title: Adult hemolytic uremic syndrome with renal microangiopathy. Outcome according to therapeutic protocol in 53 cases. French Cooperative Study Group for Adult HUS.
abstract: We gathered data on adult patients suffering from hemolytic uremic syndrome with renal biopsy-proven thrombotic microangiopathy (n = 85) and excluded those associated with cancer, lymphoma, progressive systemic sclerosis and AIDS (n = 32). The 53 patients selected had a follow-up of more than 6 months (m = 38 months; range: 6-180 months). The aim of the study was to analyze the therapy-dependent outcome in these 53 patients. In such a retrospective and multicentric survey, different therapeutic protocols were used, including plasma exchange (PE), infusions of fresh-frozen plasma, corticosteroids, IV immunoglobulins or supportive therapy alone; all were used differently. The 5 year patient and kidney survival rates were 90 and 60%, respectively, for the selected group. A better patient survival rate was observed in the PE-treated group, compared to all other patients without PE therapy (p < 0.03). In addition, for patients requiring dialysis at presentation, renal improvement was better in the PE-treated group compared to patients receiving only supportive therapy (p = 0.05). When looking at the therapy-dependent outcome of renal function according to the pathologic type (glomerular, arteriolar or mixed), we observed that all PE-treated patients with pure glomerular lesions recovered, unlike those with arteriolar or mixed lesions (p = NS). On the other hand, no long-term beneficial effects of "active therapy" (37 patients) compared to supportive therapy alone (16 patients) were observed on renal function, but the 2 groups were not completely comparable.(ABSTRACT TRUNCATED AT 250 WORDS)
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007668:Kidney / Q000098:blood supply*; D008297:Male; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange; D012189:Retrospective Studies; D013927:Thrombosis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D013997:Time Factors