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Titolo:
SIGNS OF TESTICULAR INSUFFICIENCY IN ADRENOMYELONEUROPATHY AND NEUROLOGICALLY ASYMPTOMATIC X-LINKED ADRENOLEUKODYSTROPHY - A RETROSPECTIVE STUDY
Autore:
ASSIES J; GOOREN LJG; VANGEEL B; BARTH PG;
Indirizzi:
UNIV AMSTERDAM,DEPT PSYCHIAT & INTERNAL MED,ACAD MED CTR,POB 22700 NL-1100 DE AMSTERDAM NETHERLANDS UNIV AMSTERDAM,DEPT NEUROL,ACAD MED CTR NL-1100 DE AMSTERDAM NETHERLANDS UNIV AMSTERDAM,DEPT PEDIAT,ACAD MED CTR NL-1100 DE AMSTERDAM NETHERLANDS FREE UNIV AMSTERDAM HOSP,DEPT INTERNAL MED,DIV ANDROL AMSTERDAM NETHERLANDS
Titolo Testata:
International journal of andrology
fascicolo: 5, volume: 20, anno: 1997,
pagine: 315 - 321
SICI:
0105-6263(1997)20:5<315:SOTIIA>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
REPLACEMENT THERAPY; FATTY-ACIDS; LONG-CHAIN; CELLS; VARIANT; DISEASE;
Keywords:
HYPOGONADISM; HYPOTHALAMO-PITUITARY-TESTIS AXIS; TESTIS FUNCTION; X-LINKED ADRENOLEUKODYSTROPHY;
Tipo documento:
Article
Natura:
Periodico
Citazioni:
35
Recensione:
Indirizzi per estratti:
Citazione:
J. Assies et al., "SIGNS OF TESTICULAR INSUFFICIENCY IN ADRENOMYELONEUROPATHY AND NEUROLOGICALLY ASYMPTOMATIC X-LINKED ADRENOLEUKODYSTROPHY - A RETROSPECTIVE STUDY", International journal of andrology, 20(5), 1997, pp. 315-321

Abstract

X-linked adrenoleukodystrophy (X-ALD) is characterized by central nervous system demyelination, and impaired steroidogenesis in the adrenalcortex and testis. Most patients develop adrenocortical insufficiency. We studied retrospectively the frequency and severity of testicular dysfunction in 26 men with X-ALD. Twenty-one had adrenomyeloneuropathyand five patients were neurologically asymptomatic. In addition to obtaining a routine history and physical examination, we studied plasma levels of testosterone, sex hormone binding globulin, the free androgen index, and the plasma concentrations of dehydroepiandrosterone-sulphate, LH and FH. In a subset of patients, the testosterone response to hCG and the LH and FSH responses to GnRH were also determined. Clinical signs of gonadal dysfunction were manifested by diminished libido (46%), largely overlapping with erectile dysfunction (58%), and failure of the testes to descend (15%). Physical examination revealed diminished body sexual hair (50%), gynaecomastia (35%), and small testes (12%). Laboratory studies showed low plasma total testosterone levels in 12%, and an insufficient increase after stimulation with hCG in 88% (15 of 17 patients tested). Plasma LH concentration was increased in 16%, and the plasma FSH level was elevated in 32%. The response of LH concentrations to GnRH stimulation was abnormally high in 47% (nine of 19 patients studied), and the response of FSH levels was too low in 16% (three of 19 patients tested). In conclusion, in a retrospective study of 26 men' with X-ALD, in 20 some signs of clinical hypogonadism were found. Plasma testosterone values were generally in the normal range, but upon testing of the hypothalamo-pituitary-testis axis some abnormalities became apparent.

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Documento generato il 03/12/20 alle ore 16:10:07