The renal manifestations and outcome of @entity2106 / XXXX in adults.

@entity1 @entity39 @entity16 @entity11849 @entity810 @entity1381 @entity1011 @entity204 @entity2106
@entity2106 / @entity1381 ( @entity2106 / @entity11849 ) is generally regarded to be a rare disease. The present study was undertaken to identify presenting features, prognostic variables, pathological features and outcome associated with @entity2106 / @entity11849 . The present study is a retrospective chart review of 68 @entity1 treated with plasmapheresis for @entity2106 / @entity11849 at a single tertiary referral medical institution from 1980-1992. The annual number of @entity1 with @entity2106 / @entity11849 treated with plasmapheresis increased from an average of one case per year in 1980 to nine cases per a year in 1992. The in-hospital mortality for @entity1 presenting with @entity2106 / @entity11849 was 25%. Forty four percent of @entity1 presented with an elevated serum @entity1011 , and 16% required hemodialysis support. Of the seven @entity1 who survived and required hemodialysis support only two @entity1 continued on dialysis. None of the @entity1 presenting with a normal serum @entity1011 required dialysis at any time in their course. @entity1 age, sex, presenting platelet count, white blood cell count, hemoglobin level and presence of @entity16 were not significantly associated with @entity204 or need for dialysis. The histopathological features of @entity2106 / @entity11849 (fibrin/platelet thrombi in renal vessels and glomeruli, fibrinoid @entity39 of vessel walls) were found in all five cases autopsied. The incidence of @entity2106 / @entity11849 may be increasing. Alternative possibilities for the increased frequency of cases seen include greater diagnostic suspicion and referral bias. Despite the use of plasmapheresis, mortality during the initial hospital admission was almost 25%. In retrospect prognosis could not be predicted based on admission biochemical or clinical variables. The majority of @entity1 who developed @entity810 and survived to hospital discharge recovered renal function and became independent of dialysis.
