Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q16637 (SMA)
 8,107 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Autoimmune hepatitis is a chronic inflammatory liver disorder of unknown etiology associated with serum autoantibodies and hypergammaglobulinemia. This disease has a broad spectrum of presentations ranging from asymptomatic to fulminant hepatic failure. A 36 year old female with past history of hypothyroidism developed jaundice 2 months prior to admission. Outpatient evaluation revealed ANA and anti-SMA antibodies in high titers, negative viral markers for hepatitis, and hypergammaglobulinemia. A presumptive diagnosis of autoimmune hepatitis was made; steroids were recommended but the patient did not take them. She was admitted to the University Hospital due to increased jaundice, general malaise and ascites 5 weeks later. She deteriorated developing coagulopathy, encephalopathy and increasing hyperbilirubinemia. Intravenous corticosteroids were started. The patient improved and was discharged 3 weeks after admission. Fulminant hepatic failure has a high mortality and may require liver transplant. Our patient survived fulminant hepatic failure that resolved after corticosteroid therapy. It is important to identify and distinguish autoimmune hepatitis from other forms of liver disease because of the high percentage of response to immuno-suppressive therapy. Early diagnosis and treatment of this condition could improve survival, quality of life, and defer liver transplantation.
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PMID:Steroid therapy in fulminant hepatic failure secondary to autoimmune hepatitis. 988 78

A 70-year-old woman presented with impaired memory and depressive symptoms and two women aged 53 and 30 years, respectively, presented with general malaise and fatigue. All were diagnosed with and treated for autoimmune hepatitis (AIH). The first patient developed a relapse during treatment withdrawal; she recovered and maintained remission after the initial dose of medication had been restarted and the medication was tapered more gradually. The second patient had an incomplete remission and later developed liver failure; she was eligible for a liver transplant. The third woman became pregnant during treatment and developed a relapse after delivery; remission was induced and maintained after the immunosuppression was temporally increased. AIH is a chronic progressive liver disease characterised by abnormal serum levels of liver enzymes, hypergammaglobulinaemia, auto-antibodies against cell nuclei (ANA), smooth muscle (SMA), or liver and kidney microsomes (LKM), interface hepatitis and the absence of other chronic liver disease. Early diagnosis is essential because therapy can markedly improve prognosis. However, there is no specific diagnostic test for AIH. It is important to induce and maintain remission with immunosuppressive therapy.
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PMID:[Three patients with autoimmune hepatitis: the importance of early diagnosis and remission]. 1646 22