title: Bone mineral density, body composition and bone turnover in patients with congenital hypogonadotropic hypogonadism.
abstract: Patients with congenital hypogonadotropic hypogonadism (HH) may have reduced peak bone mass in early adulthood, and increased risk for osteoporosis despite long-term hormonal replacement therapy (HRT). To investigate the relationship between HRT history and measures of bone health in patients with HH, we recruited 33 subjects (24 men, nine women; mean age 39.8 years, range: 24.0-69.1) with congenital HH (Kallmann syndrome or normosmic HH). They underwent clinical examination, were interviewed and medical charts were reviewed. Twenty-six subjects underwent dual-energy X-ray absorptiometry for evaluation of BMD of lumbar spine, hip, femoral neck and whole body; body composition and vertebral morphology were evaluated in 22 and 23 subjects, respectively. Circulating PINP, ICTP and sex hormone levels were measured. HRT history clearly associated to bone health: BMDs of lumbar spine, femoral neck, hip and whole body were lower in subjects (n = 9) who had had long (≥5 years) treatment pauses or low dose testosterone (T) treatment as compared to subjects without such history (n = 17; all p-values < 0.05). In addition, fat mass and body mass index (BMI) were significantly higher in men with deficient treatment history (median fat mass: 37.5 vs. 23.1%, p = 0.005; BMI: 32.6 vs. 25.2 kg/m(2), p < 0.05). Serum PINP correlated with ICTP (r(s) = 0.61; p < 0.005) in men, but these markers correlated neither with circulating T, nor with serum estradiol levels in women. In conclusion, patients with congenital HH require life-long follow-up to avoid inadequate HRT, long treatment pauses and further morbidity.
authors.affiliation: Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland. eeva-maria.laitinen@helsinki.fi
authors.forename: E-M
authors.initials: EM
authors.lastname: Laitinen
authors.forename: M
authors.initials: M
authors.lastname: Hero
authors.forename: K
authors.initials: K
authors.lastname: Vaaralahti
authors.forename: J
authors.initials: J
authors.lastname: Tommiska
authors.forename: T
authors.initials: T
authors.lastname: Raivio
mesh_terms: D015502:Absorptiometry, Photon; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001823:Body Composition; D015992:Body Mass Index; D015519:Bone Density; D001862:Bone Resorption; D024042:Collagen Type I / Q000097:blood; D004958:Estradiol / Q000097:blood; D005260:Female; D012739:Gonadal Steroid Hormones / Q000097:blood; D020249:Hormone Replacement Therapy; D006801:Humans; D007006:Hypogonadism / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010024:Osteoporosis / Q000097:blood / Q000150:complications*; D010446:Peptide Fragments / Q000097:blood; D010455:Peptides / Q000097:blood; D011347:Procollagen / Q000097:blood; D051496:Receptor, Fibroblast Growth Factor, Type 1 / Q000235:genetics; D013739:Testosterone / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D015415:Biomarkers; D024042:Collagen Type I; D012739:Gonadal Steroid Hormones; D010446:Peptide Fragments; D010455:Peptides; D011347:Procollagen; C052929:collagen type I trimeric cross-linked peptide; C047202:procollagen Type I N-terminal peptide; D013739:Testosterone; D004958:Estradiol; C496348:FGFR1 protein, human; D051496:Receptor, Fibroblast Growth Factor, Type 1