Rituximab dependent Nephrotic Syndrome (A case report )

Background and aim: Plasma infusion or plasma exchange is the rescue therapy for familial or recurrent hemolytic uremic syndrome. The recommended volume of plasma infusion is 20-40ml/kg. There is no document to show how much of plasma volume actually get the patient to remission. Material and methods: This was an observational retrospective study between March 2007 and 2015. All patients with diagnosis of recurrent or familial atypical hemolytic uremic syndrome (aHUS) admitted in Ali-Asgar Children Hospital included. The total volume of plasma required to normalize platelet, LDH, eliminate hemolysis, and decrease serum creatinin was calculate. Patients with TTP, vasculitis, and postinfectious were excluded. Results: Between 2007 and 2015, twenty-four patients (M-13, F-11) with diagnosis of aHUS were included. Two patents were excluded, one occurred after sepsis and the other did not receive plasma infusion. The median age (range) was 53 months (3-144 m). 20 patients achieved remission by plasma infusion (under PD-5, under HD-4) and two patients treated by plasmapheresis. The median (range) total plasma volume required for remission was 99ml/kg (43-235 ml/kg). Conclusion: This study showed recurrent or familial HUS in acute phase require at least twice total plasma volume than those volume recommended in literature in order to control the relapse phase of disease.