Bone mineral density during 3 years of growth hormone in previously GH-treated young adults with PWS

被引:4
作者
Damen, Layla [1 ,2 ,3 ,4 ]
Grootjen, Lionne N. [1 ,2 ,3 ,4 ]
Donze, Stephany H. [1 ,2 ,3 ,4 ]
de Graaff, Laura C. G. [3 ,4 ,5 ]
van der Velden, Janielle A. E. M. [4 ,6 ]
Hokken-Koelega, Anita C. S. [1 ,2 ,3 ,4 ]
机构
[1] Dutch Growth Res Fdn, Rotterdam, Netherlands
[2] Erasmus MC, Dept Pediat, Subdiv Endocrinol, Sophia Childrens Hosp, Rotterdam, Netherlands
[3] Erasmus MC, Acad Ctr Rare Growth Disorders, Rotterdam, Netherlands
[4] Dutch Reference Ctr Prader Willi Syndrome, Rotterdam, Netherlands
[5] Erasmus MC, Div Endocrinol, Internal Med, Rotterdam, Netherlands
[6] Radboud Univ Nijmegen, Dept Pediat, Subdiv Endocrinol, Med Ctr,Amalia Childrens Hosp, Nijmegen, Netherlands
关键词
PRADER-WILLI-SYNDROME; X-RAY ABSORPTIOMETRY; BODY-COMPOSITION; FAT MASS; CHILDREN; DEFICIENCY; OSTEOPOROSIS; MULTICENTER; INFANTS; HEIGHT;
D O I
10.1530/EJE-20-1335
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective: In children with Prader-Willi syndrome (PWS), growth hormone (GH) treatment has positive effects on bone mineral density (BMD). Two 1-year studies did not show a difference between GH or placebo on BMD in young adults with PWS. However, there are no studies investigating BMD during longer-term GH treatment in young adults with PWS. Design: Open-label, a prospective study in 43 young adults with PWS. Methods: BMD of the total body (BMDTBSDS) and lumbar spine (BMAD(LS)SDS) measured by DXA. Results: In the total group, estimated mean (95% CI) of BMDTB remained similar during 3 years of GH, being -0.76 (-1.11 to -0.41) SDS at start and -0.90 (-1.27 to -0.54) SDS after 3 years (P = 0.11), as did BMAD(LS), being -0.36 (-0.72 to 0.01) SDS and -0.46 (-0.77 to -0.16) SDS, respectively (P = 0.16). In men, there was a significant decrease in BMDTBSDS during 3 years of GH, while BMAD(LS)SDS remained similar. In women, both BMDTBSDS and BMAD(LS)SDS remained similar. BMDTBSDS was associated with female sex, lean body mass and age. The majority of patients received sex steroid replacement therapy (SSRT). Conclusions: During 3 years of combined GH and SSRT treatment, BMD remained stable in the normal range in young adults with PWS. However, men showed a decline in BMDTBSDS, probably due to insufficient SSRT. We recommended to continue GH treatment in young adults with PWS and to start SSRT during adolescence unless puberty progresses normally.
引用
收藏
页码:773 / 782
页数:10
相关论文
共 49 条
  • [1] Bone Mineral Density in Children and Adolescents With Prader-Willi Syndrome: A Longitudinal Study During Puberty and 9 Years of Growth Hormone Treatment
    Bakker, N. E.
    Kuppens, R. J.
    Siemensma, E. P. C.
    van Wijngaarden, R. F. A. Tummers-de Lind
    Festen, D. A. M.
    Bindels-de Heus, G. C. B.
    Bocca, G.
    Haring, D. A. J. P.
    Hoorweg-Nijman, J. J. G.
    Houdijk, E. C. A. M.
    Jira, P. E.
    Lunshof, L.
    Odink, R. J.
    Oostdijk, W.
    Rotteveel, J.
    Van Alfen, A. A. E. M.
    Van Leeuwen, M.
    Van Wieringen, H.
    Wegdam-den Boer, M. E. J.
    Zwaveling-Soonawala, N.
    Hokken-Koelega, A. C. S.
    [J]. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2015, 100 (04) : 1609 - 1618
  • [2] Eight Years of Growth Hormone Treatment in Children With Prader-Willi Syndrome: Maintaining the Positive Effects
    Bakker, N. E.
    Kuppens, R. J.
    Siemensma, E. P. C.
    van Wijngaarden, R. F. A. Tummers-de Lind
    Festen, D. A. M.
    Bindels-de Heus, G. C. B.
    Bocca, G.
    Haring, D. A. J. P.
    Hoorweg-Nijman, J. J. G.
    Houdijk, E. C. A. M.
    Jira, P. E.
    Lunshof, L.
    Odink, R. J.
    Oostdijk, W.
    Rotteveel, J.
    Schroor, E. J.
    Van Alfen, A. A. E. M.
    Van Leeuwen, M.
    Van Pinxteren-Nagler, E.
    Van Wieringen, H.
    Vreuls, R. C. F. M.
    Zwaveling-Soonawala, N.
    de Ridder, M. A. J.
    Hokken-Koelega, A. C. S.
    [J]. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2013, 98 (10) : 4013 - 4022
  • [3] Longitudinal changes of lumbar bone mineral density (BMD) in patients with GH deficiency after discontinuation of treatment at final height; timing and peak values for lumbar BMD
    Baroncelli, GI
    Bertelloni, S
    Sodini, F
    Saggese, G
    [J]. CLINICAL ENDOCRINOLOGY, 2004, 60 (02) : 175 - 184
  • [4] Reference Intervals for Insulin-like Growth Factor-1 (IGF-I) From Birth to Senescence: Results From a Multicenter Study Using a New Automated Chemiluminescence IGF-I Immunoassay Conforming to Recent International Recommendations
    Bidlingmaier, Martin
    Friedrich, Nele
    Emeny, Rebecca T.
    Spranger, Joachim
    Wolthers, Ole D.
    Roswall, Josefine
    Koerner, Antje
    Obermayer-Pietsch, Barbara
    Huebener, Christoph
    Dahlgren, Jovanna
    Frystyk, Jan
    Pfeiffer, Andreas F. H.
    Doering, Angela
    Bielohuby, Maximilian
    Wallaschofski, Henri
    Arafat, Ayman M.
    [J]. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2014, 99 (05) : 1712 - 1721
  • [5] Determinants of body composition measured by dual-energy X-ray absorptiometry in Dutch children and adolescents
    Boot, AM
    Bouquet, J
    deRidder, MAJ
    Krenning, EP
    KeizerSchrama, SMPFD
    [J]. AMERICAN JOURNAL OF CLINICAL NUTRITION, 1997, 66 (02) : 232 - 238
  • [6] Peak bone mineral density, lean body mass and fractures
    Boot, Annemieke M.
    de Ridder, Maria A. J.
    van der Sluis, Inge M.
    van Slobbe, Ingrid
    Krenning, Eric P.
    Keizer-Schrama, Sabine M. P. F. de Muinck
    [J]. BONE, 2010, 46 (02) : 336 - 341
  • [7] Analysis of Circulating Mediators of Bone Remodeling in Prader-Willi Syndrome
    Brunetti, G.
    Grugni, G.
    Piacente, L.
    Delvecchio, M.
    Ventura, A.
    Giordano, P.
    Grano, M.
    D'Amato, G.
    Laforgia, D.
    Crino, A.
    Faienza, M. F.
    [J]. CALCIFIED TISSUE INTERNATIONAL, 2018, 102 (06) : 635 - 643
  • [8] Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study
    Butler, JV
    Whittington, JE
    Holland, AJ
    Boer, H
    Clarke, D
    Webb, T
    [J]. DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY, 2002, 44 (04) : 248 - 255
  • [9] Effects of growth hormone treatment in adults with Prader-Willi syndrome
    Butler, M. G.
    Smith, B. K.
    Lee, J.
    Gibson, C.
    Schmoll, C.
    Moore, W. V.
    Donnelly, J. E.
    [J]. GROWTH HORMONE & IGF RESEARCH, 2013, 23 (03) : 81 - 87
  • [10] Decreased bone mineral density in Prader-Willi syndrome: Comparison with obese subjects
    Butler, MG
    Haber, L
    Mernaugh, R
    Carlson, MG
    Price, R
    Feurer, ID
    [J]. AMERICAN JOURNAL OF MEDICAL GENETICS, 2001, 103 (03): : 216 - 222