Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired dysfibrinogenemia was documented in a 4-year-old child with obstructive jaundice of 1-month duration, secondary to a choledochal cyst involving the distal common bile duct. It was characterized by decreased thrombin coagulable protein with elevated immunoassayable fibrinogen resulting in abnormal thrombin and reptilase times. The liver morphology was compatible with extrahepatic obstruction, without evidence of cirrhosis or hepatocyte abnormality. All the coagulation abnormalities promptly resolved after surgical correction of the obstruction. Dysfibrinogenemia has been associated with serious liver disease in adults, including tumors, chronic active hepatitis, and cirrhosis, but never with isolated obstructive jaundice. This report documents a case of acquired dysfibrinogenemia due to extra-hepatic biliary obstruction and also emphasizes the importance of the consideration of this disorder in coagulation abnormalities associated with hepatobiliary disease.
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PMID:Dysfibrinogenemia in obstructive liver disease. 368 83

The fibrinogen molecule is becoming increasingly understood. Amino acid sequencing has been undertaken and studies of abnormal fibrinogens are leading to a more functional concept of its structure. Acquired dysfibrinogenemia appears to be a more common problem than previously thought, and may be found in patients with liver disease, cancer, fibrinolysis, and disseminated intravascular coagulation (DIC). While previous evidence suggested that anti-thrombin activity and fibrin polymerization inhibitors were formed in these conditions, recent studies suggest that slow fibrin formation occurs as a result of structural changes induced in the fibrinogen molecule itself. These relatively minor, alterations in structure cause a functional dysfibrinogenemia simulating abnormalities seen in some congenital fibrinogenopathies. A case is presented illustrating such a dysfibrinogenemia in a patient with cirrhosis of the liver and evidence for DIC.
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PMID:Fibrinogen and dysfibrinogenemia. 744 88