| Autor: |
Currimbhoy, Zinet, Kahn, Elle, Degnan, Thomas, Silverberg, Mervin |
| Zdroj: |
Indian Journal of Pediatrics; Jul1981, Vol. 48 Issue 4, p413-419, 7p |
| Abstrakt: |
A 12-year-old girl presented with a sudden onset of hepatic vein thrombosis (Budd-Chiari syndrome). Over the following two years she developed multiple thrombi. Coagulation studies performed during remissions revealed the presence of a lupus inhibitor, an anticoagulant against factor VIII, a deficiency in factors II and VII and a deficiency in plasminogen. Following two months of prednisone therapy, the lupus inhibitor and anti-factor VIII disappeared., Deficiencies in factor VII, II and plasminogen remained. The patient experienced a total of four episodes of ascites with coagulation defects, and a hypocomplementemia terminating in liver failure and death 26 months after onset. Autopsy findings included generalized vascular intimal hyperplasia associated with thrombosis. We concluded that the patient had an immune mediated systemic vasculitis with major involvement of the liver. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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