孤立性低促性腺激素性性腺功能减退和全垂体功能减退男性的性类固醇、前体和代谢物缺乏：一项基于气相色谱-质谱联用的比较研究。
Sex steroids, precursors, and metabolite deficiencies in men with isolated hypogonadotropic hypogonadism and panhypopituitarism: a GCMS-based comparative study.
作者信息
Giton Frank, Trabado Séverine, Maione Luigi, Sarfati Julie, Le Bouc Yves, Brailly-Tabard Sylvie, Fiet Jean, Young Jacques
机构信息
Assistance Publique-Hôpitaux de Paris (F.G., S.T., J.S., Y.L.B., S.B.-T., J.Y.); Centre d'Investigations Biologiques (F.G.), Hôpital Henri Mondor, F-94010 Créteil, France; INSERM Unité 955 Eq07 (F.G., J.F.), Centre de Recherches Chirurgicales, Faculté de Médecine Paris Est, Université Paris Est, F-77454 Marne-la-Vallée; Faculté de Médecine Paris-Sud (S.T., J.S., S.B.-T., J.Y.), Université Paris-Sud, Laboratoire de Génétique Moléculaire, Pharmacogénétique, et Hormonologie (S.T., S.B.-T.) and Service d'Endocrinologie et des Maladies de la Reproduction (L.M., J.S., J.Y.), Hopital Bicêtre, and INSERM Unité 693 (S.T., S.B.-T., J.Y.), F-94275 Le Kremlin Bicêtre, France; and Laboratoire d'Explorations Fonctionnelles (Y.L.B.), Hôpital Trousseau, F-75571 Paris, France; Université Pierre et Marie Curie (Y.L.B.), F-75005 Paris, France; and INSERM Unité Mixte de Recherche en Santé 938 (Y.L.B.), F-75020 Paris, France.
出版信息
J Clin Endocrinol Metab. 2015 Feb;100(2):E292-6. doi: 10.1210/jc.2014-2658. Epub 2014 Nov 13.
CONTEXT
Both testicular and adrenal steroid secretions are impaired in men with panhypopituitarism (Hypo-Pit), whereas only testicular steroid secretion is impaired in men with isolated gonadotropin deficiency (IHH) caused by normosmic congenital hypogonadotropic hypogonadism or Kallmann syndrome.
OBJECTIVE
The objective of the study was to compare the serum levels of sex steroids, precursors, and metabolites between men with complete IHH and those with Hypo-Pit.
PATIENTS
We studied 42 healthy men, 16 untreated men with IHH (normosmic congenital hypogonadotropic hypogonadism/Kallmann syndrome) and 23 men with Hypo-Pit (14 with craniopharyngioma, 9 with congenital hypopituitarism) receiving hydrocortisone, thyroxine, and GH replacement therapy but not T.
METHODS
Gas chromatography/mass spectrometry (GCMS) was used to measure the serum levels of sex steroids [T, dihydrotestosterone (DHT), and estradiol (E2)], their precursors (pregnenolone, 17-hydroxypregnenolone, dehydroepiandrosterone, androstenediol, progesterone, 17-hydroxyprogesterone, and androstenedione), and their metabolites (androsterone, estrone, and estrone sulfate) as well as pregnenolone and dehydroepiandrosterone sulfate esters.
RESULTS
All the above-mentioned steroids, and notably T, DHT, and E2, were significantly lower in IHH patients than in controls but remained well above the detection limit of the relevant assays. In Hypo-Pit men, all these steroids were dramatically and significantly lower than in IHH. Interestingly, T, DHT, and E2, as well as pregnenolone and dehydroepiandrosterone sulfate esters, were undetectable or barely detectable in the Hypo-Pit men.
CONCLUSIONS
Steroid deficiencies are marked but partial in men with complete IHH. In contrast, men with Hypo-Pit have a very severe overall steroid deficiency. These deficiencies could affect health and quality of life.
背景
全垂体功能减退症（Hypo - Pit）男性的睾丸和肾上腺类固醇分泌均受损，而由嗅觉正常的先天性低促性腺激素性性腺功能减退症或卡尔曼综合征引起的孤立性促性腺激素缺乏症（IHH）男性仅睾丸类固醇分泌受损。
目的
本研究的目的是比较完全性IHH男性与Hypo - Pit男性的血清性激素、前体和代谢产物水平。
患者
我们研究了42名健康男性、16名未经治疗的IHH男性（嗅觉正常的先天性低促性腺激素性性腺功能减退症/卡尔曼综合征）和23名Hypo - Pit男性（14名患有颅咽管瘤，9名患有先天性垂体功能减退症），这些Hypo - Pit男性接受氢化可的松、甲状腺素和生长激素替代治疗，但未接受睾酮治疗。
方法
采用气相色谱/质谱联用（GCMS）法测定血清性激素水平[睾酮（T）、双氢睾酮（DHT）和雌二醇（E2）]、其前体（孕烯醇酮、17 - 羟孕烯醇酮、脱氢表雄酮、雄烯二醇、孕酮、17 - 羟孕酮和雄烯二酮）及其代谢产物（雄酮、雌酮和硫酸雌酮）以及孕烯醇酮和硫酸脱氢表雄酮酯。
结果
所有上述类固醇，尤其是T、DHT和E2，在IHH患者中显著低于对照组，但仍远高于相关检测方法的检测限。在Hypo - Pit男性中，所有这些类固醇均显著低于IHH患者。有趣的是，在Hypo - Pit男性中，T、DHT和E2以及孕烯醇酮和硫酸脱氢表雄酮酯无法检测到或仅勉强可检测到。
结论
完全性IHH男性存在明显但部分的类固醇缺乏。相比之下，Hypo - Pit男性存在非常严重的整体类固醇缺乏。这些缺乏可能影响健康和生活质量。
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