Adrenal and testicular function in 14 patients with adrenoleukodystrophy or adrenomyeloneuropathy

Samuel M. Libber, Claude J. Migeon, Frank R. Brown, Hugo W. Moser

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1-8
Number of pages8
JournalHormone Research in Paediatrics
Volume24
Issue number1
DOIs
StatePublished - Jan 1 1986

Keywords

  • Adrenal failure
  • Adrenoleukodystrophy
  • Adrenomyeloneuropathy
  • Primary hypogonadism
  • Testicular insufficiency

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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