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Patel R.D.,G R Doshi And K M Mehta Ikdrc Dr Hl Trivedi Institute Of Transplantation Science Its | Vanikar A.V.,G R Doshi And K M Mehta Ikdrc Dr Hl Trivedi Institute Of Transplantation Science Its | Gumber M.A.,IKDRC ITS | Anodia K.V.,G R Doshi And K M Mehta Ikdrc Dr Hl Trivedi Institute Of Transplantation Science Its | And 3 more authors.
Clinical Laboratory | Year: 2013

Background: Atypical hemolytic uremic syndrome (aHUS), although rare, is the most common cause of acute renal failure (ARF) in children and has poor prognosis. We present a single centre experience of aHUS. Methods: Thirty six children (29 males, 7 females), with mean age 7.9 years, presented with ARF, 2 children also had tonic-clonic type convulsions. Their hematology examination revealed hemolytic anemia with serum creatinine (SCr) of 5.54 mg/dL. Acute HUS was observed in 75%, acute-on-chronic HUS in 19.4%, and patchy cortical necrosis (PCN) in 5.6% biopsies. A mean of 5.4 plasma exchanges (PE) were carried out. Supportive management of anti-hypertensives and prednisone was also given. Recovery end points were establishment of urine output and improvement of SCr and hematological profile. Results: Hematology and renal function profile improved variably in all children, 5.6% died, relapse was observed in 80.5% over a mean of 70 days; 13.9% children are doing well over a mean follow-up of 268.8 days. Thus, poor prognosis was observed in 86.1% children. Children with acute or chronic HUS and PCN did not recover. Six children who recovered had acute HUS. Conclusions: aHUS in Indian children occurs at an older age of around 8 years and chronic/irreversible changes on histopathology examination are harbingers of poor prognosis. PE is life-saving; however, further research for developing strategies to improve long-term survival is needed.

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