Atypical hemolytic-uremic syndrome - A case series from a tertiary care hospital from Eastern India

• Main Authors: Mukesh Kumar Jain (Author), Nikunj Kishore Rout (Author), Amit Ranjan Rup (Author), Sibabratta Patnaik (Author), Chinmay Kumar Behera (Author), Reshmi Mishra (Author), Bandya Sahoo (Author)
• Format: Book
• Published: Wolters Kluwer Medknow Publications, 2021-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2349-6592; 2455-7099; 10.4103/jpcc.jpcc_184_20
• Keywords: atypical hemolytic-uremic syndrome; eculizumab; mycophenolate; plasma exchange; Pediatrics; RJ1-570
• Source: Journal of Pediatric Critical Care, Vol 8, Iss 3, Pp 145-148 (2021)

Atypical hemolytic-uremic syndrome (aHUS) is a form of thrombotic microangiopathy that occurs due to dysregulation of alternate pathway of complement system, which progressively causes systemic complications, end-stage renal disease, and death. As prognosis is poor compared to typical hemolytic-uremic syndrome, early diagnosis and treatment is crucial for favorable outcome. We came across seven patients of aHUS in our pediatric intensive care unit in the last 5 years. Plasma exchange (PE) along with immunosupressives was used for treatment. First child who did not receive PE died. Rest six patients underwent PE and attained hematological remission; however, one later on progressed to chronic kidney disease and expired. All others are on regular follow-up and doing well. A high index of suspicion is required to diagnose aHUS. Early PE can give a better prognosis.