Clinical profile and outcome of D- haemolytic-uraemic syndrome in children from south India

Abstract

Normal 0 false false false EN-IN X-NONE X-NONE Background. Haemolytic-uraemic syndrome (HUS) occurring without a diarrhoeal prodrome is termed D- HUS and has a poorer prognosis than D+ HUS, with high mortality and potential for long-term renal and non-renal morbidity. Methods. We studied nine children with D- HUS from the Pediatric Nephrology division of the Medical College, Trivandrum, India, over a period of 5 years. The clinical, haematological and renal profiles of all patients were recorded. All patients were treated with fresh-frozen plasma with or without dialysis. Results. The aetiology of HUS was not apparent in any of the cases, except in one patient who had a history of ayurvedic treatment for chronic cough. The mean age of the patients was 7.5 years with a male:female ratio of 1:2. Hypertension (HT) was present in eight cases (88%). Plasmapheresis was performed in 22% of patients; 67% underwent dialysis. Renal biopsy was performed in six patients. Four patients (44%) had good renal recovery while two progressed to end-stage renal disease. One child died in the acute phase, and one had a relapse of HUS. HT persisted in 44% and proteinuria in 33% of patients. Conclusion. Early comprehensive management including dialysis, plasma infusions, and aggressive management of HT yields a good outcome in D- HUS. Persistent HT and progressive, chronic kidney disease require long-term management. /* Style Definitions */ table.MsoNormalTable {mso-style-name:Table Normal; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin:0cm; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:Calibri,sans-serif; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:Times New Roman; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Times New Roman; mso-bidi-theme-font:minor-bidi;}