A mild form of LIMITED SCLERODERMA, a multi-system disorder. Its features include symptoms of CALCINOSIS; RAYNAUD DISEASE; ESOPHAGEAL MOTILITY DISORDERS; sclerodactyly, and TELANGIECTASIS. When the defect in esophageal function is not prominent, it is known as CRST syndrome.
Permanent dilation of preexisting blood vessels (CAPILLARIES; ARTERIOLES; VENULES) creating small focal red lesions, most commonly in the skin or mucous membranes. It is characterized by the prominence of skin blood vessels, such as vascular spiders.
A characteristic symptom complex.
An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress.
A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. Lesions may be characterized as patches or plaques (morphea), bands (linear), or nodules.
INFLAMMATION of any ARTERIES.
A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.
FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cirrhosis involves the destruction of small intra-hepatic bile ducts and bile secretion. Secondary biliary cirrhosis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.
Hand-held tools or implements used by health professionals for the performance of surgical tasks.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
Disorders affecting the motor function of the UPPER ESOPHAGEAL SPHINCTER; LOWER ESOPHAGEAL SPHINCTER; the ESOPHAGUS body, or a combination of these parts. The failure of the sphincters to maintain a tonic pressure may result in gastric reflux of food and acid into the esophagus (GASTROESOPHAGEAL REFLUX). Other disorders include hypermotility (spastic disorders) and markedly increased amplitude in contraction (nutcracker esophagus).
A heterogeneous group of disorders, some hereditary, others acquired, characterized by abnormal structure or function of one or more of the elements of connective tissue, i.e., collagen, elastin, or the mucopolysaccharides.
Failure of a professional person, a physician or lawyer, to render proper services through reprehensible ignorance or negligence or through criminal intent, especially when injury or loss follows. (Random House Unabridged Dictionary, 2d ed)
Insurance against loss resulting from liability for injury or damage to the persons or property of others.
Accountability and responsibility to another, enforceable by civil or criminal sanctions.
The alterations of modes of medical practice, induced by the threat of liability, for the principal purposes of forestalling lawsuits by patients as well as providing good legal defense in the event that such lawsuits are instituted.
The clear constricted portion of the chromosome at which the chromatids are joined and by which the chromosome is attached to the spindle during cell division.
Fibrous cords of CONNECTIVE TISSUE that attach bones to each other and hold together the many types of joints in the body. Articular ligaments are strong, elastic, and allow movement in only specific directions, depending on the individual joint.
The immovable joint formed by the lateral surfaces of the SACRUM and ILIUM.
A nerve originating in the lumbar spinal cord (L2 to L4) and traveling through the lumbar plexus to the lower extremity. The obturator nerve provides motor innervation to the adductor muscles of the thigh and cutaneous sensory innervation of the inner thigh.
The largest of three bones that make up each half of the pelvic girdle.
Shiny, flexible bands of fibrous tissue connecting together articular extremities of bones. They are pliant, tough, and inextensile.
VERTEBRAE in the region of the lower BACK below the THORACIC VERTEBRAE and above the SACRAL VERTEBRAE.
Conditions characterized by pain involving an extremity or other body region, HYPERESTHESIA, and localized autonomic dysfunction following injury to soft tissue or nerve. The pain is usually associated with ERYTHEMA; SKIN TEMPERATURE changes, abnormal sudomotor activity (i.e., changes in sweating due to altered sympathetic innervation) or edema. The degree of pain and other manifestations is out of proportion to that expected from the inciting event. Two subtypes of this condition have been described: type I; (REFLEX SYMPATHETIC DYSTROPHY) and type II; (CAUSALGIA). (From Pain 1995 Oct;63(1):127-33)
Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life.
Messages between computer users via COMPUTER COMMUNICATION NETWORKS. This feature duplicates most of the features of paper mail, such as forwarding, multiple copies, and attachments of images and other file types, but with a speed advantage. The term also refers to an individual message sent in this way.
The two longitudinal ridges along the PRIMITIVE STREAK appearing near the end of GASTRULATION during development of nervous system (NEURULATION). The ridges are formed by folding of NEURAL PLATE. Between the ridges is a neural groove which deepens as the fold become elevated. When the folds meet at midline, the groove becomes a closed tube, the NEURAL TUBE.
The hollow, muscular organ that maintains the circulation of the blood.
Small double-stranded, non-protein coding RNAs, 21-25 nucleotides in length generated from single-stranded microRNA gene transcripts by the same RIBONUCLEASE III, Dicer, that produces small interfering RNAs (RNA, SMALL INTERFERING). They become part of the RNA-INDUCED SILENCING COMPLEX and repress the translation (TRANSLATION, GENETIC) of target RNA by binding to homologous 3'UTR region as an imperfect match. The small temporal RNAs (stRNAs), let-7 and lin-4, from C. elegans, are the first 2 miRNAs discovered, and are from a class of miRNAs involved in developmental timing.
A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability.
Component of dermatan sulfate. Differs in configuration from glucuronic acid only at the C-5 position.
A naturally occurring glycosaminoglycan found mostly in the skin and in connective tissue. It differs from CHONDROITIN SULFATE A (see CHONDROITIN SULFATES) by containing IDURONIC ACID in place of glucuronic acid, its epimer, at carbon atom 5. (from Merck, 12th ed)
A subclass of closely-related SOX transcription factors. Members of this subfamily have been implicated in regulating the differentiation of OLIGODENDROCYTES during neural crest formation and in CHONDROGENESIS.
Enzymes that catalyze the epimerization of chiral centers within carbohydrates or their derivatives. EC 5.1.3.
Congenital MEGACOLON resulting from the absence of ganglion cells (aganglionosis) in a distal segment of the LARGE INTESTINE. The aganglionic segment is permanently contracted thus causing dilatation proximal to it. In most cases, the aganglionic segment is within the RECTUM and SIGMOID COLON.
A subnormal level of BLOOD PLATELETS.
The number of PLATELETS per unit volume in a sample of venous BLOOD.

Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin-1, in patients with scleroderma and other connective tissue diseases. (1/32)

A duplication in the fibrillin-1 gene has been implicated as the cause of the tight skin 1 (tsk1) phenotype, an animal model of scleroderma or systemic sclerosis (SSc). In addition to the production of abnormal fibrillin-1 protein, the tsk1 mouse also produces autoantibodies to fibrillin-1. Among a population of Choctaw Native Americans with the highest prevalence of SSc yet described, a chromosome 15q haplotype containing the fibrillin-1 gene has been strongly associated with SSc. With a recombinant human fibrillin-1 protein, autoantibodies to fibrillin-1 were detected in the sera of Native American SSc patients that correlated significantly with disease. Abs to fibrillin-1 also were detected in sera from Japanese, Caucasian, and African-American SSc patients. Compared with other ethnic groups, Japanese and Native American SSc patients had significantly higher frequencies of anti-fibrillin-1 Abs. Sera from patients with diffuse SSc, calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasias syndrome and mixed connective tissue disease also had significantly higher frequencies of anti-fibrillin-1 Abs than sera from controls or patients with other non-SSc connective tissue diseases (lupus, rheumatoid arthritis, and Sjogren's syndrome). Ab specificity for fibrillin-1 was demonstrated by the lack of binding to a panel of other purified autoantigens. The results presented demonstrate for the first time the presence of high levels of anti-fibrillin-1 Abs in a significant portion of patients with SSc.  (+info)

Phlebosclerosis of the colon with positive anti-centromere antibody. (2/32)

A 56-year-old woman with symptoms of chronic bowel disease presented a peculiar calcification of the mesenteric vein of the ascending to transverse colon on barium enema study. The resected colon was hard and black. Histo-pathologic examinations demonstrated fibrous change of the colon with a calcified and hyaline-deposited mesenteric vein. No cell infiltration was observed. These findings were compatible with phlebosclerosis and also with systemic sclerosis. Positive anti-centromere antibody and Raynaud's phenomenon, hallmarks of a variant systemic sclerosis, the CREST syndrome were observed. We therefore speculated that the pathogenesis of the phlebosclerosis of the colon is related to the CREST syndrome.  (+info)

The mammalian centromere: structural domains and the attenuation of chromatin modeling. (3/32)

The centromere-kinetochore complex can be divided into distinct domains based on structure and function. Previous work has used CREST auto-antibodies with various microscopic techniques to map the locations of proteins within the centromere-kinetochore complex and to analyze the maturation of prekinetochores before mitosis. Here we have focused on the centromere-specific histone Centromere Protein (CENP)-A and its spatial relationship to other histones and histone modifications found in condensed chromatin. We demonstrate that the phosphorylation of histone H3 is essentially excluded from a specific region of centromeric chromatin, defined by the presence of CENP-A. Interspersion of CENP-B with phosphorylated H3 in the inner centromere indicates that the exclusion of H3 modification is not a general property of alpha-satellite DNA. We also demonstrate that these regions are functionally distinct by fragmenting mitotic chromatin into motile centromere-kinetochore fragments that contain CENP-A with little or no phosphorylated H3 and nonmotile fragments that contain exclusively phosphorylated H3. The sequence of CENP-A diverges from H3 in a number of key residues involved in chromosome condensation and in transcription, potentially allowing a more specialized chromatin structure within centromeric heterochromatin, on which kinetochore plates may nucleate and mature. This specialized centromere subdomain would be predicted to have a very tight and static nucleosome structure as a result of the absence of H3 phosphorylation and acetylation.  (+info)

Clinical, serological and genetic study in patients with CREST syndrome. (4/32)

OBJECTIVE: To assess the clinical, serological and genetic features of Japanese patients with CREST syndrome. PATIENTS AND METHODS: Clinical features, autoantibodies and human histocompatibility leukocyte antigen (HLA) typing were studied in thirty patients with CREST syndrome, including 29 females and one male, with a mean age of 59.0 years (ranging from 40 to 76 years). RESULTS: Interstitial pneumonia on chest X-ray and renal involvement were rare. Mitral regurgitation and tricuspid regurgitation were present in 56.7% and 76.7%, respectively. Sjoren's syndrome (SS) and primary biliary cirrhosis (PBC) were highly associated, however the positivity of the marker antibodies to those syndromes, such as anti-SSA, anti-SSB, anti-mitochondrial (AMA) and anti-smooth muscle autoantibodies were less frequent than that of primary SS and PBC without the other autoimmune diseases. The histological findings of PBC were all early stages in Scheuer's classification. HLA-Cw6 were associated with CREST-PBC overlap syndrome (p<0.05). However the HLA antigen was not correlated with CREST syndrome, and the frequency of HLA-DR2 between CREST syndrome with or without PBC was significantly different (p<0.01). CONCLUSION: It was suggested that there was a genetic difference between CREST syndrome alone and CREST-PBC overlap syndrome and there were differences (the positivity of AMA and the severity of bile duct lesion) between PBC and CREST-PBC overlap syndrome.  (+info)

Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome? (5/32)

OBJECTIVE: We report the cases of two patients with the complete CREST variant (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) of systemic sclerosis (SSc) who developed autoimmune hepatitis. RESULTS: Our findings suggest that autoimmune hepatitis can be considered to be one of the liver manifestations associated with SSc. Our data also indicate that, because liver involvement may precede skin manifestations, evaluation for SSc is appropriate when autoimmune hepatitis is noted, and that the evaluation should include clinical examination, testing for antinuclear antibodies (especially for anticentromere antibodies) and nailfold capillaroscopy. CONCLUSIONS: From a practical point of view, our two cases emphasize that suspicion of autoimmune hepatitis in SSc patients presenting with cytolytic hepatitis will help to achieve both accurate diagnosis and optimal management.  (+info)

Progressive interstitial renal fibrosis due to Chinese herbs in a patient with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome. (6/32)

A 58-year-old woman with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome presented with slowly progressive renal dysfunction. She was normotensive with normal plasma renin activity and lacking symptoms of vasculitis. Mild proteinuria was of tubular origin, but serological tests and an absence of sicca symptoms excluded the possibility of Sjogren's syndrome. Light microscopic study of renal biopsy showed interstitial fibrosis with ectasia and degeneration of proximal tubule and lymphocyte infiltration. There were no remarkable changes in the glomeruli. Chromatographic analysis of the Chinese herbs regimen that she had been taking for several years demonstrated aristolochic acid. She was diagnosed as Chinese herbs nephropathy. Therapy with oral prednisolone was markedly effective in improving renal function and anemia. To our knowledge, this is the first report of Chinese herbs nephropathy complicating connective tissue disease. It is important to consider the possibility of Chinese herbs nephropathy when patients treated with Chinese herbs develop renal dysfunction.  (+info)

Induction of kinetochore-positive and kinetochore-negative micronuclei in CHO cells by ELF magnetic fields and/or X-rays. (7/32)

To test the genotoxic effects of extremely low frequency (ELF) magnetic fields, the induction of micronuclei by exposure to ELF magnetic fields and/or X-rays was investigated in cultured Chinese hamster ovary (CHO) cells, using the cytokinesis block method. Micronuclei derived from acentric fragments or from whole chromosomes were evaluated by immunofluorescent staining using anti-kinetochore antibodies from the serum of scleroderma (CREST syndrome) patients. A 60 Hz ELF magnetic field at 5 mT field strength was applied, either before or after 1 Gy X-ray irradiation or without additional X-ray irradiation. No statistically significant difference in the frequency of micronuclei in CHO cells was observed between a sham exposure (no exposure to an ELF magnetic field) and a 24 h ELF magnetic field exposure. Exposure to an ELF magnetic field for 24 h before X-ray irradiation or for 18 h after X-ray irradiation did not affect the frequency of X-ray-induced micronuclei. However, the number of kinetochore-positive micronuclei was significantly increased in the cells subjected to X-ray irradiation followed by ELF magnetic field exposure, but not in the cells treated with ELF magnetic field exposure before X-ray irradiation, compared with exposure to X-rays alone. The number of spontaneous kinetochore-positive and kinetochore-negative micronuclei was not affected by exposure to an ELF magnetic field alone. Our data suggest that exposure to an ELF magnetic field has no effect on the number of spontaneous and X-ray-induced micronuclei. However, ELF magnetic field exposure after but not before X-ray irradiation may somehow accelerate X-ray-induced lagging of whole chromosomes (or centric fragments) in CHO cells.  (+info)

Human ninein is a centrosomal autoantigen recognized by CREST patient sera and plays a regulatory role in microtubule nucleation. (8/32)

Centrosome is the major microtubule organizing center in mammalian cells that plays a critical role in a variety of cellular events by the microtubule arrays emanating from it. Despite its significance, the molecular mechanisms underlying the structure and function of the centrosome are still not clear. Herein we describe the identification of three isotypes of human ninein by expression library screening with autoimmune sera from CREST patients. All three ninein isotypes exhibit centrosomal localization throughout the cell cycle when GFP-tagged fusion proteins are expressed transiently in mammalian cells. Construction of serial deletions of GFP-tagged ninein reveals that a stretch of three leucine zippers with a flanking sequence is required and sufficient for centrosomal targeting. Overexpression of ninein results in mislocalization of gamma-tubulin, recruiting it to ectopic (noncentrosomal) ninein-containing sites which are not active in nucleating microtubules. In these cells, nucleation of microtubules from the centrosome is also inhibited. These results thus suggest a regulatory role for ninein in microtubule nucleation.  (+info)

Gastric antral vascular ectasia (GAVE) is a rare, but serious, cause of upper gastrointestinal bleeding (4% of all gastric hemorrhages). The endoscopic findings are characteristic: linear images, oriented radially and converging on the pylorus (watermelon stomach1,4).. This condition is associated with underlying chronic diseases, mostly with liver cirrhosis. In autoimmune diseases, it is related to the presence of Raynauds phenomenon, and is the form most widely reported of the limited subtype of scleroderma (calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST syndrome]).5,6 The largest series of patients with GAVE includes 45 individuals, 62% of whom also had a connective tissue disease, predominantly with Raynauds phenomenon (31%) and sclerodactyly (20%).7. The etiology and pathogenesis of GAVE are unknown; one hypothesis is that the histological changes, especially in the lamina propria, are due to a fibromuscular proliferation, vascular ...
Gastric antral vascular ectasia (GAVE) is a rare, but serious, cause of upper gastrointestinal bleeding (4% of all gastric hemorrhages). The endoscopic findings are characteristic: linear images, oriented radially and converging on the pylorus (watermelon stomach1,4).. This condition is associated with underlying chronic diseases, mostly with liver cirrhosis. In autoimmune diseases, it is related to the presence of Raynauds phenomenon, and is the form most widely reported of the limited subtype of scleroderma (calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST syndrome]).5,6 The largest series of patients with GAVE includes 45 individuals, 62% of whom also had a connective tissue disease, predominantly with Raynauds phenomenon (31%) and sclerodactyly (20%).7. The etiology and pathogenesis of GAVE are unknown; one hypothesis is that the histological changes, especially in the lamina propria, are due to a fibromuscular proliferation, vascular ...
We report a case of 62-year-old Tunisian woman with a 10-year history of a CREST syndrome (systemic sclerosis meeting the criteria of the CREST syndrome of the 1980 American College of Rheumatology classification for raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia). Her daughter is treated in neurology for myasthenia gravis. Our patient presents a 5-month history of increasing inflammatory right thumb pain concomitant with the discovery of a subcutaneous hard mass (A). Radiographs of her thumb showed a sub-cutaneous calcification (as another part of the CREST syndrome criteria) (B). X-rays of hands found an acro-osteolysis (C). In the CREST syndrome, the calcific deposits can be subclinical. But, when symptomatic, it becomes painful, tender and an inflammatory reaction can occur facing the calcinosis.
CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc) is a multisystem connective tissue disorder. The acronym CREST refers to the five main features: calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. It is associated with detectable antibodies against centromeres (a component of the cell nucleus), and usually spares the kidneys (a feature more common in the related condition systemic scleroderma). If the lungs are involved, it is usually in the form of pulmonary arterial hypertension. CREST causes thickening and tightening of the skin with deposition of calcific nodules (calcinosis). Raynauds phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induce an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transitions in skin ...
Crest syndrome is an acronym for Calcinosis; Reynaudssyndrome; Esophagealdysmotility; Sclerodactyly and Telangiectasia. It is also known as Limited
The paper outlines the experience with minimally invasive surgical technologies for esophageal injury in the presence of systemic connective tissue disease. The clinical example clearly demonstrates the importance of timely diagnosis of esophageal stricture in CREST syndrome and suggests that it is necessary to treat these difficult-to-treat patients at large multiprofile hospitals ...
CREST syndrome is related to antibodies that combat centromeres (regions of DNA chromosomes) like ones in the kidneys and lungs. Characterized as an
Tips to help with your thrombocytopenia: Crest Syndrome Thrombocytopenia. My thrombocytopenia, Online resources for thrombocytopenia.
The answer to yesterdays mystery is … limited scleroderma (a.k.a. CREST syndrome)! Great job Priscilla, Kmu, American Girl Mommy, Erin, Rich S, guest, OB1, Phung, Shelly Langlois, mabel, vor120806, Kimberly Helton, CB, Kelly, ColorMaven, Jerry Skrocki, Amanda, Michelle, Tina M., Angelita, ER SEC, Mary, todd dombrowski, JP3, kim labounty, Dazed & Confused, JB, and PJ.. Ill give partial credit to those who named one component of CREST syndrome, Raynauds phenomenon: D2, Patti Ann, Carol, MG, Gracie287, GetAClue, Zach A., Tara Naz, Robert Jones, the other Naomi, Shannon Vincent, Martha, sallad, Michele Liguori, Veronica, Deborah Cote, JP, and GG.. CREST syndrome, or limited cutaneous systemic scleroderma, is an autoimmune disease that causes scarring of different tissues in the body, most notably the skin. The cause of the disease is unknown, but it is likely some combination of genetic factors and environmental triggers. First, inflammatory white blood cells infiltrate the skin and ...
The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis, scleroderma. CREST is an acronym for the clinical features that are seen in a patient with this disease. The C stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The R stands for Raynauds phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The E represents esophageal dysmotility, which can cause difficulty in swallowing. The S is for sclerodactyly, tightening of the skin causing the fingers to bend. Finally, the letter T is for telangiectasia, dilated vessels on the skin of the fingers, face, or inside of the mouth. Usually only 2 of the 5 symptoms of the CREST syndrome is necessary to be diagnosed with the disease. ...
CREST syndrome A less severe form of scleroderma is called CREST.. The CREST syndrome represents symptoms including calcium skin deposits; Raynauds phenomenon (a condition in which the blood vessels of the fingers and toes go into spasm when triggered by factors such as cold, stress, or illness; the result is cold, painful, or numb fingers and toes which in severe cases may become gangrenous); esophageal dysfunction (problems with the esophagus, the tube between the mouth and the stomach); sclerodactyly (skin damage on fingers); and telangiectasia (spider veins), limits skin damage to the fingers.. However, this disease, when coupled with pulmonary hypertension (elevated blood pressures within the lungs), can lead to heart and respiratory failure.. ...
Without Raynauds you cannot diagnose Scleroderma. I think it is completely unprofessional for your doctor to say you have this, or even suspect it - since hes not a rheumatologist. If you dont have Raynauds you dont have this... Something like 98% of those with Scleroderma have very severe Raynauds. Multiple people on this board have Raynauds, but were talking severe Raynauds where your fingers turn mutliple colors and you literally have to put them under warm water to get them back to normal ...
Morphology. Virtually all organs are involved.. 1. Skin. Bundles of the collagen bind the skin to the underlying tissue.. Changes take place in three stages.. Stage I: There is edema which is non-pitting and microscopically there is edema, perivascular lymphocytic infiltrate, capillaries and small arteries are thickened, and there is collagen deposition.. Stage II: Due to an increase in collagen formation, wrinkles disappear. The epidermis becomes thin.. Stage III: This is the atrophic stage where claw-like hand, the face is drawn and mask. Microscopically there is atrophy of the epidermis and increase collagen. Calcification may be seen.. In the late stages due to loss of blood supply ulceration may take place.. Skin involvement may be a part of CREST syndrome. Where:. C = Calcinosis.. R = Raynauds phenomenon.. E = Esophageal involvement.. S = sclerodactyly (localized sclerosis of the finger).. T = Telangiectasia.. 2. Gastrointestinal. Gastrointestinal involvement is seen ,50% of the cases. ...
Main page of ISN Scleroderma Symptom Photo Repository. Pictures include images of amputations, calcinosis, hand contractures, CREST syndrome, gangrene, Raynauds, sclerodactyly, and telangeiectasia.
In this video from Scleroforums, learn more about Scleroderma and Sclerodactyly from Amanda Thorpe for the nonprofit International Scleroderma Network.
The Raynauds phenomenon is named after the French doctor who first wrote about in the 1850. There is Raynauds phenomenon or simply Raynauds is a disorder to the characterized by decreased blood flow usually to the fingers and less frequently to the ears, toes, nipples, knees, or nose. The Raynauds can occur alone or may occur with other diseases. The diseases most frequently by the associated with Raynauds are autoimmune or connective tissue diseases, among others, such as the followings are systemic lupus erythematous and scleroderma and CREST syndrome, Buergers disease, polymyositis, Sjogrens syndrome, occlusive vascular disease, rheumatoid arthritis, cryoglobulinemia. They are one theory links blood disorders to the characterized by increased platelets or red blood cells that may increase the blood thickness. The phenomenon of heart disease will be varying from one person to another person in the real world.. Risk Factor:. The risk of factor is anything that may be increases a persons ...
By: Connie ODonnell How Scleroderma and Paraplegia can have a bright spot in the lives of one couple.. I was not diagnosed with Scleroderma Crest Syndrome until I was into my 50s, but suffered from Raynauds and painful finger ulcers for many years. There isnt a photo of me that I am not sporting bandaids on my fingers. Finally these drove me to a doctor, then a wound care facility. I suffered through much painful debriding until finally I had to have my index fingers amputated. I am an artist and initially this procedure was devastating. However, over the years, my fingers have stiffened and become bent, but I have managed to adapt. I am now almost 65 and still draw and paint, and can diaper a grand child with some difficulty. Six years ago my husband suffered a spinal cord injury and is paralyzed just below his arms. We consider ourselves lucky. He is the hands and I am the legs in our relationship, so together we are a whole.....or somewhat close to one.. ...
Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs.[2][6][8] The disease can be either localized to the skin or involve other organs, as well.[2] Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure.[1] One form of the condition, known as CREST syndrome, classically results in calcium deposits, Raynauds syndrome, esophageal problems, thickening of the skin of the fingers and toes, and areas of small, dilated blood vessels.[1] The cause is unknown, but it may be due to an abnormal immune response.[2] Risk factors include family history, certain genetic factors, and exposure to silica.[3][4][5] The underlying mechanism involves the abnormal growth of connective tissue, which is believed to be the result of the immune system attacking healthy tissues.[6] Diagnosis is based on symptoms, supported by a skin biopsy or blood tests.[6] While no ...
Extremely low-frequency (ELF) magnetic fields have previously been shown to affect conformation of chromatin, cell proliferation, and calcium metabolism. Possible mutagenic and carcinogenic effects of ELF have also been discussed and tested. In this study, intrachromosomal recombination in the hprt gene after exposure to ELF magnetic field was investigated using the SPD8 recombination assay. SPD8 cells, derived from V79 Chinese hamster cells were exposed to ELF at a specific combination of static and ELF magnetic fields, that has been proven to have effects on chromatin conformation in several cell types. The genotoxic agent camptothecin (CPT) was used either as a positive control or simultaneously with ELF. We also analysed the effect of ELF and CPT on chromatin conformation with the anomalous viscosity time dependence (AVTD) technique, cell growth kinetics, and cell survival with clonogenic assay. DNA fragmentation was analysed by pulsed field gel electrophoresis (PFGE). ELF did not induce ...
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Learn all about sclerodactyly, a condition that can cause the hands to take on a claw shape. This article looks at the symptoms, causes, and treatment.
Gene Information Proteins of the large Rab GTPase family (see RAB1A; MIM 179508) have regulatory roles in the formation targeting and fusion of intracellular transport vesicles. RAB11FIP3 is one of many proteins that interact with and regulate Rab GTPases (Hales et al. 2001 [PubMed 11495908]).[supplied by OMIM Mar 2008]. ...
With local nerve compression, calcinosis cutis may cause neuropathic pain, and the presence of adjacent nerves should be a consideration in any excision of a deposition. Valenzuela, A, Chung L., Calcinosis. pathophysiology and management. Curr Opin Rheumatol. vol. 27. 2015 Nov. pp. 542-8. (The authors present an overview of recent studies regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis.) Dima, A, Balanescu, P, Baicus, C. Pharmacological treatment in calcinosis cutis associated with connective-tissue diseases. Rom J Intern Med. vol. 52. 2014. pp. 55-67. (A review of pharmacological treatment options for calcinosis in patients with ACTD.) Del Barrio-Díaz, P, Moll-Manzur, C, Álvarez-Veliz, S, Vera-Kellet, C. Topical sodium metabisulfite for the treatment of calcinosis cutis: A promising new therapy. Br J Dermatol. 2016 Jan 22. (Case series of four patients with calcinosis cutis, secondary to dermatomyositis, ...
Raynaud phenomenon is known to occur in around 10% of the healthy female population and in around 5% of men. In the vast majority of cases, the blood vessels show no evidence of damage. In this case - so called Primary Raynaud phenomenon - the issue appears to be exaggerated spasm of healthy blood vessels.. Raynaud phenomenon is also the most common first symptom of scleroderma and is an important feature in other rheumatologic illnesses such as lupus. In this situation, the blood vessels are damaged and the opening for blood flow is much reduced. Raynaud episodes in scleroderma may reflect normal blood vessel constriction in response to cold superimposed on the narrow plumbing.. Blood flow to the fingers in normal health is around 40 times as much as is needed for oxygen supply and tissue nutrition. When the body is hot, blood flow increases to the hands so as to dissipate heat. This is much like a dog panting on a hot day. Conversely, when the body is cold, it is normal to reduce blood flow to ...
The symptoms of Raynauds phenomenon, arthralgia, and dysphagia point toward the diagnosis of scleroderma. Scleroderma, or systemic sclerosis, is characterized by a systemic vasculopathy of small and medium-sized vessels, excessive collagen deposition in tissues, and an abnormal immune system. It is an uncommon multisystem disease affecting women more often than men. There are two variants of scleroderma ;a relatively benign type called the CREST syndrome C-calcinosis.R- raynaud phen. E- esophagal dysmotility.S-sclerodactyly.T-talangiectasia and a more severe, diffuse disease ...
CALCINOSIS CUTIS TREATMENT PDF - BACKGROUND: Dystrophic calcinosis cutis is a common manifestation in connective tissue diseases, but theres still no consensus on treatment. OBJECTIVES.
Calcinosis cutis is a descriptive term for the deposition of insoluble calcium salts in the cutaneous and subcutaneous tissue. Based upon the etiology of calcium deposition, there are five subtypes of calcinosis cutis: dystrophic, metastatic, idiopat
Antinuclear antibodies are associated with rheumatic diseases including Systemic Lupus Erythematous (SLE), mixed connective tissue disease, Sjogrens syndrome, scleroderma, polymyositis, CREST syndrome, and neurologic SLE.
The Waardenburg syndromes (WS) are rare genetic disorders described classically as neural crest syndromes and characterized by deafness and pigmentation abnormalities. WS type IV (WS4) is attributed to mutations in the SOX10 gene, which regulates pigment cell development. Here, Kirsten Dutton and colleagues study otic vesicle development using a zebrafish model of WS4, the colourless/sox10 mutant. WS4 patients and mutant fish display a similar spectrum of abnormalities in the ear, pigment cells and enteric neurons. Additionally, the researchers found that only a few neural crest cells contribute to the developing zebrafish ear, and therefore are unlikely to be wholly responsible for the resulting auditory deficits. Their work helps explain the origin of inner ear deficits that lead to hearing loss and vestibular problems in WS4 patients.. Page 68. ...
Raynaud Syndrome Symptom Checker: Possible causes include Raynaud Disease. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.
Qué es el fenómeno de Raynaud? El fenómeno de Raynaud es una enfermedad que afecta los vasos sanguíneos. Si usted tiene el fenómeno de Raynaud, tiene periodos llamados episodios cuando el cuerpo no envía suficiente sangre a las manos y los pies. Los episodios generalmente ocurren cuando la persona tiene frío o se siente estresada. Durante un episodio, los dedos de las manos y los pies pueden sentirse muy fríos o entumecidos. El fenómeno de Raynaud también se conoce como la enfermedad de Raynaud o el síndrome de Raynaud ...
Qué es el fenómeno de Raynaud? El fenómeno de Raynaud es una enfermedad que afecta los vasos sanguíneos. Si usted tiene el fenómeno de Raynaud, tiene periodos llamados episodios cuando el cuerpo no envía suficiente sangre a las manos y los pies. Los episodios generalmente ocurren cuando la persona tiene frío o se siente estresada. Durante un episodio, los dedos de las manos y los pies pueden sentirse muy fríos o entumecidos. El fenómeno de Raynaud también se conoce como la enfermedad de Raynaud o el síndrome de Raynaud ...
Metastatic calcinosis cutis information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis.
Diagnosis and conservative treatment of raynauds syndrome (Raynaud disease ) (costs for program #151501) ✔ University Hospital Münster ✔ Department of Gerneral Internal Medicine, Nephrology, Hypertension Diseases and Rheumatology (Medical Department D) ✔ BookingHealth.com
Diagnosis of raynauds syndrome (Raynaud disease ) (costs for program #242505) ✔ University Hospital Hamburg-Eppendorf ✔ Department of Pediatrics ✔ BookingHealth.com
Definition of Raynaud's disease in the Legal Dictionary - by Free online English dictionary and encyclopedia. What is Raynaud's disease? Meaning of Raynaud's disease as a legal term. What does Raynaud's disease mean in law?
Raynaud and Seiver Disease affect the feet, but in different ways. Raynauds Disease causes spasms, while Seivers Disease can mimic Achilles tendonitis.
Q: While waiting at the bus stop, my 13-year-old daughters fingers got cold and turned blue, with a clear line between the blue and normal parts. I am going to take her to the pediatrician next week to have her checked, but I wanted to know what this could be.A: The description you give sounds a lot like pediatric Raynaud phenomenon (RP), a transient constriction of the small arteries that can compromise the blood flow to the fingers (less commonly the toes or even the ears), typically
Q: While waiting at the bus stop, my 13-year-old daughters fingers got cold and turned blue, with a clear line between the blue and normal parts. I am going to take her to the pediatrician next week to have her checked, but I wanted to know what this could be.A: The description you give sounds a lot like pediatric Raynaud phenomenon (RP), a transient constriction of the small arteries that can compromise the blood flow to the fingers (less commonly the toes or even the ears), typically
Q: While waiting at the bus stop, my 13-year-old daughters fingers got cold and turned blue, with a clear line between the blue and normal parts. I am going to take her to the pediatrician next week to have her checked, but I wanted to know what this could be.A: The description you give sounds a lot like pediatric Raynaud phenomenon (RP), a transient constriction of the small arteries that can compromise the blood flow to the fingers (less commonly the toes or even the ears), typically
This page includes the following topics and synonyms: Raynauds Phenomenon, Raynauds Syndrome, Raynaud Disease, Raynaud Phenomenon, Raynaud Syndrome.
A 6-month-old boy was referred to our department for the presence of diffuse multiple whitish, pseudomilia-like papules on the skin of the extremities since birth. The patient was well-appearing, and afebrile, with normal vital signs. The examination revealed multiple, yellow-white, firm, small, subcutaneous nodules and papules (Figure 1, A-C). The lesions were present only on the extremities, arms and legs. The lesions were in different stages, with a precise evolution process toward extrusion of a whitish material, erosion and healing (Figure 1, A ...
Q: While waiting at the bus stop, my 13-year-old daughters fingers got cold and turned blue, with a clear line between the blue and normal parts. I am going to take her to the pediatrician next week to have her checked, but I wanted to know what this could be.. Q: While waiting at the bus stop, my 13-year-old daughters fingers got cold and turned blue, with a clear line between the blue and normal parts. I am going to take her to the pediatrician next week to have her checked, but I wanted to know what this could be ...
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies. ...
Mr. Raynaud, a recent immigrant from France, finds employment as a high school literature teacher in the U.S. One day, a female student bursts into tears in the middle of class. Raynaud walks over
Raynaud's phenomenon, a disorder that affects the blood vessels in the fingers, toes, ears, and nose, can occur as a primary or secondary condition.
Laborator Synevo. Informaţii generale şi recomandari pentru determinarea profilului miozita. Miopatiile inflamatorii idiopatice reprezinta un grup de afectiuni caracterizate prin slabiciunea musculaturii proximale a membrelor, atrofie musculara, semne biochimice şi electromiografice de leziune musculara, anomalii histologice, modificari cutanate caracteristice. Apar cu o incidenta de 0,1-1/100 000 locuitori/an şi sunt mai frecvente la femei. Din acest grup fac parte: polimiozita, dermatomiozita, miozita cu corpi de incluziune, miozita asociată cu neoplazie (sindrom paraneoplazic) şi miozita asociată cu alte boli ale tesutului conjunctiv6;7.. Din punct de vedere histologic miozita poate fi diferenţiata in miozita interstitiala fara distructia fibrei musculare, miozita focala cu infiltrat inflamator si leziuni ale fibrei musculare si miozita difuza.. Polimiozita (PM) se caracterizează clinic prin febra, slabiciune musculara, artralgii, posibil sindrom Raynaud.. In dermatomiozita (DM) ...
Berry, T., Luther W, Bhatnagar N, Jamin Y, Poon E, Sanda T, Pei D, Sharma B, Vetharoy WR, Hallsworth A, Ahmad Z, Barker K, Moreau L, Webber H, Wang W, Liu Q, Perez-Atayde A, Rodig S, Cheung NK, Raynaud F, Hallberg B, Robinson SP, Gray NS, Pearson AD, Eccles SA, Chesler L, George RE. The ALK(F1174L) Mutation Potentiates the Oncogenic Activity of MYCN in Neuroblastoma. Cancer Cell. 2012 Jul 10;22(1):117-30. ...
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Looking for online definition of Raynaud syndrome in the Medical Dictionary? Raynaud syndrome explanation free. What is Raynaud syndrome? Meaning of Raynaud syndrome medical term. What does Raynaud syndrome mean?
Looking for online definition of Raynaud in the Medical Dictionary? Raynaud explanation free. What is Raynaud? Meaning of Raynaud medical term. What does Raynaud mean?
Considering a prevalence of 5-10% in the general population, there is still a place and probably a need for the development of new treatment concepts. Ginkgo biloba has shown to have anti-oxidative and anti-platelet activities. In a small placebo controlled trial in patients with the Raynaud´s phenomenon promising results for the Ginkgo biloba extract were shown. EGb 761® is known to be safe and well tolerated. Based on the above considerations, EGb 761® may be an effective treatment for Raynaud´s phenomenon.. Aim: To determine the efficacy and safety of EGb 761® in patients with Raynaud´s phenomenon on the frequency, duration, and severity of vasospastic attacks compared to placebo. ...
Scleroderma is a disease of the connective tissue featuring prominent skin thickening. Scleroderma can lead to scarring, blood vessel problems, varying degrees of inflammation, and is apparently a manifestation of an overactive immune system. Scleroderma is classified into localized scleroderma and systemic sclerosis. CREST syndrome is a limited form of systemic sclerosis. Patients with scleroderma can have specific antibodies (ANA, anticentromere or antitopoisomerase) in their blood, which strongly suggests an autoimmune etiology ...
CREST Syndrome (Limited Scleroderma) Systemic scleroderma, or systemic sclerosis, is the term for the disease that is characterized by diffuse thickening of the
Antinuclear antibodies are associated with rheumatic diseases including Systemic Lupus Erythematous (SLE), mixed connective tissue disease, Sjogrens syndrome, scleroderma, polymyositis, CREST syndrome, and neurologic SLE. ...
Disorders of calcium-phosphate-parathormone balance, are very important issues in ESRD patients, that may lead to severe complications, as dystrophic calcinosis cutis, a rare disease, caused by calcium salt deposits in cutaneous or subcutaneous tissues and many organs.. We present the case of a 47 years old woman, in ESRD due to membranous glomerulopathy, treated by peritoneal dialysis, who, after 7 months of dialysis, developed painful masses on second finger and fifth metacarpus of the right hand.. Laboratory and instrumental data showed hyperparathyroidism with a parathyroid mass consistent with adenoma.. Increasing of therapy with phosphate binders and cinacalcet only, was not effective to solve cutaneous masses, that were biopsied. Histological exam revealed deposition of amorphic material with calcific component, consistent with cutaneous dystrophic calcinosis.. We further increased dialysis and therapy and we observed complete regression of masses in 2 months.. Key words: dystrophic ...
Not everyone with scleroderma develops this degree of skin hardening. However, it is this symptom that has earned scleroderma the nickname of the disease that turns people to stone.. Systemic scleroderma often affects the hands. The initial stage is swelling (edema), which can last for weeks, months, or years. Often the swelling is intermittent and worse in the morning. It can cause the fingers to look like sausages, with far fewer wrinkles. Skin tightness in the hands can make it impossible to pinch the skin on the fingers ...
No absolute contraindications exist.. Copyright © 2017, 2013 Decision Support in Medicine, LLC. All rights reserved. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. The Licensed Content is the property of and copyrighted by DSM. ...
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This is a photo of a calcium deposit (calcinosis) on a dime. The calcinosis was removed from a patient who has had systemic sclerosis (scleroderma) for a year. ISN Photo Repository.
Generation of a ninein polyclonal antibody. (A) Ninein is a 2096-chain amino acid protein. The 381-689 fragment of ninein (solid bar) was used to generate the n
1. How Can Follow-Up of Patients with Raynaud Phenomenon be Optimized?. Med Sci Monit Basic Res, 2015, 21 p. 47. DOI: 10.12659/MSMBR.893998. Citations as recorded by: ...
Let X be an algebraic curve of genus g⩾2 embedded in its Jacobian variety J. The Manin-Mumford conjecture (proved by Raynaud) asserts that X contains only finitely many points of finite order. When X is defined over a number field, Bogomolov conjectured a refinement of this statement, namely that except for those finitely many points of finite order, the Néron-Tate heights of the algebraic points of X admit a strictly positive lower bound. This conjecture has been proved by Ullmo, and an extension to all subvarieties of Abelian varieties has been proved by Zhang soon after. These proofs use an equidistribution theorem in Arakelov geometry due to Szpiro, Ullmo, and Zhang. Using more classical techniques of diophantine geometry, David and Philippon have given another proof which, moreover, provides an effective lower bound. In the talk, I will present the equidistribution statement, and his powerful generalization due to Yuan. I will then give the proof of the Bogomolov
... but is also seen the more limited form of systemic scleroderma called CREST syndrome. However, CREST syndrome is more closely ...
It is a part of CREST syndrome, referring to the five main features: calcinosis, Raynaud syndrome, esophageal dysmotility, ... Winterbauer RH (1964). "Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutaneous calcinosis: a syndrome ...
Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia (CREST syndrome) are associated ...
"Cranial neural crest ablation of Jagged1 recapitulates the craniofacial phenotype of Alagille syndrome patients". Hum. Mol. ... GeneReviews/NCBI/UW/NIH entry on Alagille syndrome OMIM entries on Alagille syndrome JAG1+protein,+human at the US National ... McCright B, Lozier J, Gridley T (2002). "A mouse model of Alagille syndrome: Notch2 as a genetic modifier of Jag1 ... Notch signaling Alagille syndrome Autosomal dominant Haploinsufficiency Tetralogy of fallot In situ hybridization Conditional ...
Other possible causes include gout, sarcoidosis, CREST syndrome, diabetes mellitus, alcohol use disorder, uremia, and chronic ...
In addition, calcinosis is seen in Limited Cutaneous Systemic Sclerosis, also known as CREST syndrome (the "C" in CREST). In ... Calcinosis cutis in a dog with Cushing's syndrome Calcinosis cutis in human tissue Calcinosis List of cutaneous conditions ...
Ravassard P, Côté F, Grondin B, Bazinet M, Mallet J, Aubry M (2000). "ZNF74, a gene deleted in DiGeorge syndrome, is expressed ... in human neural crest-derived tissues and foregut endoderm epithelia". Genomics. 62 (1): 82-5. doi:10.1006/geno.1999.5982. PMID ... "Isolation of a zinc finger gene consistently deleted in DiGeorge syndrome". Hum. Mol. Genet. 2 (10): 1583-7. doi:10.1093/hmg/ ...
... mutations in PACS1 cause defective cranial-neural-crest migration and define a recognizable intellectual-disability syndrome". ... colloquially called PACS1 Syndrome) that is characterized by global developmental delay, intellectual disability, and specific ...
"Livedo Reticularis and Bowel Ischemia after Carbon Dioxide Arteriography in a Patient with CREST Syndrome". Journal of Vascular ... "Polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature ('FILS syndrome ... Kazmier F, Sheps S, Bernatz P, Sayre G (1966). "Livedo reticularis and digital infarcts: a syndrome due to cholesterol emboli ... Daneshpazhooh M, Nazemi TM, Bigdeloo L, Yoosefi M (2007). "Mucocutaneous findings in 100 children with Down syndrome". Pediatr ...
Pulmonary veno-occlusive disease This condition has been reported in patients with Ehlers Danlos syndrome, CREST syndrome and ... Pulmonary capillary hemangiomatosis associated with CREST syndrome: A challenge of diagnosis and treatment. Chin Med J (Engl) ... Chest 151(4):821-828 Park MA, Shin SY, Kim YJ, Park MJ, Lee SH (2017) Vascular Ehlers-Danlos syndrome with cryptorchidism, ... Reactive pulmonary capillary hemangiomatosis and pulmonary veno-acclusive disease in a patient with repaired scimitar syndrome ...
... or tumors of neural crest origin. Central hypoventilation syndrome is a heterogeneous group of seemingly overlapping diseases. ... LO-CHS/HD or LOCHS/HD Congenital central hypoventilation syndrome (CCHS) Prader-Willi syndrome Congenital central ... ROHHAD syndrome is a disease that is potentially lethal and incurable. Fifteen patients with ROHHAD were evaluated by Diego Ize ... Lee P, Su YN, Yu CJ, Yang PC, Wu HD (February 2009). "PHOX2B mutation-confirmed congenital central hypoventilation syndrome in ...
Because the traits commonly affected by domestication syndrome are all derived from NCC in development, the neural crest ... The Neural Crest Hypothesis relates adrenal gland function to deficits in neural crest cells during development. The Single ... Domestication syndrome is a term often used to describe the suite of phenotypic traits arising during domestication that ... Neural crest cells (NCC) are vertebrate embryonic stem cells that function directly and indirectly during early embryogenesis ...
... retiring in 1987 after being diagnosed with CREST syndrome. After that, she was invited to several AAGPBL Players Association ...
... include neural crest disorders such as piebaldism, Waardenburg syndrome, or other depigmentation conditions such as vitiligo. ... are largely responsible for generating skin and eye colour in poikilothermic animals and are generated in the neural crest ...
They occur in some autoimmune diseases, frequently in limited systemic scleroderma (formerly called CREST syndrome), and ...
... syndrome is the suite of phenotypic traits arising during domestication that distinguish crops from their wild ... docility in mammals and birds results partly from a slowed pace of neural crest development, that would in turn cause a reduced ... The theory was unable to explain curly tails nor domestication syndrome exhibited by plants. A side effect of domestication has ... Wilkins, Adam S.; Wrangham, Richard W.; Fitch, W. Tecumseh (July 2014). "The 'Domestication Syndrome' in Mammals: A Unified ...
... are one of the features of the acronymically named CREST syndrome, a form of systemic scleroderma. The syndrome recognises the ... Osler-Weber-Rendu syndrome) Ataxia-telangiectasia Sturge-Weber syndrome, a nevus formation in the skin supplied by the ... Klippel-Trenaunay syndrome Maffucci syndrome (multiple enchondromas and hemangiomas) Hereditary hemorrhagic telangiectasia ( ... Tempi syndrome Tobacco smoking Before any treatment of leg telangectasia (spider veins) is considered, it is essential to have ...
This may be the case in the CREST syndrome, of which Raynaud's is a part. ... Raynaud syndrome, also known as Raynaud's phenomenon, is a medical condition in which spasm of arteries cause episodes of ... The prognosis of primary Raynaud syndrome is often very favorable, with no mortality and little morbidity. However, a minority ... "Association between treatment with central nervous system stimulants and Raynaud's syndrome in children: A retrospective case- ...
Anti-centromere antibodies are associated with limited cutaneous systemic sclerosis, also known as CREST syndrome, primary ... In Sjögren's syndrome, anti-Ro and anti-La antibodies correlate with early onset, increased disease duration, parotid gland ... The presence of both antibodies is found in 30-60% of Sjögren's syndrome, anti-Ro antibodies alone are found in 50-70% of ... Anti-La antibodies are also found in SLE; however, Sjögren's syndrome is normally also present. Anti-Ro antibodies are also ...
One form of the condition, known as CREST syndrome, classically results in calcium deposits, Raynaud's syndrome, esophageal ... causing a limited systemic form and the CREST syndrome). Other autoantibodies can be seen, such as anti-U3 or anti-RNA ... Eosinophilia-myalgia syndrome is a form of eosinophilia caused by L-tryptophan supplements. Eosinophilic fasciitis affects the ... Atrophoderma of Pasini and Pierini Pansclerotic morphea Morphea profunda Linear scleroderma Systemic scleroderma CREST syndrome ...
Sclerosis of the esophagus, such as with systemic sclerosis or in CREST syndrome may cause hardening of the walls of the ... Half of these cases may be part of a syndrome where other abnormalities are also present, particularly of the heart or limbs. ... Strictures may also develop in chronic anemia, and Plummer-Vinson syndrome. Two of the most common congenital malformations ...
"The 'Domestication Syndrome' in Mammals: A Unified Explanation Based on Neural Crest Cell Behavior and Genetics". Genetics 197 ...
Crest • Crossbite • Crouzon syndrome • Crown-to-root ratio • Crown • Crown • Crown lengthening • Crunchiness • Curve of spee • ... Frey's syndrome • Fungiform papilla • G. Walter Dittmar • Gardner's syndrome • Gargling • Gaspard Fauteux • Gene Derricotte • ... Tariq Faraj • E. Lloyd Du Brul • Eagle syndrome • Early childhood caries • Eastman Kodak • Ed Lafitte • Eco-friendly dentistry ... Peutz-Jeghers syndrome • Piercing • Phil Samis • Philip A. Traynor • Philip Blaiberg • Philtrum • Pierre Corbeil • Pierre ...
Association of an Irregularly Shaped Anterior Choroidal Aneurysm With CREST Syndrome. A case report. Journal of Neurosurgery. ...
"The 'Domestication Syndrome' in Mammals: A Unified Explanation Based on Neural Crest Cell Behavior and Genetics". Genetics ( ...
... see also CREST syndrome) Calciphylaxis Calculi Calderon-Gonzalez-Cantu syndrome Calloso genital dysplasia Callus disease ... CCA syndrome Ccge syndrome CCHS CDG syndrome type 1A CDG syndrome type 1B CDG syndrome type 1C CDG syndrome type 2 CDG syndrome ... syndrome Coffin-Siris syndrome COFS syndrome Cogan-Reese syndrome Cogan syndrome Cohen-Gibson syndrome Cohen-Hayden syndrome ... syndrome type 1 Cockayne syndrome type 2 Cockayne syndrome type 3 Cockayne's syndrome Codas syndrome Codesette syndrome Coeliac ...
... of cases of the more limited form of systemic scleroderma called CREST syndrome. Anti Scl-70 antibodies are associated with ...
Calcinosis-Raynaud phenomenon-esophageal dysmotility-sclerodactyly-telangiectasia syndrome (CREST syndrome) Chilblain lupus ... Turner syndrome Ulnar-mammary syndrome Van Der Woude syndrome Von Hippel-Lindau syndrome Watson syndrome Werner syndrome (adult ... Freeman-Sheldon syndrome, Windmill-Vane-Hand syndrome) Wilson-Turner syndrome Wolf-Hirschhorn syndrome (4p- syndrome) X-linked ... Rombo syndrome Rothmund-Thomson syndrome (poikiloderma congenitale) Rud syndrome Say syndrome Scalp-ear-nipple syndrome (Finlay ...
Conditional inactivation of TGF-βr2 of osteochondroprogenitor cells in the cranial neural crest resulted in faster ... Conradi-Hünermann syndrome. Other dwarfism. *Fibrochondrogenesis. *Short rib - polydactyly syndrome *Majewski's polydactyly ...
Toxic oil syndrome. References[edit]. *^ Bolton P, Lindgren CE, Redmon GL (1991). "A mystery ailment revealed". American ... Eosinophilia-myalgia syndrome is a rare, sometimes fatal neurological condition linked to the ingestion of the dietary ... Blackburn WD (June 1997). "Eosinophilia myalgia syndrome". Seminars in Arthritis and Rheumatism. 26 (6): 788-93. doi:10.1016/ ... "Rare Disease Database: Eosinophilia-Myalgia Syndrome". National Organization for Rare Disorders. NORD. Archived from the ...
Overexpression of SOD1 has been linked to the neural disorders seen in Down syndrome.[38] In patients with thalassemia, SOD ... Superoxide dismutase is also not expressed in neural crest cells in the developing fetus. Hence, high levels of free radicals ... a potential mechanism underlying adult respiratory distress syndrome". The American Journal of Pathology. 173 (4): 915-26. doi: ... "Cell damage by excess CuZnSOD and Down's syndrome". Biomedicine & Pharmacotherapy. 48 (5-6): 231-40. doi:10.1016/0753-3322(94) ...
The tissues that sit above the alveolar bone crest are considered the free gingiva. In healthy periodontium, the gingival ... Individual risk factors include: Gender, smoking and alcohol consumption, diabetes, obesity and metabolic syndrome, ... down syndrome etc. Treatment and preventions are a very important concept in the management and maintenance of periodontitis as ... Alveolar crest fibres of periodontal ligament [PDL] J. Horizontal fibres of PDL K. Oblique fibres of PDL ...
DiGeorge syndrome is a genetic disorder caused by the deletion of a small section of chromosome 22. This results in a midline ... and extend outward and backward into the surrounding mesoderm and neural crest-derived mesenchyme in front of the ventral aorta ... DiGeorge syndrome is the most common congenital cause of thymic aplasia in humans. In mice, the nude mouse strain are ... These syndromes are caused by defective hematopoietic progenitor cells which are the precursors of both B- and T cells. This ...
Quadratus lumborum arises from the posterior part of the iliac crest and extends to the rib XII and lumbar vertebrae I-IV. It ... ISBN 978-1-4511-1945-9. Morris, Craig E. (2005). Low Back Syndromes: Integrated Clinical Management. McGraw-Hill. ISBN 0-07- ... Maximus has a wide origin stretching from the posterior part of the iliac crest and along the sacrum and coccyx, and has two ... Quadratus arises on the ischial tuberosity and is inserted into the intertrochanteric crest. The superior and inferior gemelli ...
Iliac crest spur. *Psoas tendinitis. *Trochanteric tendinitis. Diagnosis[edit]. This section is empty. You can help by adding ... Rotator cuff syndrome of shoulder and allied disorders. *Periarthritis of shoulder. *Scapulohumeral fibrositis ...
Marfan syndrome. *Trauma, such as traumatic aortic rupture, most often thoracic and distal to the left subclavian artery[17] ... This contribution of the neural crest to the great artery smooth muscle is unusual as most smooth muscle is derived from ... cells that form the aorticopulmonary septum that separates the aorta and pulmonary artery is derived from cardiac neural crest ...
Related to urethritis is so called urethral syndrome. *Passage of kidney stones through the urethra can be painful, which can ...
... such as with systemic sclerosis or in CREST syndrome may cause hardening of the walls of the esophagus and interfere with ... Strictures may also develop in chronic anemia, and Plummer-Vinson syndrome.[4] ... Half of these cases may be part of a syndrome where other abnormalities are also present, particularly of the heart or limbs. ...
The first pharyngeal pouch syndrome associates middle ear anomalies to the malleus and incus structures as well as to the non- ... All three ossicles develop from the neural crest.[16] Eventually cells from the tissue surrounding the ossicles will experience ... These anomalies include chromosome syndromes such as ring 18. Children may also present cases of abnormal ear canals and low ...
... with the crest of the breast halfway between the elbow and shoulder,[63] usually over the nipples,[64] ideally while standing ... "Amazon's Syndrome" by physicians, may require corrective surgery due to morphological alterations caused by variations in shape ...
... and Down's syndrome.[59][29][76] Recent studies have concluded that 5-9% of Down's syndrome cases are due to paternal effects, ... neural crest cell disruption, endocrine disruption, oxidative stress, vascular disruption and specific receptor- or enzyme- ... "Search Jablonski's Syndromes Database". United States National Library of Medicine.. *^ Croen, Lisa; Todoroff, Karen; Shaw, ... When the order is not known, it is a syndrome.. Examples of primarily structural congenital disorders[edit]. A limb anomaly is ...
1q21.1 deletion syndrome/1q21.1 duplication syndrome/TAR syndrome/1p36 deletion syndrome) *1 ... neural crest cell disruption, endocrine disruption, oxidative stress, vascular disruption, and specific receptor- or enzyme- ... syndromes involving multiple systems, and Down syndrome.[60][29][77] Recent studies have concluded that 5-9% of Down syndrome ... "Search Jablonski's Syndromes Database". United States National Library of Medicine.. *^ Croen, Lisa; Todoroff, Karen; Shaw, ...
Spinous processes of vertebrae T7-L5, thoracolumbar fascia, iliac crest, inferior 3 or 4 ribs and inferior angle of scapula. ... Beer GM, Kompatscher P, Hergan K (1996). "Poland's syndrome and vascular malformations". Br J Plast Surg. 49 (7): 482-4. doi: ... or to correct pectoral hypoplastic defects such as Poland's syndrome.[13][14] An absent or hypoplastic latissimus dorsi can be ... "Autologous reconstruction of a complex form of Poland syndrome using 2 abdominal perforator free flaps". Ann Plast Surg. 74 (5 ...
... the migration path through which neural crest cells migrate is rich in HA.[26] HA is closely associated with the cell migration ... "Over the counter (OTC) artificial tear drops for dry eye syndrome". Cochrane Database Syst Rev. 2: CD009729. doi:10.1002/ ...
The wavelength is the distance from one peak of the wave's electric field (wave's peak/crest) to the next, and is inversely ...
Katie Eloise Foster, The Role of Neural Crest Cells in the Development, Organisation and Migration of the Thymus, Research ... T. Petteri Arstila ja Hanna Jarva, Human APECED; a Sick Thymus Syndrome?, Front Immunol. 2013; 4: 313., 7. oktoober 2013. doi: ... UpToDate, Christine M Seroogy, MD, E Richard Stiehm, MD, Elizabeth TePas, MD, MS, DiGeorge syndrome: Epidemiology and ... Robert P. Lisak, Handbook of Myasthenia Gravis and Myasthenic Syndromes, MARCEL DEKKER INC, lk 208, 1994 ...
... which are associated with antiphospholipid syndrome.[90] Antiphospholipid syndrome is also related to the onset of neural lupus ... "Neurological syndromes in systemic lupus erythematosus and their association with antiphospholipid syndrome". Neurol. Neurochir ... Inflammation of the pleurae known as pleurisy can rarely give rise to shrinking lung syndrome.[25] SLE can cause pleuritic pain ... Neuropsychiatric syndromes can result when SLE affects the central or peripheral nervous system. The American College of ...
L4 is at highest point of iliac crest. S2 is at the level of posterior superior iliac spine. Furthermore, C7 is easily ... Bertolotti's syndrome. *Spinal disc herniation. *Lumbar spinal stenosis. ReferencesEdit. This article incorporates text in the ...
... it may be associated with rarer genetic syndromes such as DiGeorge syndrome, which is inherited as an autosomal dominant ... The parathyroid glands originate from the interaction of the endoderm of the third and fourth pouch and neural crest mesenchyme ... Hyperparathyroidism is most commonly caused by a benign proliferation of chief cells in single gland, and rarely MEN syndrome. ... syndrome. Hypoparathyroidism will occur after surgical removal of the parathyroid glands. Occasionally, an individual's tissues ...
... is also found in tissues that are not derived from the neural crest, including the wall of the digestive tract, kidneys, ... GeneReviews/NIH/NCBI/UW entry on Mowat-Wilson syndrome ZEB2 protein, human at the US National Library of Medicine Medical ... ZEB2 transcripts are found in tissues differentiated from the neural crest such as the cranial nerve ganglia, dorsal root ... Mutations in the ZEB2 gene are associated with the Mowat-Wilson syndrome. This disease exhibits mutations and even complete ...
... compartment syndromes or fat embolism. Compound fractures involve the bone's penetration through the skin. Some complex ... ", "crest", "spine", "eminence", "tubercle" or "tuberosity", depending on the protrusion's shape and location. In general, long ...
... iliac crest, epicondyles, ... Felty's syndrome. *3.Crystal arthropathy: Gout. * ...
The crest above the shield and helmet is a seven-pointed gold star on a blue and gold wreath, representing the 6 states and the ... Mirroring the tall poppy syndrome which brings back to Earth the high fliers, the egalitarian Australian society has a ... and is known as the tall poppy syndrome, where people who grow greater than their peers are harshly criticised as being ...
A primary component of Cushing's syndrome is the accumulation of fat in the abdominal region, and it is hypothesized that ... should be measured at the midpoint between the lower margin of the last palpable rib and the top of the iliac crest, using a ... and the National Health and Nutrition Examination Survey used results obtained by measuring at the top of the iliac crest. ... relationship between cortisol and central fat distribution has primarily been studied in individuals with Cushing's syndrome.[ ...
CREST is an acronym for the clinical features that are seen in a patient ... The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis, scleroderma ... Usually only 2 of the 5 symptoms of the CREST syndrome is necessary to be diagnosed with the disease. ... The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis (scleroderma ...
In 1910, Thibierge and Weissenbach described the first case report of what was later called CRST (calc... ... syndrome is a member of the heterogeneous group of sclerodermas, and its name is an acronym for the cardinal clinical features ... CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) ... CREST Syndrome) and CREST Syndrome What to Read Next on Medscape. Related Conditions and Diseases. * CREST Syndrome ...
Crest syndrome definition at Dictionary.com, a free online dictionary with pronunciation, synonyms and translation. Look it up ...
"CREST syndrome: MedlinePlus Medical Encyclopedia Image". medlineplus.gov. "CREST syndrome - Genetic and Rare Diseases ... CREST syndrome can be noted in up to 10% of patients with primary biliary cholangitis. The combination of symptoms was first ... CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue ... Other symptoms of CREST syndrome can be exhaustion, weakness, difficulties with breathing, pain in hands and feet, dizziness ...
CREST is a form of Systemic Sclerosis (scleroderma) which is characterized by Calcinosis (calcium deposits), Raynauds, ... Overview: What is CREST?. "Pure" CREST. "Plus" CREST. Progression. CREST Antibodies. CREST Overlaps. Treatments. CREST Patient ... CREST Syndrome. "Patients with CREST syndrome are a subset of patients with scleroderma…" Medscape. ... "Pure" CREST. "Pure"CREST - by itself: "Pure" CREST is diagnosed when patients have two or more symptoms of CREST but they do ...
My name is Jay and I have scleroderma with CREST syndrome. I have calcium deposits, Raynauds, thyroid problems, heartburn, and ... CREST Syndrome. Calcinosis. Difficulty Swallowing (Dysphagia). Heartburn (Reflux). Raynauds. Skin Tightening (Sclerodactyly). ... I remember saying, "What? Like toothpaste?" I had CREST or at least the CR of CREST. If I only had known what the future had in ... So ended the (C) in Crest Syndrome and now on to (R) Raynauds. ... Jay: Scleroderma and CREST. The pain was so intense that tears ...
... I have suffered from Raynauds for years and cannot stand the cold.. I have no idea ...
Comparison of the CRST syndrome with the common acrosclerotic type of scleroderma is made, revealing several common features ... The CRST syndrome is a recently defined symptom-complex consisting of (1) calcinosis cutis, (2) Raynauds phenomenon, (3) ... CRST Syndrome. Richard D. Carr, M.D.; Eldred B. Heisel, M.D. ... The CRST syndrome does appear to pursue a prolonged and more ... CRST Syndrome.. Ann Intern Med. 1966;64:1174. doi: 10.7326/0003-4819-64-5-1174_2 ...
CREST syndrome is related to antibodies that combat centromeres (regions of DNA chromosomes) like ones in the kidneys and lungs ... CREST Syndrome Misdiagnosis. CREST syndrome is related to antibodies that combat centromeres (regions of DNA chromosomes) like ... Common Misdiagnoses of CREST Syndrome. Due to the general symptoms CREST syndrome patients may feel such as indigestion and ... Symptoms of CREST Syndrome. CREST syndrome involves a host of symptoms depending to the pathology involved. Symptoms for ...
An award-winning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, GI, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, hand-out notes, interactive material, most commons lists and pictorial differential diagnoses
Facial recognition is central to the diagnosis of many syndromes, and craniofacial patterns may reflect common etiologies. In ... These defects of the craniofacial (CF) skeleton arise from aberrant cranial neural crest cell (NCC) migration. These effects ... to reveal that BBS patients and mouse mutants have mid-facial defects involving homologous neural crest-derived structures ... the pleiotropic Bardet-Biedl syndrome (BBS), a primary ciliopathy with intraflagellar transport dysfunction, patients have a ...
... previously CREST syndrome, standing for calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, ... Overlap syndromes present particular diagnostic challenges. Patients with polymyositis/systemic sclerosis overlap have been ... described with the typical features of systemic sclerosis in addition to polymyositis, dermatomyositis, and Sjogrens syndrome ...
... also known as iliac crest pain syndrome, involves an inflammation or tear of the iliolumbar ligament. This ligament extends ... from the spine to the iliac crest, which is the back of the pelvis. It can lead to referred pain in the groin, the pelvis, the ... Iliolumbar ligament injury , Iliolumbar Syndrome , Iliac crest pain syndrome. Ross Hauser, MD , Caring Medical Regenerative ... Clues during the examination you may have Iliolumbar ligament injury , Iliolumbar Syndrome , Iliac crest pain syndrome. In the ...
CREST syndrome is a variant of progressive systemic sclerosis (PSS) and stands for C - Calcinosis R - Raynaud phenomenon E - ... CREST syndrome. Dr Henry Knipe ◉ ◈ and A.Prof Frank Gaillard ◉ ◈ et al. ... CREST syndrome is a variant of progressive systemic sclerosis (PSS) and stands for ... Disproportionately severe calcinosis cutis in an 88-year-old patient with CREST syndrome. Skeletal Radiol. 2001;30 (8): 478-81 ...
Barretts Esophageal Adenocarcinoma with CREST Syndrome. Zeitschrift:. Esophagus > Ausgabe 3/2017 Autoren:. Tomohiro Kunishige ... Clinical, serological and genetic study in patients with CREST syndrome. Intern Med. 2000;39:451-6. CrossRefPubMed ... Barretts Esophageal Adenocarcinoma with CREST Syndrome Autoren:. Tomohiro Kunishige Sohei Matsumoto Kohei Wakatsuki Kazuhiro ... The patient was diagnosed with Barretts adenocarcinoma and CREST syndrome, which is a form of scleroderma. Subtotal ...
Musculocontractural Ehlers-Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells ... which is designated the neural crest (NC). Musculocontractural Ehlers-Danlos syndrome (MCEDS) is a heritable connective tissue ... disorder with distinct craniofacial features; this syndrome comprises multiple congenital malformations that are caused by ...
Loss of MicroRNAs in Neural Crest Leads to Cardiovascular Syndromes Resembling Human Congenital Heart Defects. Zhan-Peng Huang ... Loss of MicroRNAs in Neural Crest Leads to Cardiovascular Syndromes Resembling Human Congenital Heart Defects ... Loss of MicroRNAs in Neural Crest Leads to Cardiovascular Syndromes Resembling Human Congenital Heart Defects ... Loss of MicroRNAs in Neural Crest Leads to Cardiovascular Syndromes Resembling Human Congenital Heart Defects ...
... such as Treacher Collins syndrome, Nager syndrome and Miller syndrome (Trainor and Andrews, 2013), which indicates that the NC ... pharyngeal arch in Treacher Collins syndrome, Nager syndrome and Miller syndrome (Trainor and Andrews, 2013), which suggests a ... Musculocontractural Ehlers-Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells ... Musculocontractural Ehlers-Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells ...
Chromosome 13q deletion with Waardenburg syndrome: further evidence for a gene involved in neural crest function on 13q. ... Chromosome 13q deletion with Waardenburg syndrome: further evidence for a gene involved in neural crest function on 13q. ...
... here are a few serious complications associated with CREST syndrome. As there is no perfect cure to treat CREST syndrome, it ... which are collectively known as CREST syndrome.. CREST syndrome, commonly know as limited Scleroderma, mainly affects the ... Gastrointestinal problems: CREST syndrome can affect any tissue along your digestive tract and causes damage to your esophagus ... Most visible signs of CREST syndrome like tight, thick skin on your fingers and face can change the appearance of your skin. It ...
CHARGE syndrome modeling using patient-iPSCs reveals defective migration of neural crest cells harboring CHD7 mutations ELIFE ... To better delineate neural crest defects in CHARGE syndrome, we generated induced pluripotent stem cells (iPSCs) from two ... These results support the historical inference that CHARGE syndrome patients exhibit defects in neural crest migration, and ... CHARGE syndrome is caused by heterozygous mutations in the chromatin remodeler, CHD7, and is characterized by a set of ...
We report 2 cases of thoracic neural crest tumors complicating the course in patients with Beckwith-Wiedemann syndrome (BWS). ... Neural Crest Development Neural Crest Development Neural Crest Development Neural Crest Development. Oct 20, 2017 ... Beckwith-wiedemann syndrome and neural crest tumors. *. Dominique Sirinelli, Benjamin Silberman, J. J. Baudon, Patrick ... We report 2 cases of thoracic neural crest tumors complicating the course in patients with Beckwith-Wiedemann syndrome (BWS). ...
CREST syndrome is one expression of a larger disease known as systemic sclerosis (or, if youre stuck in your ways like me, ... CREST syndrome is one expression of a larger disease known as systemic sclerosis (or, if youre stuck in your ways like me, ... CREST syndrome is one expression of a larger disease known as systemic sclerosis (or, if youre stuck in your ways like me, " ... CREST syndrome. Sep 21, 2014 , Cardiac pathology, Pulmonary pathology, Renal pathology, Skin pathology ...
CREST syndrome). LcSSc is also known as morphea.. Diffuse Systemic Sclerosis is distinguished from the localized variant (CREST ... Systemic Sclerosis (CREST syndrome). Introduction. Systemic sclerosis, otherwise known as Scleroderma, is a rare connective ... This is more pronounced for CREST syndrome, with a female to male ratio of 10:1. The diffuse variant is more evenly distributed ... Limited Scleroderma (CREST) syndrome. The clinical features and disease progression are more insidious. Limited scleroderma ( ...
Acrodermatitis Syndrome, Crest Syndrome, Cutis Laxa, Fifths Disease, Hirsutism, Ichthyosis Vulgaris, Hyperhidrosis, Melanoma, ...
Definition: "CREST syndrome" is more appropriately referred to as "limited scleroderma." In this variant of systemic sclerosis ... Those with longstanding CREST syndrome are at greatest risk for developing progressive pulmonary hypertension, usually late in ... Uncommon Findings: Small bowel disease is infrequently encountered in those with longstanding CREST syndrome and may manifest ... Clinical and serological comparison of 17 chronic progressive systemic sclerosis (PSS) and 17 CREST syndrome patients matched ...
... My thrombocytopenia, Online resources for thrombocytopenia. ... Crest Syndrome Thrombocytopenia. The study showed that it truly is not just the surge of estrogen on a regular period it is ... It is crest syndrome thrombocytopenia important flavonoids in the bloodstream it can do for you. To give you and feel the power ... Rid your body clear fat and was routines specifically on the face headache myogenic headache crest syndrome thrombocytopenia ...
I had to look up CREST Syndrome. I dont have it, but I do have some of the symptoms of it. I have hEDS. I have the Raynauds ... Auto I do not have CREST either but I have Polymyalgia Rhuematica and Myositis which I had not heard of until I was diagnosis. ... What is CREST? Care to enlighten those of us who have never heard of it? ... Im so sorry you going thru a disease that doesnt have enough info just like Sjögrens syndrome and fibromyalgia. ...
What is CREST Syndrome? Learn about common symptoms, coexisting conditions, and risk factors associated with this autoimmune ... Studies have found a greater prevalence of CREST syndrome in females, African Americans, and Choctaw Native Americans. Exposure ... Sjögrens syndrome, SLE, autoimmune thyroiditis, hypothyroidism, rheumatoid arthritis, cardiovascular disease, type 1 diabetes ...
  • CREST ( calcinosis , Raynaud phenomenon , esophageal dysmotility , sclerodactyly, and telangiectasia) syndrome is a member of the heterogeneous group of sclerodermas , and its name is an acronym for the cardinal clinical features of the syndrome. (medscape.com)
  • In 1910, Thibierge and Weissenbach described the first case report of what was later called CRST (calcinosis cutis, Raynaud phenomenon, sclerodactyly, and telangiectasia) syndrome in English by Winterbauer who, in 1964, described a series of 8 patients with the features that make up the abbreviation CRST. (medscape.com)
  • Additionally, CREST syndrome stands as an acronym for five chief features including: Calcinosis, Raynard's syndrome, esophageal dysmotility, Sclerodactyly and Telangiectasia. (medicalmalpracticehelp.com)
  • CREST is an acronym for the clinical features that are seen in a patient with this disease, namely Calcinosis, Raynaud's phenomenon, Esophageal dysfunction , Sclerodactyly, and Telangiectasia. (hellodoktor.com)
  • The T in CREST stands for Telangiectasia. (chronicallyelle.blog)
  • We report a case of a 61-year-old man with a history of CREST syndrome (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) who presented for evaluation of thrombocytopenia. (elsevier.com)
  • Beacham BE, Kurgansky D. Unilateral naevoid telangiectasia syndrome associated with metastatic carcinoid tumour. (medscape.com)
  • Kreft B, Marsch WC, Wohlrab J. Unilateral nevoid telangiectasia syndrome. (medscape.com)
  • Peripheral phenomena in a woman with calcinosis, raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome-associated pulmonary hypertension. (nih.gov)
  • Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. (nih.gov)
  • Primary sclerosing cholangitis associated with CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) in an elderly woman: a case report. (nih.gov)
  • [ 2 , 3 ] Although he noted esophageal dysmotility in 4 of 8 patients, he did not include this feature in his original description of CRST syndrome. (medscape.com)
  • [ 4 ] noted the frequent occurrence of esophageal dysmotility and suggested that the acronym CREST may be more appropriate. (medscape.com)
  • Scleroderma has been further subclassified into localized scleroderma (morphea), limited cutaneous systemic sclerosis (previously CREST syndrome, standing for calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) and diffuse cutaneous systemic sclerosis. (oncologynurseadvisor.com)
  • Its name is an acronym for the common clinical features of the syndrome, which are calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. (autoimmunityblog.com)
  • Solid plus liquid dysphagia is due to esophageal motility disorder (or dysmotility) either in the upper esophagus ( myasthenia gravis , stroke , or dermatomyositis ) or lower esophagus ( systemic sclerosis , CREST syndrome, or achalasia ). (wikipedia.org)
  • Because it was designed for research applications and not for clinical diagnosis, it has been criticized for its low sensitivity in identifying early disease and milder forms of systemic scleroderma such as CREST syndrome. (medscape.com)
  • It takes only two of the five CREST symptoms for a diagnosis of CREST (either "pure" or "plus") to be made. (sclero.org)
  • Facial recognition is central to the diagnosis of many syndromes, and craniofacial patterns may reflect common etiologies. (uniprot.org)
  • Auto I do not have CREST either but I have Polymyalgia Rhuematica and Myositis which I had not heard of until I was diagnosis. (catchmypain.com)
  • I just told him about my swallowing issues, joint pain, muscle stiffness and now he has me going on a goose chase for crest syndrome blood tests and I just researched the auto immune disease and it's not a good diagnosis. (aboutbfs.com)
  • CREST Syndrome is my main diagnosis, and that diagnosis came in October of 2017. (chronicallyelle.blog)
  • After having it removed and tested, my dermatologist reached out to my rheumatologist and told her to consider CREST as my diagnosis, and that is how I was eventually diagnosed. (chronicallyelle.blog)
  • Our work will advance understanding and diagnosis of this multisystemic syndrome and may lead to improved clinical interventions in patients. (grantome.com)
  • The clinical example clearly demonstrates the importance of timely diagnosis of esophageal stricture in CREST syndrome and suggests that it is necessary to treat these difficult-to-treat patients at large multiprofile hospitals. (vrachjournal.ru)
  • Differential diagnosis includes bullous pemphigoid, Stevens-Johnson syndrome, and epidermolysis bullosa acquisita. (amazonaws.com)
  • CREST syndrome is associated with detectable antibodies against centromeres (a component of the cell nucleus), and usually spares the kidneys (a feature more common in the related condition systemic scleroderma). (wikipedia.org)
  • Although some doctors still believe CREST is a useful subcategory, the existing research studies have been unable to predict consistently how (or whether) the disease will progress to Diffuse Systemic Scleroderma in any specific individual. (sclero.org)
  • The anticentromere antibody (ACA) test is primarily ordered to help diagnose the autoimmune disorder limited cutaneous scleroderma, a form of systemic scleroderma , and CREST syndrome. (labtestsonline.org)
  • CREST syndrome, or limited cutaneous systemic scleroderma, is an autoimmune disease that causes scarring of different tissues in the body, most notably the skin. (timesunion.com)
  • They are also referred to as anti-DNA topoisomerase I antibody (anti-topo I). Anti Scl-70 antibodies (also called anti-topoisomerase I after the type I topoisomerase target) is a type of anti-nuclear autoantibody seen mainly in diffuse systemic scleroderma, but is also seen the more limited form of systemic scleroderma called CREST syndrome. (wikipedia.org)
  • Primary biliary cirrhosis accompanied by CREST syndrome. (sclero.org)
  • Granulomatous uveitis, CREST syndrome, and primary biliary cirrhosis. (sclero.org)
  • We report the case of a 70-year old woman with primary biliary cirrhosis, CREST syndrome and vascular gastric lesions corresponding to watermelon stomach. (uclouvain.be)
  • CREST is an acronym for the clinical features that are seen in a patient with this disease. (medlineplus.gov)
  • It's easier to use the acronym CREST instead of listing all the features. (pathologystudent.com)
  • hence the term CREST is mainly of historic interest, yet the acronym has great instructive value. (rheumaknowledgy.com)
  • The CRST syndrome is a recently defined symptom-complex consisting of (1) calcinosis cutis, (2) Raynaud's phenomenon, (3) sclerodactyly and (4) telangiectasis closely resembling hereditary hemorrhagic telangiectasis. (annals.org)
  • Disproportionately severe calcinosis cutis in an 88-year-old patient with CREST syndrome. (radiopaedia.org)
  • But in this case it usually quite clear in terms of physical changes in the hand appearance, low mobility in joints, quite strong Raynaud syndrome (many of us have it becasue of generally bad microcirculation), calcinosis (formation of hard underscim calcium salts deposits under the skin), cracking skin etc. (aboutbfs.com)
  • Other symptoms of CREST syndrome can be exhaustion, weakness, difficulties with breathing, pain in hands and feet, dizziness and badly healing wounds. (wikipedia.org)
  • Crest syndrome involves the production of autoimmune anti-nuclear and anti-centromere antibodies, though their cause is not currently understood. (wikipedia.org)
  • Antibodies: Anticentromere Antibodies (ACA) are the most common antibodies found in CREST Syndrome. (sclero.org)
  • CREST syndrome is related to antibodies that combat centromeres (regions of DNA chromosomes) like ones in the kidneys and lungs. (medicalmalpracticehelp.com)
  • A minority of CREST patients have anti-RNP or anti-SCL-70 antibodies. (rheumaknowledgy.com)
  • However, CREST syndrome is more closely associated with anti-centromere antibodies. (wikipedia.org)
  • Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). (springermedizin.de)
  • Also called CREST syndrome or progressive systemic sclerosis, scleroderma affects the connective tissue . (encyclopedia.com)
  • Several authors recognized this limitation and responded by categorizing patients with scleroderma syndromes into 2 groups: those with diffuse cutaneous scleroderma and those with a limited form of scleroderma. (medscape.com)
  • When CREST symptoms appear along with another form of Scleroderma, it is referred to as, for example, "Limited Scleroderma plus CREST" or "Diffuse Scleroderma plus CREST. (sclero.org)
  • The patient was diagnosed with Barrett's adenocarcinoma and CREST syndrome, which is a form of scleroderma. (springermedizin.de)
  • There is a more severe form of scleroderma that involves more than just the CREST symptoms, which has a much poorer prognosis. (timesunion.com)
  • Those with longstanding CREST syndrome are at greatest risk for developing progressive pulmonary hypertension, usually late in the disease. (rheumaknowledgy.com)
  • The rapidly fatal 'scleroderma-pulmonary-renal syndrome (SPRS)' may develop, which manifests as a fulminant course of acute normotensive renal failure associated with diffuse alveolar hemorrhage. (derangedphysiology.com)
  • Pulmonary edema caused by inhaled nitric oxide therapy in two patients with pulmonary hypertension associated with the CREST syndrome. (nih.gov)
  • Severe isolated pulmonary hypertension as main manifestation of Crest syndrome]. (nih.gov)
  • CREST syndrome and pulmonary hypertension: a dark prognosis]. (nih.gov)
  • Combination therapy with oral sildenafil and beraprost for pulmonary arterial hypertension associated with CREST syndrome. (nih.gov)
  • Pulmonary hypertension in the CREST syndrome: variant of systemic sclerosis (scleroderma)--a case report. (nih.gov)
  • Raynaud's phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. (wikipedia.org)
  • Of note, Raynaud's phenomenon is quite common in both PSS and CREST, and can be seen ini up to 10% of female non- smokers. (rheumaknowledgy.com)
  • The R in CREST stands for Raynaud's Phenomenon. (chronicallyelle.blog)
  • These cases are referred to as undifferentiated connective tissue disease or overlap syndrome. (medlineplus.gov)
  • Overlap syndrome-May involve features of scleroderma and other connective tissue syndromes. (epnet.com)
  • Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. (epnet.com)
  • To our knowledge, there are no reports of a small lymphocytic lymphoma (SLL) in association with limited cutaneous systemic sclerosis with classic features of the CREST syndrome. (elsevier.com)
  • Most craniofacial disorders are associated with defects in a migratory stem and progenitor cell population, which is designated the neural crest (NC). (sigmaaldrich.com)
  • [ 5 ] reviewed 13 patients with CREST and CRST syndromes and found the syndromes equivalent. (medscape.com)
  • CREST syndrome can be noted in up to 10% of patients with primary biliary cholangitis. (wikipedia.org)
  • Read personal stories from CREST patients and caregivers of CREST patients. (sclero.org)
  • Due to the general symptoms CREST syndrome patients may feel such as indigestion and difficulty in swallowing, many misdiagnoses and delayed treatment as a result. (medicalmalpracticehelp.com)
  • In the pleiotropic Bardet-Biedl syndrome (BBS), a primary ciliopathy with intraflagellar transport dysfunction, patients have a characteristic facial "gestalt" that dysmorphologists have found difficult to characterize. (uniprot.org)
  • Here, we use dense surface modeling (DSM) to reveal that BBS patients and mouse mutants have mid-facial defects involving homologous neural crest-derived structures shared by zebrafish morphants. (uniprot.org)
  • Clinical, serological and genetic study in patients with CREST syndrome. (springermedizin.de)
  • To better delineate neural crest defects in CHARGE syndrome, we generated induced pluripotent stem cells (iPSCs) from two patients with typical syndrome manifestations, and characterized neural crest cells differentiated in vitro from these iPSCs (iPSC-NCCs). (researchmap.jp)
  • These results support the historical inference that CHARGE syndrome patients exhibit defects in neural crest migration, and provide the first successful application of patient-derived iPSCs in modeling craniofacial disorders. (researchmap.jp)
  • We report 2 cases of thoracic neural crest tumors complicating the course in patients with Beckwith-Wiedemann syndrome (BWS). (semanticscholar.org)
  • Abnormality of chromosome 11 in patients with features of Beckwith-Wiedemann syndrome. (semanticscholar.org)
  • In patients affected by Horner syndrome, there is sometimes a slight elevation of the lower eyelid, a condition known as upside-down ptosis, notes Mayo Clinic. (reference.com)
  • One hundred sixty-one cases of DiGeorge syndrome (111 previously reported in which details concerning individual patients were given and 50 observed) were analyzed for occurrence and type of cardiovascular anomalies. (meta.org)
  • Therefore, in about half of the patients with DiGeorge syndrome the major anomaly was one that is rare. (meta.org)
  • Conversely, of those patients with interrupted aortic arch, 68% had DiGeorge syndrome, as did 33% of all patients with truncus arteriosus. (meta.org)
  • Although tetralogy of Fallot was also seen often in DiGeorge syndrome (10 patients), these cases represented less than 2% of the total number of cases of tetralogy of Fallot. (meta.org)
  • Patients with CREST/limited scleroderma can have a normal life expectancy. (timesunion.com)
  • ACA was present in 44% of the CREST patients as opposed to only 12% of those with SS and diffuse skin involvement. (springer.com)
  • While less than half the CREST patients had ACA, this antibody appears to identify those patients within the CREST variant with skin involvement confined to sclerodactyly as opposed to those with acrosclerosis. (springer.com)
  • In the office during your consultation you may describe the pain as severe at times and radiating out from the center of your low back to the top of your hip or pelvis, (the iliac crest) and into your groin region. (caringmedical.com)
  • These names may include Iliolumbar syndrome or Iliac crest pain syndrome. (caringmedical.com)
  • These defects of the craniofacial (CF) skeleton arise from aberrant cranial neural crest cell (NCC) migration. (uniprot.org)
  • The cranial neural crest arises from the forebrain, midbrain and hindbrain. (els.net)
  • Differential diagnoses include Sharp syndrome, systemic lupus erythematosus, antiphospholipid syndrome, and polyarteritis nodosa (see these terms). (nih.gov)
  • Yes the KD was by genetic blood test but the general dr thought my joint annoyance and swallowing mobility and stiffness was due to crest scleradoma, he mentioned both names. (aboutbfs.com)
  • DiGeorge syndrome is a genetic condition that affects chromosome 22, states Mayo Clinic. (reference.com)
  • Mount Sinai researchers have been awarded a $3.2 million grant from the National Institutes of Health to pursue a deeper understanding of Down syndrome, the most common genetic cause of intellectual and developmental disabilities in children and young adults, affecting more than 200,000 individuals in the United States. (news-medical.net)
  • The study showed that it truly is not just the surge of estrogen on a regular period it is even crest syndrome thrombocytopenia better to know suffer from it these days. (mythrombocytopenia.net)
  • Rid your body clear fat and was routines specifically on the face headache myogenic headache crest syndrome thrombocytopenia traction and help add immeasurably to a more energy. (mythrombocytopenia.net)
  • It is crest syndrome thrombocytopenia important flavonoids in the bloodstream it can do for you. (mythrombocytopenia.net)
  • CREST syndrome, also known as limited scleroderma , is a widespread connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs. (hellodoktor.com)
  • A person may also have any other autoimmune disease " plus CREST. (sclero.org)
  • Characterized as an autoimmune disease, the syndrome severely affects and attacks the body's connective tissues, the skin, muscles, blood vessels, the digestive tract others parts and organs of the body. (medicalmalpracticehelp.com)
  • CREST syndrome is believed to be an autoimmune disease. (autoimmunityblog.com)
  • Inhibition of neural crest migration underlies craniofacial dysmorphology and Hirschsprung's disease in Bardet-Biedl syndrome. (uniprot.org)
  • CREST syndrome , commonly know as limited Scleroderma, mainly affects the connective tissue of your body, which in turn causes various other health disorders. (healthwatchcenter.com)
  • Gilbert's syndrome is a common condition that affects the liver's ability to process bilirubin, according to Mayo Clinic. (reference.com)
  • Cryoglobulinemic vasculitis in a patient with CREST syndrome. (nih.gov)
  • CREST syndrome symptoms may be misdiagnosed with other conditions such as rheumatoid arthritis or gastrointestinal disorders. (medicalmalpracticehelp.com)
  • In 1979, CRST was expanded to CREST by Shulman's group, also at Johns Hopkins, who added esophageal involvement to the cardinal manifestations. (sclero.org)
  • Diffuse Systemic Sclerosis is distinguished from the localized variant (CREST) mainly based on the extent of cutaneous symptoms which are more extensive and prevalent in diffuse sclerosis. (statpearls.com)
  • There are two main types, limited disease (CREST syndrome) and diffuse disease. (medlineplus.gov)
  • Heterozygous mutations in the gene encoding CHD7, an ATP-dependent chromatin remodeler homologous to the Drosophila trithorax group protein Kismet, result in a complex constellation of congenital anomalies called CHARGE syndrome, a sporadic, autosomal dominant disorder characterized by malformations of the craniofacial structures, peripheral nervous system, ears, eyes and heart. (grantome.com)
  • Neural crest is a transient cell population that is ectodermal in origin, but undergoes a major transcriptional reprogramming to acquire a remarkably broad differentiation potential and ability to migrate throughout the body to give rise to craniofacial bones and cartilages, peripheral nervous system, and cardiac structures. (grantome.com)
  • Research proposed here will uncover molecular and cellular mechanisms underlying CHARGE syndrome, a leading cause of deaf-blindness, congenital heart disease and craniofacial malformations. (grantome.com)
  • Craniofacial development requires the co‐ordinated integration of signals from the endoderm, mesoderm, ectoderm, neuroectoderm and neural crest cells (NCCs). (els.net)
  • CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. (wikipedia.org)
  • Preliminary work implicated defective formation of the neural crest in CHARGE syndrome etiology. (grantome.com)
  • Inflammation of the vascular system from Behcet's syndrome can cause blood clots or even aneurysms, according to Mayo Clinic. (reference.com)
  • Syndrome of fetal gigantism, renal hamartomas, and nephroblastomatosis with Wilms' tumor. (semanticscholar.org)
  • For example, a patient with Calcinosis and Raynaud's would have CREST (which for precision may also be written as CRest, but it is CREST nonetheless. (sclero.org)
  • We present an unusual case of acute central retinal artery occlusion and cotton wool spots as the presenting feature in a patient subsequently diagnosed with systemic sclerosis, in particular with features of CREST syndrome. (biomedcentral.com)
  • The patient was a 52-year-old man with a 20-year history of limited scleroderma, as well as antiphospholipid syndrome. (reumatologiaclinica.org)
  • Crest syndrome in a male patient. (bvsalud.org)
  • It is believed that CREST syndrome is an autoimmune disorder, in which the immune system of one's body turns against its own. (medicaladviceplus.com)
  • The immune system in people with CREST syndrome appears to stimulate cells called fibroblasts to produce excess amounts of collagen. (hellodoktor.com)
  • CHARGE syndrome is caused by heterozygous mutations in the chromatin remodeler, CHD7, and is characterized by a set of malformations that, on clinical grounds, were historically postulated to arise from defects in neural crest formation during embryogenesis. (researchmap.jp)
  • Thickening generally only involves the skin of the fingers distal to the metacarpophalangeal joints in CREST. (wikipedia.org)
  • Symptoms for Reynaud's syndrome, for instance, include coldness, numbness in fingers and toes, and pain in different parts of the body. (medicalmalpracticehelp.com)
  • Most visible signs of CREST syndrome like tight, thick skin on your fingers and face can change the appearance of your skin. (healthwatchcenter.com)
  • Try to identify the early signs of CREST syndrome, like numbness, pain or even color changes in your fingers as well as gradual thickening of skin. (healthwatchcenter.com)