Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic mastocytosis is an uncommon disorder of mast cell proliferation in connective tissues. Mast cell degranulation may occur on exposure to various stimuli and drugs. The release of histamine, heparin and vasoactive substances such as prostaglandin D2 may cause severe hypotension and other anaphylactoid manifestations. Anaesthetic management should include perioperative stabilization of mast cells and avoidance of the use of histamine-releasing drugs. Intradermal skin testing is useful in predicting the sensitivity to drugs that may be used during anaesthesia. We present a patient with systemic mastocytosis who underwent uneventful cholecystectomy.
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PMID:Anaesthetic management of systemic mastocytosis. 222 49

Systemic mastocytosis is characterized by an abnormal proliferation of tissue mast cells. Though rarely a surgical disease, it occasionally presents as variceal bleeding secondary to portal hypertension. Ultrastructural studies of the liver and spleen and portal pressure measurements support the hypothesis that a perisinusoidal intrahepatic fibrosis is responsible for the increased portal pressure. When variceal bleeding complicates systemic mastocytosis, shunt surgery is indicated, with the type of shunt dictated by both hematologic and hemodynamic issues. Satisfactory blockade of histamine release can be achieved preoperatively by disodium cromoglycate and/or histamine antagonists to obviate any systemic effects precipitated by shunting of mast cell-rich splenic blood into the systemic circulation.
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PMID:Variceal bleeding, hypersplenism, and systemic mastocytosis. Pathophysiology and management. 313 Aug 25

Systemic mastocytosis associated with urticaria pigmentosa seems to be strikingly more common than previously assumed. The diagnosis can be established by the investigation of bone marrow sections, whereas bone marrow smears are less reliable. Some mediators are produced by the enhanced number of mast cells; telemethyl imidazole acetic acid is the most suitable mediator to calculate the size of the mast cell pool. Investigations like this might offer an alternative to the examination of bone marrow sections in future.
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PMID:[Systemic mastocytosis]. 323 39

Systemic mastocytosis is a rare disorder characterised by the infiltration of many tissues by abnormal numbers of mast cells. Life-threatening episodes of bronchospasm and hypotension are common in response to a variety of triggers which cause mast cell degranulation. We present the case history of a patient with severe mast cell disease who successfully underwent splenectomy and portocaval anastomosis. Peri-operative therapy was directed towards mast cell stabilisation, and histamine-releasing drugs were avoided. Cardiovascular data, together with blood histamine levels and thrombin times, indicated that mast cell degranulation did not occur. A review of the current literature concerning mast cell disease as it relates to anaesthesia is presented and suggestions for the safe management of such cases are given.
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PMID:Histamine levels and cardiovascular responses during splenectomy and splenorenal shunt formation in a patient with systemic mastocytosis. 366 31

Systemic mastocytosis is a disease characterized by an excessive accumulation of mast cells, and associated with skin lesions, flushing, diarrhea, tachycardia, and psychiatric manifestations. In order to define more clearly the psychiatric manifestations, ten patients with this disorder underwent unstructured psychiatric interviews and a battery of psychologic testing. Both revealed a pattern of cognitive and affective changes in the majority of these patients, best categorized as an atypical or mixed organic brain syndrome. The cognitive changes consisted of diminished attention and memory, and the affective changes of anger, irritability, and, to a lesser extent, depression. These manifestations fluctuated with the level of disease activity, and appeared in some cases to respond to histamine antagonists and disodium cromoglycate, medications used to control the excessive mast cell activity. It is important for psychiatrists to be aware that mental status changes can represent psychiatric manifestations of mastocytosis, a readily treatable medical disorder.
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PMID:Mixed organic brain syndrome as a manifestation of systemic mastocytosis. 374 21

Systemic mastocytosis is an uncommon disorder due to multiorgan infiltration by mast cells. The authors report the case of a man whose mastocytosis was revealed in an unusual way by hepatomegaly and portal hypertension of the sinusoidal type. This case was also characterized by the absence of urticaria pigmentosa, the presence of seborrheic warts in which mast cell infiltration was noted and the absence of digestive symptoms. The peculiarities of this case are compared to the published data.
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PMID:[Hepatomegaly with portal hypertension indicative of systemic mastocytosis]. 376 29

Systemic mastocytosis, with its diffuse infiltration of mast cells into various organs, has resulted in intestinal malabsorption and bleeding diatheses. The pathophysiology underlying these phenomena is unclear, but may be related to the release of histamine and heparin containing mast cell granules. A patient with systemic mastocytosis had malabsorption and developed massive bleeding after percutaneous liver biopsy. Histologic involvement of skin, duodenum, rectum, liver, and bone marrow was documented. Mastocytosis should be considered in the differential diagnosis of malabsorption.
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PMID:Bleeding after liver biopsy in a patient with systemic mastocytosis and malabsorption. 404 81

Systemic mastocytosis is a multiorgan disease that most commonly affects the skin and skeletal system. Radiographically, the skeletal changes in the majority of patients consist of either a wide-spread mixture of bone lysis and osteosclerosis or generalized osteoporosis. The osteoporotic form is less well known but may lead to severe generalized demineralization and pathologic fractures. Mast cells secrete a number of substances, two of which (heparin and prostaglandins) are believed to have a role in the induction of osteoporosis. Sclerotic lesions are induced by another mast cell by-product, histamine. One hundred seventy-eight cases of bony mastocytosis have been reported in the literature, including the four patients in the present report. Special staining procedures are necessary for identification of mast cells. Diagnosis may be delayed in patients who do not have the skin lesions (urticaria pigmentosa) and in the osteoporotic form of the disease.
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PMID:Pathologic fracture in systemic mastocytosis. Radiographic spectrum and review of the literature. 635 46

Systemic mastocytosis is a rare disease characterized by mast cell infiltration of organs. Bony pain is present in up to 28% of cases and is frequently chronic and difficult to palliate. Historical attempts at pain control have exclusively involved medical therapy. We report three cases of refractory bone pain in two patients with advanced systemic mast cell disease and associated bony involvement, which were treated with radiotherapy. This report represents some of the first uses of radiotherapy in this disease. Two patients with a primary diagnosis of systemic mastocytosis and bony pain unresponsive to medical therapy were referred for palliative radiotherapy. In the first case, referral was made because of a painful thoracolumbar spine and left shoulder, and in the second, for bilateral lower extremity pain. Patients were irradiated on megavoltage equipment to 30 Gy in 200 and 300 cGy daily fractions. For the first patient, treatment reduced pain scores from 8/10 (severe) to 3-4/10 (moderate) by 1 month posttreatment, with subsequent varying pain until his death 4 months after his second treatment. The second patient achieved pain relief from 10/10 pretreatment to 1-2/10 while on treatment. This proved durable for 9 months until her death due to disease progression. The first patient had a slight exacerbation of his thrombocytopenia during his initial treatment, but otherwise neither patient experienced any acute complications from the radiation treatments. When patients with advanced systemic mastocytosis require large narcotic doses for incomplete bone pain control associated with demonstrable bony involvement, the relatively slight risks of palliative radiation to bone may be favorably weighed against the likelihood of pain relief.
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PMID:Radiotherapy of refractory bone pain due to systemic mast cell disease. 751 91

A 75-year-old woman with known systemic mastocytosis presented with abdominal pain, ascites, and bile duct thickening on computed tomography and ultrasonography. A liver biopsy specimen showed infiltration with mast cells. Endoscopic retrograde cholangiography showed ductal changes compatible with those found in primary sclerosing cholangitis. Brush cytology of the intrahepatic bile ducts confirmed mast cell infiltration. Systemic mastocytosis can infiltrate the biliary system, producing a cholangiopathy radiographically similar to primary sclerosing cholangitis.
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PMID:Mast cell cholangiopathy: another cause of sclerosing cholangitis. 755 53


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