title: Testosterone replacement therapy to improve secondary sexual characteristics and body composition without adverse behavioral problems in adult male patients with Prader-Willi syndrome: an observational study.
abstract: Prader-Willi syndrome (PWS), a complex genetic disorder, arises from suppressed expression of paternally inherited imprinted genes on chromosome 15q11-q13. Characteristics include short stature, intellectual disability, behavioral problems, hypogonadism, obesity, and reduced bone and muscle mass. Testosterone replacement (TR) remains controversial due to concerns regarding behavioral problems. To evaluate the effects of TR on secondary sexual characteristics, body composition, and behavior in adult males with PWS, 22 male PWS patients over the age of 16 with behavioral scores of less than grade 4 on the Modified Overt Aggression Scale (MOAS) underwent monthly intramuscular TR (125 mg). Pubertal change, body composition and behavior were evaluated before and after 24 months of therapy. Serum testosterone, LH, and FSH did not change. Increased pubic hair was observed in 16 of 22 patients (72.7%). Percent body fat decreased from 47.55 ± 2.06% to 39.75 ± 1.60% (n = 18) (P = 0.018). Bone mineral density increased from 0.8505 ± 0.0426 g/cm(2) to 0.9035 ± 0.0465 g/cm(2) (n = 18) (P = 0.036), and lean body mass increased from 18093.4 ± 863.0 g to 20312.1 ± 1027.2 g (n = 18) (P = 0.009). The MOAS was unchanged, from 4.5 ± 2.0 at the beginning of the study to 3.0 ± 1.7 at the end of study indicating no increase in aggression. No behavioral problems were observed. Based on this pilot study, TR with 125 mg monthly is a potentially safe and useful intervention for adult males with PWS.
authors.affiliation: Department of Pediatrics, Dokkyo Medical University Koshigaya Hospital, Saitama, Japan. kidoyasu@dokkyomed.ac.jp
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kido
authors.forename: Satoru
authors.initials: S
authors.lastname: Sakazume
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Abe
authors.forename: Yuji
authors.initials: Y
authors.lastname: Oto
authors.forename: Hisashi
authors.initials: H
authors.lastname: Itabashi
authors.forename: Masahisa
authors.initials: M
authors.lastname: Shiraishi
authors.forename: Atsunori
authors.initials: A
authors.lastname: Yoshino
authors.forename: Yuriko
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Kazuo
authors.initials: K
authors.lastname: Obata
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Murakami
authors.forename: Toshiro
authors.initials: T
authors.lastname: Nagai
mesh_terms: D000293:Adolescent; D000328:Adult; D001519:Behavior / Q000187:drug effects*; D001823:Body Composition / Q000187:drug effects*; D015992:Body Mass Index; D020249:Hormone Replacement Therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D011218:Prader-Willi Syndrome / Q000097:blood / Q000188:drug therapy*; D012741:Sexual Maturation / Q000187:drug effects*; D013739:Testosterone / Q000494:pharmacology* / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D013739:Testosterone
keywords: Prader-Willi syndrome; hypogonadism; testosterone replacement therapy