[Marfan syndrome and cystic kidneys of the adult type].
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.
Biermann
C W
CW
Urologische Klinik und Poliklinik, Kantonsspital Basel.
Gasser
T C
TC
Breuer
C
C
Rutishauser
G
G
D000328:Adult; D000784:Aortic Dissection / Q000235:genetics / Q000473:pathology / Q000601:surgery; D001014:Aortic Aneurysm / Q000235:genetics / Q000473:pathology / Q000601:surgery; D001807:Blood Vessel Prosthesis; D006801:Humans; D007668:Kidney / Q000473:pathology; D008297:Male; D008382:Marfan Syndrome / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D009392:Nephrectomy; D007690:Polycystic Kidney Diseases / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D012086:Reoperation
Marfan-Syndrome und Zystennieren vom Erwachsenen-Typ.