Frank Giton1, Séverine Trabado, Luigi Maione, Julie Sarfati, Yves Le Bouc, Sylvie Brailly-Tabard, Jean Fiet, Jacques Young. 1. 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.
Abstract
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.
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.
Authors: John J Stocking; Michael V Fiandalo; Elena A Pop; John H Wilton; Gissou Azabdaftari; James L Mohler Journal: J Natl Compr Canc Netw Date: 2016-09 Impact factor: 11.908
Authors: Robert P Millar; Charlotte Sonigo; Richard A Anderson; Jyothis George; Luigi Maione; Sylvie Brailly-Tabard; Philippe Chanson; Nadine Binart; Jacques Young Journal: J Endocr Soc Date: 2017-10-16