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Diagnosis and Management of Atypical Hemolytic Uremic Syndrome In Children: Single Centre Experience

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Abstract

Atypical hemolytic uremic syndrome (aHUS) although rare is the commonest cause of acute renal failure (ARF) in children and has poor prognosis. We present single centre experience of aHUS. 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 s. creatinine (SCr), 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. Mean 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, improvement of SCr and hematological profile. Hematology and renal function profile improved variably in all children, 5.6 % died, relapse was observed in 80.5 % over mean 70 days; 13.9 % children are doing well over mean follow-up of 268.8 days. Thus poor prognosis was observed in 86.1 % children. Children with acute on chronic HUS and PCN did not recover. Six children who recovered had acute HUS. 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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Abbreviations

ARF:

Acute renal failure

aHUS:

Atypical hemolytic uremic syndrome

CAPD:

Continuous ambulatory peritoneal dialysis

CBC:

Complete blood counts

CRF:

Chronic renal failure

FFP:

Fresh frozen plasma

Hb:

Hemoglobin

HUS:

Hemolytic uremic syndrome

IF:

Immunofluorescence

LDH:

Lactic dehydrogenase

MAHA:

Microangiopathic hemolytic anemia

PE:

Plasma exchange

SCr:

Serum creatinine

TLC:

Total leucocyte count

VWF:

Von Willebrand factor

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Authors and Affiliations

  1. Department of Pathology, Laboratory Medicine and Transfusion Services and Immunohematology, G. R. Doshi and K. M. Mehta Institute Of Kidney Diseases and Research Centre (IKDRC)-Dr. H.L. Trivedi Institute Of Transplantation Sciences (ITS), Civil Hospital Campus, Asarwa, Ahmedabad, 380016, Gujarat, India

    Rashmi D. Patel, Aruna V. Vanikar, Kamal V. Kanodia & Kamlesh S. Suthar

  2. Department of Nephrology and Transplantation Medicine, G. R. Doshi and K. M. Mehta Institute Of Kidney Diseases and Research Centre (IKDRC)-Dr. H.L. Trivedi Institute Of Transplantation Sciences (ITS), Civil Hospital Campus, Asarwa, Ahmedabad, 380016, Gujarat, India

    Manoj R. Gumber, Himanshu V. Patel & Hargovind L. Trivedi

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  1. Rashmi D. Patel
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  2. Aruna V. Vanikar
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  3. Manoj R. Gumber
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  6. Himanshu V. Patel
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  7. Hargovind L. Trivedi
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Correspondence to Rashmi D. Patel.

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Patel, R.D., Vanikar, A.V., Gumber, M.R. et al. Diagnosis and Management of Atypical Hemolytic Uremic Syndrome In Children: Single Centre Experience. Indian J Hematol Blood Transfus 30, 342–346 (2014). https://doi.org/10.1007/s12288-013-0262-3

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  • DOI: https://doi.org/10.1007/s12288-013-0262-3

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