Challenges in management of atypical hemolytic uremic syndrome: Bottle neck in resource limited settings

Treatment of atypical hemolytic uremic syndrome cases is challenging right from establishing correct and timely diagnosis to execution of management protocol. A seven-year-old male child from poor socioeconomic status was admitted with chief complaints of fever, 3 episodes of vomiting and passage of cola coloured urine. Based on clinical and laboratory findings, diagnosis was established. However, ADAMTS13 levels and genetic studies required for diagnosis could not be performed due to financial constraints and non-availability of these tests. TPE kits were arranged from charitable organizations. Six TPE procedures were performed using Cobe Spectra cell separator. Central venous catheter was placed in femoral vein. TPE kit was primed with compatible packed red blood cells before each procedure. Patient was non-cooperative and irritable in first three procedures and was sedated. A total of 1300 ml plasma was exchanged in each procedure with group specific fresh frozen plasma. After second TPE procedure, patient started improving with decrease in plasma discoloration and periorbital edema. Renal function tests along with hematological parameters became normal after 6th TPE procedure. Patient was discharged in a stable condition. On follow up, C3 levels were normal with adequate platelet count and normal renal functions suggesting complete remission.