Artigo Revisado por pares

Hypergonadotropic-hypogonadism in the Prader-Labhart-Willi syndrome

1979; Elsevier BV; Volume: 94; Issue: 3 Linguagem: Inglês

10.1016/s0022-3476(79)80596-x

ISSN

1097-6833

Autores

L. EVERETT SEYLER, Karunyan Arulanantham, Christina O'Connor,

Tópico(s)

Genetic Syndromes and Imprinting

Resumo

Hypogonadism is an almost invariable feature o f the Prader-Labhart-Will i syndrome 1 and is more severe and persistent in males than females. ~, ~ Although the exact cause for the hypogonadism in this syndrome is not known, previous studies have presented evidence for a hypothalamic deficiency.'-'. ~. ~ We report here studies of a 16,year-old boy with PLW syndrome whose responses to LRH and hCG show that the hypogonadism in this syndrome can be due to gonadal deficiency rather than to an incapacity to initiate or sustain pubertal hypothalamic maturation.

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