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An unusual form of renal disease associated with gout and hypertension
  1. W. Van Goor1,
  2. C. J. Kooiker2,
  3. E. J. Dorhout Mees
  1. University Hospital, Utrecht, The Netherlands
  2. Pathological Institute, University of Utrecht, Utrecht, The Netherlands

    Abstract

    A family is described many of whose members suffered from renal insufficiency, hypertension, gout, and hyperuricaemia in conjunction. Adequate information was obtained on 72 subjects from five generations. In 17, one or more of the above mentioned abnormalities was or had been present. The hereditary distribution suggested an autosomal dominant disease entity.

    The renal disease was characterized by an early loss of urinary concentrating power, minimal proteinuria, and death at a relatively early age dominating the clinical picture.

    The histological picture in three biopsies and one necropsy showed predominant tubular atrophy and interstitial fibrosis, with striking tubular basement membrane thickening.

    It is suggested that these patients suffered from a hereditary degenerative renal disease. The question whether hyperuricaemia was primary or secondary in these cases is discussed.

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    Footnotes

    • 1 Present address: Deaconess Hospital, Haarlem, The Netherlands.

    • 2 Pathological Institute, Pasteurstraat, Utrecht, The Netherlands.

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