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

  • Rashmi D. Patel
  • Aruna V. Vanikar
  • Manoj R. Gumber
  • Kamal V. Kanodia
  • Kamlesh S. Suthar
  • Himanshu V. Patel
  • Hargovind L. Trivedi
Original Article

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.

Keywords

Hemolytic uremic syndrome Thrombotic thrombocytopenic purpura Plasma exchange Microangiopathic hemolytic anemia 

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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Copyright information

© Indian Society of Haematology & Transfusion Medicine 2013

Authors and Affiliations

  • Rashmi D. Patel
    • 1
  • Aruna V. Vanikar
    • 1
  • Manoj R. Gumber
    • 2
  • Kamal V. Kanodia
    • 1
  • Kamlesh S. Suthar
    • 1
  • Himanshu V. Patel
    • 2
  • Hargovind L. Trivedi
    • 2
  1. 1.Department of Pathology, Laboratory Medicine and Transfusion Services and ImmunohematologyG. R. Doshi and K. M. Mehta Institute Of Kidney Diseases and Research Centre (IKDRC)-Dr. H.L. Trivedi Institute Of Transplantation Sciences (ITS)Asarwa, AhmedabadIndia
  2. 2.Department of Nephrology and Transplantation MedicineG. R. Doshi and K. M. Mehta Institute Of Kidney Diseases and Research Centre (IKDRC)-Dr. H.L. Trivedi Institute Of Transplantation Sciences (ITS)Asarwa, AhmedabadIndia

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