Clinical Investigation

Kidney International (1984) 25, 701–707; doi:10.1038/ki.1984.77

Pathology of fetal congenital nephrosis: Immunohistochemical and ultrastructural studies

Juhani Rapola1, Hannu Sariola1 and Peter Ekblom1

1Children's Hospital and Department of Pathology, University of Helsinki, Helsinki, Finland

Correspondence: Dr J Rapola, Children's Hospital, University of Helsinki, Stenbäckinkatu 11, SF-00290 Helsinki 29, Finland

Received 3 June 1983; Revised 21 September 1983.

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Abstract

Pathology of fetal congenital nephrosis: Immunohistochemical and ultrastructural studies. The kidneys of four human fetuses aged 18 to 20 weeks of gestation with congenital nephrosis of the Finnish type (CNF) were studied by immunohistochemistry and electron microscopy to elucidate the pathogenesis of the disease. The immunohistochemical stainings for laminin, type IV collagen, fibronectin, brushborder antigens (BBA), Tamm-Horsfall protein (TH), and binding of wheat germ agglutinin (WGA) did not reveal changes as compared to the age-matched control kidneys. Proximal tubules of the CNF kidneys showed excessive accumulation of coarse granular alpha fetoprotein (AFP) and large absorption droplets with paracrystalline and membraneous structures were seen in electron microscopy. The dilated tubules were both of proximal and distal origin as judged from the BBA and TH stainings. It is suggested that the formation of the dilated tubules results from degeneration caused by obstruction and excessive protein loading of the tubular epithelium. Demonstration of the coarse granular AFP in the proximal tubular epithelium and lumina indicates heavy fetal proteinuria and serves as an additional diagnostic marker for prenatal CNF.

Pathologie de la néphrose foetale congénitale: Études immunohistochimiques et ultrastructurales. Les reins de quatre foetus humains âgés de 18 à 20 semaines de gestation atteints de néphrose congénitale du type Finlandais (CNF) ont été étudiés en immunohistochimie et microscopie électronique pour élucider la physiopathologie de la maladie. Les colorations immunohistochimiques pour la laminine, le collagène de type IV, la fibronectine, les antigènes de la bordure en brosse (BBA), la protéine de Tamm-Horsfall (TH), et la liaison de l'agglutinine de germe de blé (WGA) n'ont pas révélé de modification par rapport à des reins contrôles d'âge identique. Les tubules proximaux des reins de CNF présentaient une accumulation excessive de larges grains d'alpha foetoprotéine (AFP) et de larges gouttelettes d'absorption avec des structures paracristallines et membraneuses ont été vues en microscopie électronique. Les tubules dilatés étaient d'origine proximale et distale, comme le montrait les colorations des BBA et de TH. Il est suggéré que la formation de tubules dilatés résulte de la dégénérescence dûe à l'obstruction et à une surcharge protéinique excessive de l'épithélium tubulaire. La démonstration de larges grains d'AFP dans l'épithélium et la lumière tubulaire proximale indique une protéinurie foetale importante, et sert de marqueur diagnostique supplémentaire pour la CNF prénatale.

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