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      Hereditary Nephropathy with Nerve Deafness (Alport’s Syndrome)

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          Abstract

          Alport’s syndrome is a glomerulopathy of unknown pathogenesis that should be considered in any patient with unexplained hematuria. Four new kindreds of carefully studied family backgrounds were investigated; renal functional tests, otorhinolaryngological and ophthalmological tests, chromosomal maps, light and immunofluorescent microscopic studies were carried out. Renal biopsies of three male patients were also examined by electron microscopy. Characteristic ultrastructural lesions were found: the basement membrane showed segmentary thickenings with characteristic focal and local splitting of the lamina densa, giving rise to electron-lucent spaces.

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          Author and article information

          Journal
          NEF
          Nephron
          10.1159/issn.1660-8151
          Nephron
          S. Karger AG
          1660-8151
          2235-3186
          1974
          1974
          28 November 2008
          : 13
          : 5
          : 404-415
          Affiliations
          Sezione di Nefrologia, Ospedale Civile di Vimercate, Centro di Microscopia Elettronica, Istituto di Farmacologia dell’Università di Milano e Divisione di Nefrologia, Ospedale San Carlo Borromeo, Milano
          Article
          180417 Nephron 1974;13:404–415
          10.1159/000180417
          4431551
          © 1974 S. Karger AG, Basel

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          Pages: 12
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