GONADAL FUNCTION IN BLOOMS SYNDROME

GONADAL FUNCTION IN BLOOMS SYNDROME
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DOI:
10.1111/j.1365-2265.1977.tb02013.x
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发表时间:
1977-01-01
影响因子:
3.2
通讯作者:
LARON, Z
LARON, Z
中科院分区:
医学3区
文献类型:
--
作者:
KAULI, R;PRAGERLEWIN, R;LARON, Z

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Five patients with Bloom''s syndrome aged from 2 8/12-27 yr, all of whom had hypogonadism, were subjected to an i.v. LHRH [luteinizing hormone-releasing hormone] test, and 2 of them to an i.m. HCG [human chorionic gonadotropin] test. There was increased responsiveness of plasma LH [luteinizing hormone] FSH [follicle stimulating hormone] indicating that the hypogonadism is primary in nature and of early development. The tubular element of the testis seems to be mainly affected, as indicated by the particularly high FSH response to LHRH stimulation, a history of sterility in the 2 adult patients and documented azoospermia in 1 of them. The Leydig cells seem to be less affected and secrete sufficient androgens to enable puberty within acceptable normal limits. Hypogonadism seems to be a major characteristic of Bloom''s syndrome.