| CASE REPORT |
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| Year : 2021 | Volume
: 8
| Issue : 3 | Page : 145-148 |
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Atypical hemolytic-uremic syndrome - A case series from a tertiary care hospital from Eastern India
Mukesh Kumar Jain1, Nikunj Kishore Rout2, Amit Ranjan Rup1, Sibabratta Patnaik1, Chinmay Kumar Behera1, Reshmi Mishra1, Bandya Sahoo1
1 Department of Pediatrics, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Nephrology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India
Correspondence Address:
Dr. Sibabratta Patnaik
Department of Pediatrics, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jpcc.jpcc_184_20
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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.
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