Huttunen N P, Rapola J, Vilska J, Hallman N
Int J Pediatr Nephrol. 1980 Mar;1(1):10-6.
The quantitative histopathological analysis was performed on 68 renal specimens of 50 children with congenital nephrotic syndrome of Finnish type (CNF). Thirty five of samples were biopsies and 33 were taken at autopsy. The kidneys of the CNF children were 2-3 times larger by weight than those of normal children. The renal histological picture varied greatly from one patient to another. The glomeruli of the CNF patients showed more often fibrotic lesions and mesangial hypercellularity than those of the normal infants, while immature glomeruli were seen more frequently in the controls than in CNF children. Tubular atrophy and tubular dilatations were also common histological feature in the CNF children. Glomerular fibrosis and tubulo-interstitial changes showed significant progression during the course of the disease while mesangial cell proliferation did not increase with age. The decreased tubular cell mass was replaced by interstitial tissue in the kidneys of older CNF infants. The proportion of the total glomerular volume to the volume of the whole kidney was about the same in CNF as in normal children. Because of the greater kidney size in CNF the absolute volume of the total glomerular mass is increased in this disease.
对50例芬兰型先天性肾病综合征（CNF）患儿的68份肾脏标本进行了定量组织病理学分析。其中35份样本为活检标本，33份为尸检标本。CNF患儿的肾脏重量比正常儿童的肾脏重2至3倍。不同患者的肾脏组织学表现差异很大。与正常婴儿相比，CNF患者的肾小球更常出现纤维化病变和系膜细胞增多，而在对照组中比在CNF患儿中更频繁地见到未成熟肾小球。肾小管萎缩和肾小管扩张也是CNF患儿常见的组织学特征。在疾病过程中，肾小球纤维化和肾小管间质改变有显著进展，而系膜细胞增殖并不随年龄增加。在年龄较大的CNF婴儿的肾脏中，减少的肾小管细胞团被间质组织所取代。CNF患者肾小球总体积占整个肾脏体积的比例与正常儿童大致相同。由于CNF患者的肾脏更大，该病中肾小球总体积的绝对量增加。
