- 期刊
神經腦垂體異位症併泛腦垂體機能低下－一病例報告
Ectopic Neurohypophysis Associated with Panhypopituitarism
摘要
本文報告一位20歲泛腦垂體機能低下(congenital panhypopituitarism)的男性患者，臨床上有明顯性腺機能低下(hypogonadism)的表徵。實驗室檢查確認有腎上腺皮質機能低下，甲狀腺機能低下，性腺機能低下及輕微的高泌乳激素血症。而施行L-dopa，Clonidine或胰島素低血糖刺激試驗後，生長激素的反應都低於正常值。在腦部磁振顯影發現：腦垂體前葉發育不全併神經腦垂體異位症(ectopic neurohypophysis)及垂體柄消失。回顧文獻的記載，若影像學檢查看不到垂體柄(absence of pituitary stalk)，表示其腦垂體前葉發育不全是較嚴重型，往往會同時缺乏多種前葉激素(multiple anterior pituitary hormone deficiency)。
並列摘要
We report a 20-year-old man with panhypopituitarism and overt hypogonadism. The laboratory findings confirmed the diagnosis of hypocortisolism, hypothyroidism, hypogonadotropism and mild hyperprolactinemia. Provocative tests revealed subnormal response of growth hormone to L-dopa, Clonidine and Insulin hypoglycemia stimulation. The magnetic resonance imaging study of brain showed: hypoplastic pituitary with ectopic neurohypophysis and absence of pituitary stalk. We reviewed the literatures and proposed that the absence of pituitary stalk in imaging study are afflicted with a more severe form of adenohypophysis hypoplasia and have multiple anterior pituitary hormone deficiency.
延伸閱讀
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