Late-onset hypogonadism関連質問票を用いて自覚症状を評価したKallmann症候群の1例

Abstract

30歳男.第2次性徴の遅延, ED, 嗅覚低下を自覚し, 性腺機能低下症との類似を知り, 外陰部の増大および勃起機能の改善を希望した.頭部MRIにて両側嗅裂, 下垂体の形態に異常は認められず.内分泌学的検査およびホルモン負荷試験よりKallmann症候群と診断した.hCG5000単位およびhMG150単位を週2回の治療を開始し, 直後より勃起力の改善を自覚し, 12ヵ月後の時点で血中テストステロン, 遊離テストステロンは正常化し, LH, FSHは若干その増加を示し, PRLは正常範囲を推移した.陰茎長, 精巣容積も増加し, 陰毛も発達し精液検査でも改善されIIEF5は23点で, AMS総スコアは39点から20点となった

We report a case of Kallmann syndrome in which the aging male's symptoms rating scale (AMS) was useful for assessment of subjective symptoms. A 30-year-old male was admitted to Tsukuba University Hospital with a complaint of delayed puberty in May 2003. He presented with hypogonadism, gynecomastia and anosmia. The plasma levels of luteinizing hormone (LH), follicle stimulating hormone (FSH) and testosterone were very low. However, the LH-releasing hormone test and human chorionic gonadotropin (hCG) loading test identified normal function of pituitary and Leydig cell. After 12 months of treatment with hCG and human menopausal gonadotropin (hMG), the amount of pubic hair and the volume of testes had increased as well as the level of serum testosterone. The total score of AMS after treatment has been improved from 39 to 20 as compared with that before treatment.