Hemolytic uremic syndrome in laboratory medicine: single centre experience in children.

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 create-nine (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.