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I.はじめに
1951年Russellが初のdiencephalic syndromcを報告したが,その後約85例の報告がある.diencephalic syndromeとは,るいそうと血中成長ホルモン(以下HGHと略)の異常高埴を示し,主に視床下部を占める脳腫瘍によりひきおこされ,通常乳児期,幼児期早期に発症するものである。われわれは2例のdiencephalic syndmmeを経験し,症例1は術後経過観察中に思春期早発症を合併した視床下部のpilocytic astrocytomaであった.症例2は松果体部ならびに視交叉部のgerminomaであった.内分泌学的検索を加え,この2例の症例報告を行いたい.
Two cases of the diencephalic syndrome were reported with hormonal studies.
The first case was initially admitted at her age of 4 years and 10 months old. Progressive visual disturbance and horizontal nystagmus had developed one year prior to the admission. Her weight gain had stopped since 10 months old. Partial removal of the hypothalamic pilocytic astrocytoma and 5,000 rad of lineac radiotherapy brought satisfactory effects on her previous symptoms. She was readmitted, however, at her age of 8 years and 10 months old because of precocious puberty.
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