Ciężkie powikłania i następstwa pierwotnego zespołu nerczycowego u dzieci

Abstract

IntroductionSince the introduction of corticosteroids, the overall outcome in primary NS in children is good. However, despite the improvement in survival, children with NS experience some degree of morbidity and mortality that result from acute complications of the disease, long-lasting proteinuria and immunosuppressive treatment. Aim of the studyAnalysis of life-threatening complications and late consequences of NS in children. Patients and methodsClinical course, pathomorphology, acute and late complications of NS in 152 patients (96 boys, 56 girls at a mean age of 4.10+3.76yr) admitted between 1.01.2000 and 30.03.2011 were investigated. Results12 episodes of AKI in 9 patients (6.0%; 4 with FSGS, 4 with MCD, 1 with DMP), 2 episodes (1.3%) of TED and 4 (2.7%) sepsis were observed. CKD with reduced glomerular filtration appeared in 11 patients (7.3%; 6 with FSGS, 4 with CNS, 1 with M-PGN), 9 of them (6.0%) were dialyzed and 7 (4.7%) received kidney transplant. Two of four transplanted patients with FSGS had recurrence of NS. Neoplastic disease was diagnosed in 2 patients (1.3%) with DMP: Hodgkin lymphoma in 17-year-old girl with SRNS and rhabdomyosarcoma of the testis in 17-year-old boy with SDNS after 15 years of treatment with corticosteroids, levamisol and cyclosporin A. Within the group, 4 patients (2.7%) died: 2 with CNS of sepsis, 1 of TED and 1 of intracranial hemorrhage during hemodialysis. ConclusionsAlthough the prognosis in NS in children is good, in a small proportion of patients there is a possibility of life-threatening or even fatal complications of the disease. They occur irrespectively of the type of glomerular changes. Treatment of children with NS should be supervised by pediatric nephrological units with easy access to renal replacement therapy.