臨床症例報告ジャーナル

An Unusual Case of Paediatric Atypical Haemolytic Uraemic Syndrome with Bilateral Purtschers-Like Retinopathy and Sub-Retinal Detachment

Abstract

Parekh R

Atypical haemolytic uremic syndrome (aHUS) is a rare disease caused by chronic, uncontrolled activation of the alternative complement pathway that leads to thrombotic microangiopathy, haemolytic anaemia, acute renal impairment and thrombocytopenia. If left untreated, aHUS can progress into end stage renal disease and permanent renal impairment. Extrarenal manifestations have also been reported in the literature. Ocular involvement is usually rare in aHUS. This is a rare case report where a 13-year-old boy with aHUS presented with bilateral purtschers like retinopathy and sub retinal detachment in addition to renal impairment. The patient’s hematologic and renal parameters and ocular manifestation improved following appropriate therapy.