Clinical profile and outcome of D- haemolyticuraemic syndrome in children from south India
AbstractBackground. 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.