[Marfan syndrome and cystic kidneys of the adult type]

Helv Chir Acta. 1992 Oct;59(3):513-5.
[Article in German]

Abstract

For the first time Bernhard Marfan described the Marfan-Syndrome in 1896; it is a meso- and ectodermed variety with the conducting symptom of "arachnodactyly". Marfan-Syndrome is an autosomal dominant hereditary disorder with high penetrance and variable expressivity. In our opinion this case of a 41-year-old patient with kidney cysts and aneurysma dissecans of the arteria ascendens by Marfan-Syndrome was described in the literature only twice. The casuistics of this Marfan-Syndrome patient shows a particular rare associated organ-change--the kidney cysts--and illustrate the frequency of this hereditary disease.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Aneurysm, Dissecting / genetics
  • Aneurysm, Dissecting / pathology
  • Aneurysm, Dissecting / surgery
  • Aortic Aneurysm / genetics
  • Aortic Aneurysm / pathology
  • Aortic Aneurysm / surgery
  • Blood Vessel Prosthesis
  • Humans
  • Kidney / pathology
  • Male
  • Marfan Syndrome / genetics*
  • Marfan Syndrome / pathology
  • Marfan Syndrome / surgery
  • Nephrectomy
  • Polycystic Kidney Diseases / genetics*
  • Polycystic Kidney Diseases / pathology
  • Polycystic Kidney Diseases / surgery
  • Reoperation