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

Susan Uthup, Radhika Chemmangattu Radhakrishnan


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.

Authors' affiliations

Susan Uthup, Department of Paediatric Nephrology, SAT Hospital, Government Medical College, Thiruvananthapuram, Kerala, India

Radhika Chemmangattu Radhakrishnan, Department of Paediatrics, SUT Academy of Medical Sciences, Thiruvananthapuram, Kerala, India

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Hemolytic uraemic syndrome; HUS; D- HUS; atypical HUS; thrombotic microangiopathy; plasmapheresis; chronic kidney disease

Cite this article

South African Journal of Child Health 2014;8(2):68-71. DOI:10.7196/SAJCH.635

Article History

Date submitted: 2013-07-19
Date published: 2014-02-05

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