Syndrome néphrotique compliqué d’ascite chyleuse chez une fillette de 2 ans et 8 mois

Abstract

We report on a case of nephrotic syndrome with focal and segmental hyalinosis complicated by chylous ascites in a girl of 2years and 8months old. This pure nephrotic syndrome in its early stage was initially treated with intensive steroid treatment at 2mg/kg/day orally for 2months, followed by a bolus of methylprednisolone. The persistence of proteinuria meant corticosteroid resistance. Renal biopsy then revealed focal and segmental hyalinosis. A recurrence of the edema-ascites syndrome was associated with macroscopic hematuria. Blood pressure and serum creatinine remained normal. Protidemia decreased to 28g/L, with severe hypoalbuminemia at 7g/L. The ascites puncture brought 1L of aseptic and milky fluid, containing 0.22g/L of proteins, 20IU/L of amylase, and 331g/L of total lipids. The treatment protocol included a hyperprotein diet, prednisone at 0.5mg/kg/day, cyclophosphamide at 2.5mg/kg/day for 1month, then once every 2days for 3months, and repeated ascites punctures. After 12ascites punctures performed every 15days, a polyuric episode occurred and the ascites disappeared. Proteinuria persisted at a nephrotic rate up to the 14thmonth, without impairment in kidney filtration function, and completely disappeared at the 20thmonth. After 5years of follow-up, proteinuria remains undetectable and the physical exam is normal. The occurrence of chylous ascites during the nephrotic syndrome is a rare event. The formation of bowel lymphangiectasia, caused by a slowdown in venous return due to the pressure exerted by ascites, is probably the predominant mechanism.