Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020473 (hyperlipidemia)
 15,891 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 25-year-old man was at first dermatologically suspected as suffering from Weber-Christian syndrome because of subcutaneous panniculitis, but his skin lesions disappeared completely during the course. Hyperlipidemia, disturbances in liver function, and leukemoid reaction became remarkable and he died of subarachnoid hemorrhage eleven months after onset. Necropsy revealed subarachnoid hemorrhage at the base of the brain, lipogranulomatous and inflammatory lesions in the upper lobe of the left lung, a remarkable fatty liver, splenomegaly, pericarditis, and foam cells in the spleen, liver, and bone marrow. A comparison with 57 autopsy cases of Weber-Christian syndrome reported in the literature showed our case to be an exceptional instance of Weber-Christian syndrome, if the present case is not to be regarded as a different disease entity.
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PMID:An unusual case which began with subcutaneous panniculitis followed by fever, severe hepatic involvement and hyperlipidemia. 87 Oct 84

A case of a 21-year-old woman with Weber-Christian disease, associated with consumption coagulopathy and adequately controlled over the past year or more by corticosteroid and heparin therapy, has been described. Through the study on this case, it was concluded that a hemorrhagic diathesis complicating Weber-Christian disease, at least in advanced cases, was characterized by consumption coagulopathy associated with enhanced blood coagulability which arises from hyperlipemia due to abnormal lipid metabolism and further, that the consumption coagulopathy was modified by diminished synthesis of clotting factors and activation of the fibrinolytic system due to the secondary hepatic impairment. It might also be likely that systemic vascular changes contribute to the activation of the clotting system as a trigger of clotting factor consumption. The complex pathophysiology of a hemorrhagic diathesis in this disease seemed to be brought about by a complex combination of such a variety of factors. In addition, decreased erythrocyte sedimentation rate observed characteristically during hemorrhage might probably have a direct correlation with hypofibrinogenemia due to consumption coagulopathy in advanced cases.
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PMID:Hemorrhagic diathesis in Weber-Christian disease. 96 27

Hyperlipidemia of initially Type V and finally of Type III was observed in a patient with Weber-Christian disease. The lipoprotein lipase (LpL) and hepatic triglyceride lipase (HTGL) activities of the post-heparin plasma were low, but detectable. Both lipase activities were higher when assayed with 20 microliters of post-heparin plasma than with more than 30 microliters, indicating the presence of some inhibitory factor in the plasma. This plasma inhibited purified LpL and HTGL from human post-heparin plasma. Zonal ultracentrifugation studies showed that the inhibitor of hepatic lipase was associated with the middle fraction of intermediate lipoprotein and low density lipoproteins (1.020 less than d less than 1.040). These results are consistent with the idea that dyslipoproteinemia in this patient was partially due to dysfunction of the catabolic system caused with an inhibitor of lipolytic enzymes.
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PMID:Dyslipoproteinemia and an inhibitor of lipolytic enzymes in Weber-Christian disease. 273 46

We describe an 8 year old female with Weber-Christian disease who manifested erythema on her legs and an intermittent high fever. A wedge skin biopsy revealed lobular panniculitis and bean-bag cells, which were characteristic to cytophagic histiocytic panniculitis (CHP). However, she showed no evidence of liver dysfunction, hyperlipidemia, or coagulation disorders usually seen in CHP. Moreover, she responded well to oral corticosteroids therapy with resultant advanced subcutaneous fat atrophy.
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PMID:Weber-Christian disease with benign cytophagic histiocytes in the skin lesion. 775 52

Non-alcoholic steatohepatitis (NASH) is one of the most common liver disorders. This is highly prevalent in obese and diabetic subjects. Persons with central obesity are at particular risk. Other clinical predictors are age more than 40-50 years and hyperlipidemias, but none of these factors is invariable for causation of NASH. Other reported associations are, celiac disease, Wilson's Disease and few other metabolic diseases. Drugs, particularly amiodarone, tamoxifen, nucleoside analogues and methotrxate have also been linked to NASH. The disease is evenly distributed in both sexes but advanced disease is more common in women. Ethnic variation exists and African Americans are less affected than Hispanic Americans. Specific clinical features of NASH are infrequent. Patients usually come to clinical attention by elevated liver enzymes found on routine evaluation but on history, about two third of patients will admit to have mild fatigue and about half will report right upper quadrant pain. Rarely, patient may present with a complication of cirrhosis. Physical examination may reveal hepatomegaly and splenomegaly. Research in last few years has stressed that development of steatosis, stetohepatitis, fibrosis with subsequent cirrhosis are most probably the result of insulin resistance. Therefore, clinical features may reflect existence of insulin resistance. Obesity, particularly central obesity is most important of these. Patients may have sleep apnea syndrome. Hypertension and manifestations of diabetes mellitus like polyuria, polydypsia, and neurological deficits may occur. Patients may have varying combination of obesity, diabetes, hyperlipidemia, hypertension and impaired fibrinolysis (syndrome X). Children with insulin resistance may show acanthosis nigricance. Patients with polycystic ovary syndrome, which consists of insulin resistance, diabetes, obesity, hirsutism, oligo or polymenorrha and hyperlipidemia may have NASH. Other rare manifestations of insulin resistance, which can be seen in patients of NASH are lipomatosis, lipoatrophy/lipodystrophy and panniculitis. Most other rare conditions known to cause NASH like peroxisomal diseases, mitochondialpathies, Weber-Christian disease, Mauriac syndrome, Madelung's lipomatosis and abetaliopprotenemia also have insulin resistance. This is believed that primary defect underlying insulin resistance is impairment in postreceptor pathways (through tyrosine kinase activity) of insulin action. Primary defect in insulin receptors appear uncommon. This results in down regulation of insulin receptor substance 1 (IRS-1) signaling by excess free fatty acids. In muscle, activated IRS-1 promotes translocation of glucose transporter protein 4 (GLUT4) to cell membrane. As a result, monocyte glucose uptake by GLUT4 increases glucose disposal from blood and reduced need for insulin. PKC-0 is a likely candidate as serine kinase in muscle regulated by fatty acids that can impair the activation of IRS-1. Insulin resistance is usually evaluated by fasting insulin levels, Quantitative Insulin Check Index (QUICKI) and Homeostasis Model Assessment of Insulin Resistance (HOMA), C-peptid/insulin ratio oral glucose tolerance test and hyper insulinemic euglycemic clamp. The clamp technique is considered the gold standard.
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PMID:Insulin resistance and clinical aspects of non-alcoholic steatohepatitis (NASH). 1619 20