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      Adrenal and Testicular Function in 14 Patients with Adrenoleukodystrophy or Adrenomyeloneuropathy

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          Testicular and adrenal function were evaluated in 12 patients with adrenoleukodystrophy and 2 patients with adrenomyeloneuropathy. Although only 5 subjects had clinical symptoms suggesting adrenal insufficiency, an additional 5 showed laboratory evidence of reduced adrenal reserve. 9 of the 14 patients developed neurological deficits prior to the onset of clinical or biological adrenal insufficiency. In the remaining 5 patients, adrenal insufficiency antedated the appearance of neurological symptoms; 2 of these 5 patients had only laboratory evidence of hypoadrenocorticism, and 3 had both clinical and laboratory abnormalities. None of the prepubertal patients had detectable signs of testicular insufficiency, while 3 of the 7 pubertal/adult patients had elevated serum LH or FSH levels. This mild testicular deficiency was seen only in association with clinical adrenal insufficiency and significant neurological impairment.

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          Author and article information

          Hormone Research in Paediatrics
          S. Karger AG
          28 November 2008
          : 24
          : 1
          : 1-8
          aDivision of Pediatric Endocrinology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Md., USA; bJohn F. Kennedy Institute, Johns Hopkins Medical Institutions, Baltimore, Md., USA; cDivision of Developmental Disabilities, Department of Pediatrics, Medical University of South Carolina, Charleston, S.C., USA
          180533 Horm Res 1986;24:1–8
          © 1986 S. Karger AG, Basel

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          Pages: 8


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