[Recurrent hemolytic-uremic syndrome with positive immunofluorescence].
The hemolytic uremic syndrome is a disease of infancy, its major clinical manifestations include reversible thrombocytopenia, hemolytic anemia, and renal failure. Although a great number of patients with HUS have been published, relapses as well as positive immunofluorescence studies are rare findings. In our patient the disease began at age of 7 years and recovered completely. At 10 1/2 years a relapse occurred and despite therapy the patient died two months later. Renal biopsy showed severe arterial and glomerular changes with remarkable similarity to the histological findings in thrombotic thrombocytopenic purpura, which could be explained as secondary hypertensive damage, and dense granular deposition of fibrinogen, IgG, IgA, C3, and Clq along the capillary loops of the glomerulus and throughout the wall of the renal arteries. The clinical data, histological findings, and the particularities of our patient with this special course of HUS are discussed.
D002648:Child; D003166:Complement Activating Enzymes / Q000032:analysis; D015922:Complement C1q; D003176:Complement C3 / Q000032:analysis; D005455:Fluorescent Antibody Technique; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007668:Kidney / Q000473:pathology; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000473:pathology; D012008:Recurrence
D003176:Complement C3; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D015922:Complement C1q; D003166:Complement Activating Enzymes
Rezidivierendes hämolytisch-urämisches Syndrom mit positiver Immunfluoreszenz.