[Nephrotic syndrome complicated by chylous ascites in a girl of 2 years and 8 months old].

Autor: Mabiala Babela JR; Service de pédiatrie nourrissons, CHU de Brazzaville, Brazzaville, Congo; Faculté des sciences de la santé, université Marien Ngouabi, Brazzaville, Congo. Electronic address: jmabialababela@yahoo.fr., Ollandzobo Ikobo LC; Service de pédiatrie nourrissons, CHU de Brazzaville, Brazzaville, Congo., Loumingou R; Faculté des sciences de la santé, université Marien Ngouabi, Brazzaville, Congo; Service de néphrologie, CHU de Brazzaville, Brazzaville, Congo., Nika ER; Service de pédiatrie nourrissons, CHU de Brazzaville, Brazzaville, Congo., Mouko A; Service de pédiatrie nourrissons, CHU de Brazzaville, Brazzaville, Congo; Faculté des sciences de la santé, université Marien Ngouabi, Brazzaville, Congo.
Jazyk: francouzština
Zdroj: Archives de pediatrie : organe officiel de la Societe francaise de pediatrie [Arch Pediatr] 2017 Jan; Vol. 24 (1), pp. 24-27. Date of Electronic Publication: 2016 Nov 21.
DOI: 10.1016/j.arcped.2016.10.010
Abstrakt: We report on a case of nephrotic syndrome with focal and segmental hyalinosis complicated by chylous ascites in a girl of 2 years and 8 months old. This pure nephrotic syndrome in its early stage was initially treated with intensive steroid treatment at 2mg/kg/day orally for 2 months, 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 1 L of aseptic and milky fluid, containing 0.22g/L of proteins, 20 IU/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 1 month, then once every 2 days for 3 months, and repeated ascites punctures. After 12 ascites punctures performed every 15 days, a polyuric episode occurred and the ascites disappeared. Proteinuria persisted at a nephrotic rate up to the 14th month, without impairment in kidney filtration function, and completely disappeared at the 20th month. After 5 years 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.
(Copyright © 2016 Elsevier Masson SAS. All rights reserved.)
Databáze: MEDLINE
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