Bone Mineral Density in Children and Adolescents With Prader-Willi Syndrome
| Autor: | Nitash Zwaveling-Soonawala, Dederieke A. M. Festen, R. J. Kuppens, M. van Leeuwen, G. C. B. (Karen) Bindels-de Heus, Danny A. J. P. Haring, Euphemia C. A. M. Houdijk, L. Lunshof, R. J. Odink, R. F. A. Tummers-de Lind van Wijngaarden, Wilma Oostdijk, M. E. J. Wegdam-den Boer, Petr E. Jira, Anita C. S. Hokken-Koelega, J. J. G. Hoorweg-Nijman, Gianni Bocca, Elbrich P. C. Siemensma, A. A. E. M. Van Alfen, Joost Rotteveel, H. Van Wieringen, N. E. Bakker |
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| Přispěvatelé: | Pediatrics, Child and Adolescent Psychiatry / Psychology, General Practice, Erasmus MC other, Other departments, Other Research, Graduate School, Paediatric Endocrinology, Pediatric surgery, ICaR - Circulation and metabolism |
| Jazyk: | angličtina |
| Rok vydání: | 2015 |
| Předmět: |
Male
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Longitudinal study Time Factors BODY-COMPOSITION Adolescent Endocrinology Diabetes and Metabolism Clinical Biochemistry Context (language use) METABOLISM Biochemistry THERAPY law.invention Endocrinology HEIGHT Randomized controlled trial Bone Density law Internal medicine YOUNG-ADULTS medicine Humans Longitudinal Studies Young adult Child Gonadal Steroid Hormones Netherlands Bone mineral Human Growth Hormone business.industry Puberty Biochemistry (medical) nutritional and metabolic diseases X-RAY ABSORPTIOMETRY RANDOMIZED CONTROLLED-TRIAL LEAN MASS nervous system diseases Growth hormone treatment ESTROGEN Child Preschool Cohort GH DEFICIENCY Body Composition Lean body mass Female business Prader-Willi Syndrome |
| Zdroj: | Journal of Clinical Endocrinology and Metabolism, 100(4), 1609-1618. Endocrine Society Journal of Clinical Endocrinology and Metabolism, 100(4), 1609-1618 Bakker, N E, Kuppens, R J, Siemensma, E P C, van Wijngaarden, R F A T, 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 2015, ' Bone Mineral Density in Children and Adolescents With Prader-Willi Syndrome: A Longitudinal Study During Puberty and 9 Years of Growth Hormone Treatment ', Journal of Clinical Endocrinology and Metabolism, vol. 100, no. 4, pp. 1609-1618 . https://doi.org/10.1210/jc.2014-4347 Journal of clinical endocrinology and metabolism, 100(4), 1609-1618. The Endocrine Society Journal of Clinical Endocrinology and Metabolism, 100(4), 1609-1618. The Endocrine Society Journal of Clinical Endocrinology and Metabolism, 100(4), 1609-1618. ENDOCRINE SOC |
| ISSN: | 0021-972X |
| DOI: | 10.1210/jc.2014-4347 |
| Popis: | Context: Longitudinal data on bone mineral density(BMD) in children and adolescents with Prader-Willi Syndrome (PWS) during long-term GH treatment are not available.Objective: This study aimed to determine effects of long-term GH treatment and puberty on BMD of total body (BMDTB), lumbar spine (BMDLS), and bone mineral apparent density of the lumbar spine (BMAD(LS)) in children with PWS.Design and Setting: This was a prospective longitudinal study of a Dutch PWS cohort.Participants: Seventy-seven children with PWS who remained prepubertal during GH treatment for 4 years and 64 children with PWS who received GH treatment for 9 years participated in the study.Intervention: The children received GH treatment, 1 mg/m(2)/day (congruent to 0.035 mg/kg/d).Main Outcome Measures: BMDTB, BMDLS, and BMAD(LS) was measured by using the same dual-energy x-ray absorptiometry machine for all annual measurements.Results: In the prepubertal group, BMDTB standard deviation score (SDS) and BMDLSSDS significantly increased during 4 years of GH treatment whereas BMAD(LS)SDS remained stable. During adolescence, BMDTBSDS and BMAD(LS)SDS decreased significantly, in girls from the age of 11 years and in boys from the ages of 14 and 16 years, respectively, but all BMD parameters remained within the normal range. Higher Tanner stages tended to be associated with lower BMDTBSDS (P = .083) and a significantly lowerBMAD(LS)SDS (P = .016). After 9 years of GH treatment, lean body mass SDS was the most powerful predictor of BMDTBSDS and BMDLSSDS in adolescents with PWS.Conclusions: This long-term GH study demonstrates that BMDTB, BMDLS, and BMAD(LS) remain stable in prepubertal children with PWS but decreases during adolescence, parallel to incomplete pubertal development. Based on our findings, clinicians should start sex hormone therapy from the age of 11 years in girls and 14 years in boys unless there is a normal progression of puberty. |
| Databáze: | OpenAIRE |
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