HYPOTHALAMIC HAMARTOMA - SOURCE OF LUTEINIZING-HORMONE-RELEASING FACTOR IN PRECOCIOUS PUBERTY
HYPOTHALAMIC HAMARTOMA - SOURCE OF LUTEINIZING-HORMONE-RELEASING FACTOR IN PRECOCIOUS PUBERTY
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DOI：
10.1056/nejm197701062960102
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发表时间：
1977-01-01
影响因子：
158.5
通讯作者：
TRAPUKDI, S
中科院分区：
文献类型：
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作者：
JUDGE, DM;KULIN, HE;TRAPUKDI, S
一个19个月大的婴儿出现下丘脑错构瘤和性早熟。老男孩提供了一个研究他们关系的机会。切除的组织具有独立的神经内分泌系统的超微结构特征，即，神经元含有神经分泌颗粒，血管内皮有孔，有双层基膜。免疫荧光研究表明，促黄体生成激素释放因子[LRF]的特异性抗体在错构瘤的抗原性。检测睾丸-下丘脑-垂体轴。克罗米芬无反应提示正常青春期特征性CNS事件缺乏成熟。性腺和大脑之间的负反馈系统是完整的，但部分抵抗类固醇抑制。下丘脑错构瘤可能通过自主产生LRF并将其释放到与垂体门静脉血液系统相通的血管中而引起性早熟。
The presence of a hypothalamic hamartoma and precocious puberty in a 19 mo. old boy provided an opportunity to study their relation. Excised tissue had the ultrastructural characteristics of an independent neuroendocrine.sbd.i.e., neurons containing neurosecretory granules and blood vessels with fenestrated endothelium and double basement membranes. Immunofluorescence studies using specific antibody to luteinizing-hormone-releasing factor [LRF] showed antigenicity to the factor in the hamartoma. The testicular-hypothalamic-pituitary-axis was tested. Clomiphene unresponsiveness suggested a lack of maturation of CNS events characteristic of normal puberty. The negative feedback system between gonad and brain was intact but partially resistant to steroid suppression. Hypothalamic hamartomas may cause precocious puberty by autonomous production and release of LRF into vessels that communicate with the pituitary portal blood system.