Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presence of high serum concentrations of immunoglobulins and hypocomplementemia is commonly observed in alcoholic patients; however, the mechanism behind their production is unknown. We studied 70 subjects (52 alcoholics and 18 healthy controls) prospectively. All the patients were active drinkers who consumed more than 100 gr of ethanol daily for at least 10 years. A biopsy of the liver was performed for all the subjects, along with immunoglobulins, C3, C4, CH100 and tests of liver function. Of the 52 alcoholics in the study, 20 showed minimal changes in hepatic steatosis (group II), 6 isolated severe alcoholic hepatitis (group III), 11 cirrhosis of the liver without alcoholic hepatitis (group IV), and 15 cirrhosis with alcoholic hepatitis (group V). There were no significant differences between the various groups according to age or quantity and duration of intake. The highest concentrations of immunoglobulins was observed in those with cirrhosis of the liver (p < 0.001) regardless of the degree of inflammation. The best correlation was found between IgA and liver histology (r = 0.64; p < 0.001). Hypocomplementemia was a factor only in those patients belonging to histological groups IV and V. Both the immunoglobulins and the complement proteins were narrowly correlated with the tests of liver function, and its alterations were more pronounced in patients with cirrhosis in Child's stage C. We conclude that the hypergammaglobulinemia and hypocomplementemia observed in the alcoholic patients are conditioned fundamentally by the degree of deterioration in liver function, indicated by cirrhosis of the liver upon presentation.
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PMID:[Serum levels of immunoglobulins and complement in alcoholic liver disease]. 811 93

Autoimmune hepatitis (AIH) is a chronic progressive liver disease characterized by high levels of aminotransferases and autoantibodies, hypergammaglobulinemia, and interface hepatitis. AIH affects all races and all ages worldwide, regardless of sex, although a preponderance of females is a constant finding. The etiology of AIH has not been completely elucidated, but immunogenetic background and environmental parameters may contribute to its development. The most important genetic factor is human leukocyte antigens (HLAs), especially HLA-DR, whereas the role of environmental factors is not completely understood. Immunologically, disruption of the immune tolerance to autologous liver antigens may be a trigger of AIH. The diagnosis of classical AIH is fairly easy, though not without pitfalls. In contrast, the diagnosis of atypical AIH poses great challenges. There is confusion as to the definition of the disease entity and its boundaries in the diagnosis of overlap syndrome, drug-induced autoimmune hepatitis, and AIH with concomitant nonalcoholic fatty liver disease (NAFLD) or chronic hepatitis C. Centrilobular zonal necrosis is now included in the histological spectrum of AIH. However, the definition and the significance of AIH presenting with centrilobular zonal necrosis have not been examined extensively. In ~20% of AIH patients who are treated for the first time with standard therapy, remission is not achieved. The development of more effective and better tolerated novel therapies is an urgent need. In this review, we discuss the current challenges and the future prospects in relation to the diagnosis and treatment of AIH, which have been attracting considerable recent attention.
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PMID:Autoimmune hepatitis: current challenges and future prospects. 2817 94

Gaucher disease (GD), a rare lysosomal storage disorder caused by deficient glucocerebrosidase activity and consequent accumulation of glycosphingolipids in the mononuclear phagocyte system, may progress to disabling and potentially life-threatening complications when left undiagnosed and untreated. Unfortunately, because of non-specific signs and symptoms and lack of awareness, patients with type 1 GD, the most common non-neuropathic variant, frequently experience diagnostic delays. Since splenomegaly and thrombocytopenia are the dominant clinical features in many GD patients leading to first medical contact, the hepatologist and the gastroenterologist need to be aware of this condition. Liver involvement has been reported in the majority of GD patients, and comprises hepatomegaly, with or without liver enzymes alteration, fibrosis/cirrhosis, portal hypertension, focal liver lesions, and cholelithiasis. Moreover, GD is associated with several biochemical alterations of potential interest for the hepatologist and the gastroenterologist, including hypergammaglobulinemia, hyperferritinemia and metabolic abnormalities, that may lead to misdiagnoses with chronic liver diseases of common etiology, such as primary hemochromatosis, autoimmune liver diseases or nonalcoholic fatty liver disease. This comprehensive review, based on the collaborative experience of physicians managing patients with GD, provides practical information on the clinical, histological and radiological hepatic manifestations of GD aiming at facilitating the diagnosis of GD for the hepatologist and the gastroenterologist.
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PMID:Liver involvement in Gaucher disease: A practical review for the hepatologist and the gastroenterologist. 3205 84