Progressive renal disease light chain deposition disease with renal chains. Medical search. Frequent questions

Hypercalcemia contributes to renal insufficiency by decreasing renal blood flow. (msdmanuals.com)
The term myeloma kidney or myeloma cast nephropathy generally refers to renal insufficiency caused by the tubulointerstitial damage that results. (msdmanuals.com)
Common manifestations include lytic lesions in bones that cause pain and/or fractures, renal insufficiency, hypercalcemia, anemia, and recurrent infections. (merckmanuals.com)

[ 2 ] The term has been associated with various glomerular abnormalities that are caused by the deposition of these monoclonal immunoglobulins (or their heavy-chain or light-chain subunits) and are broadly classified into 2 categories, organized or nonorganized, depending on the pattern of deposition. (medscape.com)

Smithline et al first used the term light-chain nephropathy in 1976 to describe a case of renal tubular dysfunction with light-chain proteinuria. (medscape.com)
The filtered light-chain proteins, reabsorbed by the proximal tubular cells via the tandem megalin/cubilin receptors, are catabolized by lysosomal enzymes. (medscape.com)
Metabolism (catabolism) of these filtered light-chain proteins depends on normal proximal tubular cell function, and damage to these cells can result in increased excretion of light-chain proteins in the urine. (medscape.com)
these light chains are filtered by glomeruli, are nephrotoxic, and, in their various forms (free, tubular casts, amyloid), can damage virtually all areas of the kidney parenchyma. (msdmanuals.com)
Overview of Tubulointerstitial Diseases Tubulointerstitial diseases are clinically heterogeneous disorders that share similar features of tubular and interstitial injury. (msdmanuals.com)
Fanconi Syndrome Fanconi syndrome consists of multiple defects in renal proximal tubular reabsorption, causing glucosuria, phosphaturia, generalized aminoaciduria, and bicarbonate wasting. (msdmanuals.com)

Laboratory workup revealed abnormal renal function with the rapid rise of creatinine from 1.04 mg/dL to 14.5 mg/dL within 3 days. (ijpmonline.org)
The physicochemical and conformational characteristics of the abnormal light chains are primarily responsible for the downstream events affecting the mesangial milieu. (heraldopenaccess.us)
Investigations performed in the last 20 years have provided detailed insights into how these abnormal light chains interact with the mesangium to produce glomerular injury. (heraldopenaccess.us)
Disorders characterized by abnormal proliferation of immunoglobulin-producing cells and abnormal proliferation of immunoglobulin monoclonal (M protein)represent part of the spectrum of disease due to the neoplastic behavior of the B lymphocyte series. (emedicodiary.com)

Multiple myeloma is a chronic, progressive and fatal malignant condition in which the fundamental abnormality is a neoplastic proliferation of plasma cells that infiltrate the bone marrow and often other body tissues. (emedicodiary.com)
It can be a primary manifestation of multiple myeloma or develop as a terminal complication during the disease. (lookformedical.com)

The term Bence Jones protein has been used to designate a urinary protein that leaves solution at approximately 56°C under certain conditions of pH and ionic strength and returns to the solution upon further heating to 100°C. The Bence Jones protein represents a homogeneous population of immunoglobulin light chains of either kappa type or lambda type and is the product of a presumed single clone of plasma cells. (medscape.com)
Light chains are divided into 2 major classes based on the amino acid sequence in the constant portion of the polypeptide chain and are designated as kappa and lambda. (medscape.com)
These are further divided into at least 10 subtypes (4 kappa and 6 lambda) based on the amino acid sequence in the variable region of the polypeptide chain. (medscape.com)
Individual immunoglobulins have either kappa or lambda light chains, but not both. (medscape.com)
Lambda light chains usually exist as dimers (44,000 d) and, therefore, are less likely to be filtered and appear in urine. (medscape.com)
At times, light chains of either kappa or lambda type may form tetramers (88,000 d), which are not filtered, and a patient may have light-chain proteinemia without light-chain proteinuria. (medscape.com)
AL fibrils are most often lambda light chains (four times more likely than kappa light chains). (renalfellow.org)
Of patients producing either IgG or IgA, 40% also have Bence Jones proteinuria, which is free monoclonal kappa ( κ ) or lambda ( λ ) light chains in the urine. (merckmanuals.com)
Positive lambda light chain stain in a glomerulus. (askhematologist.com)
Although healthy individuals have a preponderance of kappa free light chains (K/λ) = 2:1), the reverse is true in most patients with primary amyloidosis, as excess lambda light chains have a greater propensity to be amyloidogenic. (askhematologist.com)
Each Ig molecules have either 2 kappa or 2 lambda light chains. (emedicodiary.com)
There are two major types of light chains, kappa and lambda. (lookformedical.com)

Tubulointerstitial disease and glomerular damage are the most common types of renal damage. (msdmanuals.com)
The glomerulopathic light chains interact with mesangial cells where purported receptors regulate the downstream cellular mechanisms that will be activated and result in glomerular alterations. (heraldopenaccess.us)
Kidney disease usually presents as reduced glomerular filtration rate (GFR) and/or proteinuria which may progress to nephrotic syndrome. (renalfellow.org)

Diagnosis is by urine tests (sulfosalicylic acid test or protein electrophoresis) or renal biopsy. (msdmanuals.com)
A renal biopsy confirmed amyloidosis, with amyloid protein deposits in the tubules and blood vessels. (medscape.com)
In all these, light chains purified from the urine of patients with renal biopsy-proven conditions have been used. (heraldopenaccess.us)

Distinguishing characteristics - AL amyloidosis is the most prevalent type of amyloid disease in most parts of the United States (US). (renalfellow.org)

In-vitro , ex-vivo and in-vivo animal models have been created to study these disorders providing excellent platforms to elucidate pathogenesis and provide insightful information that can be translated to the practice of renal pathology, as the In-vitro and invivo platforms corroborate each other. (heraldopenaccess.us)
The polycystic kidney diseases are a group of genetically heterogeneous disorders and a leading cause of kidney failure. (basicmedicalkey.com)

The most characteristic histologic lesion of light chain deposition disease (LCDD) is nodular glomerulosclerosis, which must be distinguished from diabetic glomerulosclerosis by using electron microscopy. (medscape.com)
The glomerulopathies associated with monoclonal light chains are Light Chain Deposition Disease (LCDD) and AL (light chainrelated) amyloidosis, each creating completely different alterations in the mesangium. (heraldopenaccess.us)

Amyloidosis is caused by the deposition and aggregation of insoluble, misfolded B-pleated protein fibrils in tissue, causing progressive organ dysfunction. (medscape.com)
The two most common types of systemic acquired disease are wild-type transthyretin (ATTR) amyloidosis and monoclonal Ig light chain (AL) amyloidosis . (medscape.com)
AA amyloidosis is another form of acquired systemic disease that results from high levels of serum AA protein, an acute phase reactant associated with chronic inflammation. (medscape.com)
AA amyloidosis affects persons with chronic infections or autoinflammatory diseases and primarily involves the kidneys. (medscape.com)
Different light chains are responsible for two diseases with diametrically opposite mesangial alterations: Light chain deposition disease which results in the expansion of the mesangium due to accumulation of matrix proteins not present in the normal mesangium and AL (light chain-associated) amyloidosis where the native mesangial matrix is replaced by fibrils (amyloid). (heraldopenaccess.us)
This birefringence is the most widely available gold standard for diagnosis of amyloidosis, however neither light nor electron microscopy can distinguish different types of amyloidosis. (renalfellow.org)
The most common type of amyloidosis and used to be called primary amyloidosis results when light chains are produced in excess by clonal or frankly malignant plasma cells. (askhematologist.com)
Light-chain amyloidosis may also arise in association with non-Hodgkin's lymphoma and Waldenström's macroglobulinemia . (askhematologist.com)
Previously known as secondary amyloidosis , this condition is the result of another chronic infectious or inflammatory diseases such as rheumatoid arthritis, Crohn's disease, or ulcerative colitis. (askhematologist.com)
Though some types of amyloid deposits have been linked to Alzheimer's disease, the brain is rarely affected by amyloidosis that happens throughout the body. (askhematologist.com)

Kappa light chains usually exist as monomers (22,000 d) and are therefore small enough to be filtered through the glomerulus, but they may exist as dimers. (medscape.com)
The excess light chains are secreted into the extracellular fluid and readily pass through the glomerulus. (emedicodiary.com)

Plasma cells normally produce a slight excess of light chains that are either excreted or catabolized by the kidney, and only a minute amount of light-chain protein normally appears in the urine. (medscape.com)
Light-chain proteins appear in urine in high concentration either when the production of light-chain proteins is markedly increased or when the ability of the proximal tubules to reabsorb all the filtered protein is diminished. (medscape.com)
The presence of light-chain proteins in the urine is associated with a number of systemic diseases (see Etiology ). (medscape.com)
This process is exceedingly efficient, and only a minute amount of light-chain protein normally appears in the urine. (medscape.com)
He was referred to us with anuria, anorexia, and recurrent vomiting for the last 2 days from a peripheral health center where he was treated for gradually progressive body swelling and a decrease in urine output for the last 3 months. (ijpmonline.org)
Diagnosis typically requires demonstration of M-protein (sometimes present in urine and not serum but rarely absent entirely) and/or light-chain proteinuria, and excessive plasma cells in the bone marrow. (merckmanuals.com)
Rarely, patients have no M-protein in blood and urine, although the currently used serum free light chain assay now demonstrates monoclonal light chains in many of these formerly so-called nonsecretory patients. (merckmanuals.com)
It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. (lookformedical.com)

At least 36 proteins have been identified in humans, with 17 showing systemic involvement and the rest presenting as localized diseases. (medscape.com)

The β-pleated sheet formation of fibrils gives them the ability to bind Congo red dye (seen on light microscopy) in an organized, intercalated way that emits apple-green birefringence under polarized light. (renalfellow.org)
3. Congo red stain shows orange-colored positivity by regular microscopy and apple-green birefringence under polarized light. (renalfellow.org)
Pathologists now know that amyloid, using the Congo red stain introduced in the 1920s, looks pink with normal lighting and demonstrates apple-green birefringence under polarized light. (askhematologist.com)
Apple-green birefringence under polarized light. (askhematologist.com)

Light microscopy (hematoxylin and eosin stain at 25X power) showing nodular glomerulosclerosis (arrow) and thickening of the basement membrane. (medscape.com)
This can sometimes be seen by silver stain on light microscopy. (renalfellow.org)
The various types of amyloid are indistinguishable using light microscopy. (askhematologist.com)

Amyloidoses are a group of diseases resulting from deposition of amyloid, insoluble fibrils derived from various precursor proteins, into extracellular tissues. (renalfellow.org)

[3] stated that more than 60% of nephrectomy specimens had pathology in non-neoplastic renal parenchyma which is responsible for the deterioration of renal function disproportionate to nephron loss in the postoperative era. (ijpmonline.org)
Vascular pathology related to hypertension was most commonly encountered followed by diabetic kidney disease. (ijpmonline.org)

Mesangial injury represents a crucial event in the pathogenesis of light chain-associated glomerulopathies in patients with plasma cell dyscrasias. (heraldopenaccess.us)

Myeloma-related kidney disease is rarely caused by Ig heavy chains. (msdmanuals.com)
In myeloma, plasma cells produce monoclonal (M) Ig of a single heavy and light chain commonly referred to as a paraprotein. (emedicodiary.com)
Myeloma is a disease of middle and old age (50-70 years), male predominance. (emedicodiary.com)

Normally Ig molecule consists of 4 polypeptide chains 2 heavy chains and 2 light chains. (emedicodiary.com)
A biosynthetic precursor of collagen containing additional amino acid sequences at the amino-terminal and carboxyl-terminal ends of the polypeptide chains. (lookformedical.com)
Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. (lookformedical.com)

Pathogenesis - Clonal plasma cells make monoclonal light chains. (renalfellow.org)
Etiology and Pathogenesis ( Fig. 339-1 ) ADPKD is characterized by progressive formation of epithelial-lined cysts in the kidney. (basicmedicalkey.com)

Many genetic defects are linked to a higher chance of amyloid disease. (askhematologist.com)
Defects in the primary cilia are linked to a wide spectrum of human diseases, collectively termed ciliopathies. (basicmedicalkey.com)

The kidney is the major site of metabolism of light-chain proteins. (medscape.com)
Light chains saturate the reabsorptive capacity of the proximal tubule, reach the distal nephron, and combine with filtered proteins and Tamm-Horsfall mucoprotein (secreted by cells of the thick ascending limb of Henle) to form obstructive casts. (msdmanuals.com)
Approximately 85% of the monoclonal proteins in patients with plasma cell dyscrasia are associated with renal disease. (heraldopenaccess.us)
Normal light chains are small molecular weight proteins that are filtered through the peripheral capillary walls and are delivered to the proximal tubules where they are catabolized by endosomal activity in the apical portions of the tubules and their amino acids are then returned to the circulation. (heraldopenaccess.us)
FIGURE 339-1 Scheme of the primary cilium and cystic kidney disease proteins. (basicmedicalkey.com)
Topology of autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) proteins polycystin-1, polycystin-2, and fibrocystin/polyductin (FPC) are shown. (basicmedicalkey.com)
PC2 and FPC both interact with kinesin 2 (KIF 3A/B). Localization of disease proteins in the cilium, the transition zone, and the basal body is color coded. (basicmedicalkey.com)

A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. (lookformedical.com)

Typically presents with a slowly progressive subnephrotic proteinuria in non-Anglo-Saxon ethnic groups. (aan.org.au)

The findings that have been elucidated have paved the way for the design of novel therapeutic interventions aimed at stopping, slowing down or avoiding renal damage in patients with plasma cell dyscrasias. (heraldopenaccess.us)

renal Hypertension objective: New Hyde Park, NY: Schneider Children's prognosis. (siriuspixels.com)
Linkage and comparative mapping studies indicate that the transferred chromosome segment contains a number of candidate genes for hypertension, including genes encoding a brain dopamine receptor and a renal epithelial potassium channel. (jci.org)

In some patients there is involvement of more than one compartment, but the manifestations usually are centered in one of the renal compartments [1-5]. (heraldopenaccess.us)

Renal cell carcinoma (RCC) is the most common subtype of adult renal tumors, and its detection rate in the early stages has been increased in the dawn of advanced imaging modalities. (ijpmonline.org)
Primary renal tumors account for 2%-3% of all human malignancy, and the major subtype is renal cell carcinoma (RCC). (ijpmonline.org)

Light chains (molecular weight 22,000 d) are polypeptides that are synthesized by plasma cells and form part of immunoglobulins. (medscape.com)
Light chains (molecular weight 22,000 d) are polypeptides synthesized by plasma cells and assembled with heavy chains to form the various classes of immunoglobulins, for example, immunoglobulin G (IgG), immunoglobulin M (IgM), and immunoglobulin A (IgA). (medscape.com)

This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (lookformedical.com)

The repair of the damaged mesangium is difficult due to the absence of enough mesangial cells that can participate in the process and also the damage can be so extensive that the intrinsic processes that are available for repair (i.e. recruitment of stem cells from bone marrow and precursor stem cells in renal niches) cannot effectively carry out the recovery. (heraldopenaccess.us)

subunits: ill from Schneider Children's disease. (siriuspixels.com)

The most common abnormality is the production of excess of light chains over heavy chains. (emedicodiary.com)

This is in contrast to MM, in which kappa light chains are more predominant. (renalfellow.org)

In the context of significant weight loss, serum creatinine levels may overestimate eGFR, leading to a delayed diagnosis of chronic kidney disease (CKD) and late referral to a specialist. (medscape.com)

Without treatment, GFR may progressively decline and patients may develop end-stage kidney disease (ESKD). (renalfellow.org)
The autosomal dominant form of polycystic kidney disease (ADPKD) is the most common life-threatening monogenic disease, affecting 12 million people worldwide. (basicmedicalkey.com)
The autosomal recessive form of polycystic kidney disease (ARPKD) is rarer but affects the pediatric population. (basicmedicalkey.com)

Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule. (lookformedical.com)

Plasma cells are derived from B lymphocytes and produce immunoglobulin (Ig) which contains heavy and light chains. (emedicodiary.com)

It is a major constituent of bone (BONE AND BONES) and SKIN and consists of a heterotrimer of two alpha1(I) and one alpha2(I) chains. (lookformedical.com)
It consists of three identical alpha1(II) chains. (lookformedical.com)

Although cysts only occur in 5% of the tubules in the kidney, the enormous growth of these cysts ultimately leads to the loss of normal surrounding tissues and loss of renal function. (basicmedicalkey.com)
A fibrillar collagen consisting of three identical alpha1(III) chains that is widely distributed in many tissues containing COLLAGEN TYPE I. It is particularly abundant in BLOOD VESSELS and may play a role in tissues with elastic characteristics. (lookformedical.com)

A new mouse cardiac electrophysiology method was used to study mice harboring an alpha-myosin heavy chain Arg403Gln missense mutation (alpha-MHC403/+), which results in histological and hemodynamic abnormalities characteristic of familial hypertrophic cardiomyopathy (FHC) and sudden death of uncertain etiology during exercise. (jci.org)

No amyloid fibril deposition was observed in the pupil, lens, or vitreous. (bvsalud.org)

Here, we take the opportunity to report an unusual association of heavy chain deposition disease (HCDD) with clear cell subtypes of renal cell carcinoma in a 48-year-old male of Indian ethnicity. (ijpmonline.org)
To the best of our knowledge, this is one of the first reports demonstrating concurrent heavy chain deposition disease (HCDD) with clear cell subtypes of renal cell carcinoma in a 48- year-old male of Indian ethnicity. (ijpmonline.org)
Paraproteins containing only light chains lead to Bence Jones paraproteinemia, while the presence of only atypical heavy chains leads to heavy chain disease. (lookformedical.com)

Anatomy6

Organisms1

Diseases20

Chemicals and Drugs14

Analytical, Diagnostic and Therapeutic Techniques and Equipment8

Phenomena and Processes5

Information Science2

Insufficiency3

Depending on the pattern of deposition1

Tubular6

Abnormal4

Multiple myeloma2

Lambda12

Glomerular3

Biopsy3

Type of amyloid1

Disorders2

LCDD2

Amyloidosis10

Glomerulus2

Urine8

Systemic1

Apple-green birefringence under polarized light4

Microscopy3

Insoluble1

Pathology2

Glomerulopathies1

Myeloma3

Polypeptide chains3

Pathogenesis2

Defects2

Proteins7

Disproportionate1

Proteinuria1

Dyscrasias1

Hypertension2

Involvement1

Subtype2

Immunoglobulins2

Primary1

Precursor1

Subunits1

Excess1

Predominant1

Diagnosis1

Kidney disease3

Molecule1

Plasma1

Consists2

Tissues2

Etiology1

Vitreous1

Heavy3