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目的报道一例新生儿弥漫性系膜硬化型先天性肾病综合征,以提高对该病的认识。方法收集一例于2010年10月在中山大学附属第一医院儿科住院确诊为先天性肾病综合征弥漫性系膜硬化型的患儿临床资料,并复习文献,总结弥漫性系膜硬化型先天性肾病综合征的病因、临床表现、病理特点及预后。结果患儿生后即有水肿、大量蛋白尿等肾病综合征表现,并很快出现肾功能不全,进展至肾功能衰竭,肾活检病理符合弥漫性系膜硬化型,2个多月后死亡。结论先天性肾病综合征弥漫性系膜硬化型的临床表现为出生时或幼儿期内出现肾病综合征的特征,起病时可已有肾功能不全,进行性肾功能减退,确诊须肾穿病理,本病预后差,目前无特殊治疗。
Objective To report a case of diffuse nephrotic syndrome of neonate with diffuse mesentery sclerosis in order to improve the understanding of the disease. Methods A case of children with diffuse mesentery sclerosis diagnosed as congenital nephrotic syndrome in pediatric department of First Affiliated Hospital of Sun Yat-sen University in October 2010 was collected, and the literature was reviewed, and diffuse mesentery sclerosis congenital nephropathy Syndrome etiology, clinical manifestations, pathological features and prognosis. Results Children with edema after birth, a large number of proteinuria nephrotic syndrome, and soon renal insufficiency, progression to renal failure, renal biopsy consistent with diffuse mesentery sclerosis, more than 2 months after the death. Conclusions The clinical manifestations of diffuse mesentery sclerosis in congenital nephrotic syndrome are the features of nephrotic syndrome at birth or in early childhood. It may present with renal insufficiency and progressive renal dysfunction when diagnosed. Nephrotic syndrome The poor prognosis of the disease, there is no special treatment.