Hypothalamic Hamartoma
作者： David M. Judge , Howard E. Kulin , Robert Page , Richard Santen , Suriyonta Trapukdi
DOI: 10.1056/NEJM197701062960102
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摘要: The presence of a hypothalamic hamartoma and precocious puberty in 19-month-old boy provided an opportunity to study their relation. Excised tissue had the ultrastructural characteristics independent neuroendocrine unit -- i.e., neurons containing neurosecretory granules blood vessels with fenestrated endothelium double basement membranes. Immunofluorescence studies using specific antibody luteinizing-hormone-releasing factor showed antigenicity hamartoma. testicular-hypothalamic-pituitary axis was tested. Clomiphene unresponsiveness suggested lack maturation central-nervous-system events characteristic normal puberty. negative feedback system between gonad brain intact but partially resistant steroid suppression. These suggest that hamartomas may cause by autonomous production release into communicate pituitary portal system.
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