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

in European Journal of Endocrinology
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  • 1 Dutch Growth Research Foundation, Rotterdam, the Netherlands
  • 2 Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center-Sophia Children’s Hospital, Rotterdam, the Netherlands
  • 3 Academic Center for Rare Growth Disorders, Erasmus University Medical Center, Rotterdam, the Netherlands
  • 4 Dutch Reference Center for Prader-Willi Syndrome, Rotterdam, the Netherlands
  • 5 Internal Medicine, Division of Endocrinology, Erasmus University Medical Center, Rotterdam, the Netherlands
  • 6 Department of Pediatrics, Subdivision of Endocrinology, Radboud University Medical Center-Amalia Children’s Hospital, Nijmegen, the Netherlands

Correspondence should be addressed to L Damen Email l.damen@kindengroei.nl
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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 (BMADLSSDS) 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 BMADLS, 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 BMADLSSDS remained similar. In women, both BMDTBSDS and BMADLSSDS 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.

 

     European Society of Endocrinology

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