BG: Localization of the Goodpasture epitope to a novel chain of basement membrane collagen. (springer.com)
  • Butkowski RJ, Wieslander J, Kleppel M, Michael AF, Fish AJ: Basement membrane collagen in the kidney: Regional localization of novel chains related to collagen IV. (springer.com)
  • The treatment with corticosteroids plus cyclophosphamide was found not to improve the renal outcome of disease (p = 0.73). (ovid.com)
  • Severe GPA or MPA disease that would conventionally be treated with cyclophosphamide. (clinicaltrials.gov)
  • Cyclophosphamide is used to treat cancers and autoimmune diseases. (wikipedia.org)
  • Cyclophosphamide is also used to treat minimal change disease, severe rheumatoid arthritis, granulomatosis with polyangiitis, Goodpasture syndrome and multiple sclerosis. (wikipedia.org)
  • We present a rare case of anti-GBM disease mediated by IgA autoantibody. (kbco.hr)
  • Here we describe a case of anti-GBM disease affecting the kidney and eye. (imedpub.com)
  • Anti-GBM disease used to be known as Goodpasture syndrome. (medlineplus.gov)
  • This test is used to diagnose certain kidney diseases, such as Goodpasture syndrome and anti-glomerular basement membrane disease. (medlineplus.gov)
  • We report a case of anti-glomerular basement membrane (GBM) disease in association with human leucocyte antigen (HLA) DRB1 15:01. (scienceopen.com)
  • Human lymphocyte antigen (HLA) associations are recognized for many autoimmune diseases, but the mechanisms are not clear. (scienceopen.com)
  • Therefore, it may be possible to directly analyze interactions between the antigen and HLA molecules associated with the disease, and to examine influences on antigen presentation to T cells. (scienceopen.com)
  • Comparison of the sequences of the positively and negatively associated alleles showed that polymorphic residues in the second peptide binding region of the HLA Class II antigen binding groove segregated with disease. (scienceopen.com)
  • Human leukocyte antigen (HLA) alleles are associated with many autoimmune diseases, including anti-glomerular basement membrane (GBM) disease. (cdc.gov)
  • Susceptibility and protection against human autoimmune diseases, including type I diabetes, multiple sclerosis, and Goodpasture disease, is associated with particular human leukocyte antigen (HLA) alleles. (bireme.br)
  • We report a case of 33 year(s)-old man who was diagnosed as anti-glomerular basement membrane disease without pulmonary hemorrhage . (bvsalud.org)
  • Antibody titers correlated with progression to end stage renal disease, whereas a similar correlation was not observed with pulmonary hemorrhage. (renalandurologynews.com)
  • Though he recognized influenza as a factor in the pathogenesis, it would take decades before these findings were recognized as one of the earliest described autoimmune diseases. (renalandurologynews.com)
  • The glomerular basement membrane is a part of the kidneys that helps filter waste and extra fluid from the blood. (medlineplus.gov)
  • Here we show that expression of the tetraspanin CD9 increases markedly in PECs in mouse models of CGN and FSGS, and in kidneys from individuals diagnosed with these diseases. (nature.com)
  • Atypical Antiglomerular Basement Membrane Disease With IgG1-κ Staining Kidney International Reports Elsevier Inc, 2(1): 80-83, Jan 2017. (upenn.edu)
  • The severity and extent of glomerular damage focal (confined) or diffuse (widespread) determines how the disease is manifested. (healthcommunities.com)
  • Objective: To examine the long-term outcome of severe anti-GBM antibody disease. (elsevier.com)
  • Renal failure associated bone disease (renal osteodystrophy) may lead to an increased risk of osteoporosis or bone fracture with trauma. (medscape.com)
  • Glycoprotein iib iiia inhibitor versus bivalirudin monotherapy and up to eight times hour period as the hemostasis is controlled because the pbpc grafthis optimal relative to their mechanism of action and is usually used for gerd related if a patient who claims a history o dietary de ciency anemia, pregnancy, end stage renal disease crcl ml min. (roanokechowan.edu)