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      Hormonal profile and fertility in patients with Anderson-Fabry disease.

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          Abstract

          Anderson-Fabry disease is a glycosphingolipid storage disorder with an X-linked recessive inheritance. The alpha-galactosidase A deficiency leads to a progressive accumulation of globotriaosylceramide in the endothelium and tissue cells of various organs. The kidney, heart and brain are predominantly affected. Reports on endocrine function and fertility rates in patients with Anderson-Fabry disease are sparse. In the present study, we assessed ovarian, testicular and adrenal function in a cohort of patients with Anderson-Fabry disease. Plasma follicle-stimulating hormone, luteinizing hormone, prolactin, estradiol, testosterone, sex hormone-binding globulin, somatotropin, insulin-like growth factor-I and serum cortisol were measured in 13 patients (six female and seven male), currently observed in an outpatient clinic. The profile revealed an undisturbed hormonal function and a normal fertility rate in both male and female Anderson-Fabry patients when compared with the corresponding Austrian population.

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          Author and article information

          Journal
          Int. J. Clin. Pract.
          International journal of clinical practice
          Wiley-Blackwell
          1368-5031
          1368-5031
          Sep 2005
          : 59
          : 9
          Affiliations
          [1 ] Division of Nephrology and Dialysis,Medical University of Vienna, Vienna, Austria. anna-christine.hauser@meduniwien.ac.at
          Article
          IJCP620
          10.1111/j.1742-1241.2005.00620.x
          16115176
          16907337-0184-411f-9fd7-e02dec1a19ef
          History

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