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[伴有弥漫性系膜增生的特发性肾病综合征]

[Idiopathic nephrotic syndrome with diffuse mesangial proliferation].

作者信息

Mota F, Jiménez R, Gordillo G

出版信息

Bol Med Hosp Infant Mex. 1979 Mar-Apr;36(2):299-305.

PMID:758199
Abstract

The clinicopathologic correlation of 18 cases of idiopathic nephrotic syndrome (INS) with diffuse mesangial proliferation (MP), (over 3 cells per intercapillary space) showed clinical characteristics similar to INS with minimal glomerular lesions (MGL) in relation to age at onset, sex, period of evolution, intensity of proteinuria, hypercholesterolemia, hypoalbuminemia and edema. However, there was a greater incidence of cases with arterial hypertension, hematuria, azotemic retention and positive glomerular immunofluorescence. Out of the 18 cases, 10 were corticosensitive (group I) and 8 were corticoresistant (group II). Patients of I followed a similar course as those with MGL, while most cases of group II showed proteinuria through observation periods up to 5 years. No differences were found in the initial clinical presentation between these 2 groups. The only item with prognostic value was the intensity of the mesangial proliferation which in group I was of 3 to 5 cells per intercapillary space, while in group II, in the spaces of some glomeruli, there were up to 10 mesangial cells present. These findings suggest the convenience to practice renal biopsy before initiating treatment in children with INS and arterial hypertension, hematuria and/or azotemic retention in order to identify this group of patients that appears to be different from that with MGL.

摘要

18例特发性肾病综合征(INS)伴弥漫性系膜增生(MP,每个毛细血管襻内超过3个细胞)的临床病理相关性研究显示,在发病年龄、性别、病程、蛋白尿程度、高胆固醇血症、低白蛋白血症和水肿方面,其临床特征与微小病变性肾小球病(MGL)相似。然而,动脉高血压、血尿、氮质血症潴留和肾小球免疫荧光阳性的病例发生率更高。18例中,10例对皮质激素敏感(I组),8例对皮质激素抵抗(II组)。I组患者的病程与MGL患者相似,而II组的大多数病例在长达5年的观察期内均有蛋白尿。这两组的初始临床表现无差异。唯一具有预后价值的指标是系膜增生的程度,I组为每个毛细血管襻3至5个细胞,而II组在一些肾小球的襻内,系膜细胞多达10个。这些发现提示,对于患有INS且伴有动脉高血压、血尿和/或氮质血症潴留的儿童,在开始治疗前进行肾活检是合适的,以便识别出这组似乎与MGL不同的患者。

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