LAUSR

Objective: Hemolytic uremic syndrome (HUS) is a rare and severe form of thrombotic microangiopathy (TMA) associated with a poor renal prognosis. Atypical hemolytic uraemic syndrome (aHUS) is a form of HUS that has been clearly associated with genetic susceptibility factors related to complement regulation. Pregnancy- and postpartum-associated HUS is now considered as a part of spectrum of complement-mediated HUS. AHUS is a rare cause of malignant hypertension. For this reason we report 02 cases of postpartum aHUS observed in nephrology department. Design and method: We analyzed retrospectively the presentation and outcome of 02 sisters who had a malignant hypertension in the period of post-partum with features of hemolytic and uremic syndrome. Results: In the two cases, it was a clinical presentation of malignant hypertension needing intravenous treatment with nicardipine associated with severe acute kidney injury (AKI) requiring dialysis at presentation (table). We underwent plasma exchanges as first-line therapy then relayed by plasma infusions. Kidney biopsy performed in the first sister whose diagnosed in 2018 (with normal platelet count) showed typical features of thrombotic microangiopathy.Complement component assays showed low Factor H (<10%) in 02 cases. They evolved to chronic kidney disease and they have always a severe blood pressure although the combination of 5 antihypertensive medications. Conclusions: Post partum -related atypical HUS is a very severe disease, with a poor kidney prognosis and without specific efficacious treatment. Familial aHUS is very rare entity. We reported 02 cases of malignant hypertension which is highly prevalent in this population and it dominates the clinical presentation.