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Erschienen in: Journal of Orthopaedic Science 6/2014

01.11.2014 | Original Article

Growth hormone treatment for osteoporosis in patients with scoliosis of Prader-Willi syndrome

verfasst von: Yutaka Nakamura, Nobuyuki Murakami, Takahiro Iida, Satoshi Asano, Satoru Ozeki, Toshiro Nagai

Erschienen in: Journal of Orthopaedic Science | Ausgabe 6/2014

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Abstract

Background

Patients with Prader-Willi syndrome (PWS) have fragile bones. Osteoporosis is a major concern in scoliosis surgery. Our aim was to investigate bone mineral density (BMD) in PWS patients and to verify the efficacy of and scoliosis deterioration with growth hormone (GH) administration for osteoporosis.

Methods

We followed 148 PWS patients who underwent lumbar spine (L2–4) BMD testing. Sixty-four patients had scoliosis, and 84 were non-scoliosis patients. Patients were treated with GH (0.245 mg/kg/week) until they reached a skeletal age of 17 years for males and 15 years for females. We also evaluated the effect of GH treatment on BMD in 101 patients (60 males, 41 females) undergoing BMD testing more than twice. The mean patient age was 5.4 years. The mean duration of GH administration was 54 months.

Results

Mean lumbar BMD was 0.567 g/cm2. Fifty patients (33.8 %) had osteoporosis and 41 (27.7 %) had osteopenia. There was no significant difference in mean BMD between patients with scoliosis (0.598 g/cm2) and without scoliosis (0.548 g/cm2). GH treatment caused a significant increase in Z score (pre-GH: mean −2.28 vs. post-GH: mean −1.53, P < 0.001). There was no statistical difference in the prevalence of scoliosis between the GH treatment group (45/112, 40.1 %) and non-treatment group (19/36, 52.8 %).

Conclusions

Among patients with PWS, 61.5 % had low BMDs. GH administration significantly improved the lumbar BMD (Z score). There were no statistically significant differences in the prevalence of scoliosis among patients who received GH treatment compared to patients who did not.
Literatur
1.
Zurück zum Zitat Vestergaard P, Kristensen K, Bruun JM, Østergaard JR, Heickendorff L, Mosekilde L, Richelsen B. Reduced bone mineral density and increased bone turnover in Prader-Willi syndrome compared with controls matched for sex and body mass index–a cross-sectional study. J Pediatr. 2004;144:614–9.PubMedCrossRef Vestergaard P, Kristensen K, Bruun JM, Østergaard JR, Heickendorff L, Mosekilde L, Richelsen B. Reduced bone mineral density and increased bone turnover in Prader-Willi syndrome compared with controls matched for sex and body mass index–a cross-sectional study. J Pediatr. 2004;144:614–9.PubMedCrossRef
2.
Zurück zum Zitat Butler MG, Haber L, Mernaugh R, Carlson MG, Price R, Feurer ID. Decreased bone mineral density in Prader-Willi syndrome: comparison with obese subjects. Am J Med Genet. 2001;103:216–22.PubMedCrossRef Butler MG, Haber L, Mernaugh R, Carlson MG, Price R, Feurer ID. Decreased bone mineral density in Prader-Willi syndrome: comparison with obese subjects. Am J Med Genet. 2001;103:216–22.PubMedCrossRef
3.
Zurück zum Zitat Holm VA, Laurnen EL. Prader-Willi syndrome and scoliosis. Dev Med Child Neurol. 1981;23:192–201.PubMedCrossRef Holm VA, Laurnen EL. Prader-Willi syndrome and scoliosis. Dev Med Child Neurol. 1981;23:192–201.PubMedCrossRef
4.
Zurück zum Zitat Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol. 2002;44:248–55.PubMedCrossRef Butler JV, Whittington JE, Holland AJ, Boer H, Clarke D, Webb T. Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study. Dev Med Child Neurol. 2002;44:248–55.PubMedCrossRef
5.
Zurück zum Zitat Kroonen LT, Herman M, Pizzutillo PD. Macewen GD Prader-Willi syndrome: clinical concerns for the orthopaedic surgeon. J Pediatr Orthop. 2006;26:673–9.PubMedCrossRef Kroonen LT, Herman M, Pizzutillo PD. Macewen GD Prader-Willi syndrome: clinical concerns for the orthopaedic surgeon. J Pediatr Orthop. 2006;26:673–9.PubMedCrossRef
6.
Zurück zum Zitat Nagai T, Obata K, Ogata T, Murakami N, Katada Y, Yoshino A, Sakazume S, Tomita Y, Sakuta R, Niikawa N. Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome. Am J Med Genet A. 2006;140:1623–7.PubMedCrossRef Nagai T, Obata K, Ogata T, Murakami N, Katada Y, Yoshino A, Sakazume S, Tomita Y, Sakuta R, Niikawa N. Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome. Am J Med Genet A. 2006;140:1623–7.PubMedCrossRef
7.
Zurück zum Zitat de Lind van Wijngaarden RF, Klerk LW, Festen DA, Hokken-Koelega AC. Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008;93:1012–6.PubMedCrossRef de Lind van Wijngaarden RF, Klerk LW, Festen DA, Hokken-Koelega AC. Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008;93:1012–6.PubMedCrossRef
8.
Zurück zum Zitat Nakamura Y, Nagai T, Iida T, Ozeki S, Nohara Y. Epidemiological aspects of scoliosis in a cohort of Japanese patients with Prader-Willi syndrome. Spine J. 2009;9:809–16.PubMedCrossRef Nakamura Y, Nagai T, Iida T, Ozeki S, Nohara Y. Epidemiological aspects of scoliosis in a cohort of Japanese patients with Prader-Willi syndrome. Spine J. 2009;9:809–16.PubMedCrossRef
9.
Zurück zum Zitat Accadbled F, Odent T, Moine A, Chau E, Glorion C, Diene G, de Gauzy JS. Complications of scoliosis surgery in Prader-Willi syndrome. Spine. 2008;33:394–401.PubMedCrossRef Accadbled F, Odent T, Moine A, Chau E, Glorion C, Diene G, de Gauzy JS. Complications of scoliosis surgery in Prader-Willi syndrome. Spine. 2008;33:394–401.PubMedCrossRef
10.
Zurück zum Zitat Myers SE, Whitman BY, Carrel AL, Moerchen V, Bekx MT, Allen DB. Two years of growth hormone therapy in young children with Prader-Willi syndrome: physical and neurodevelopmental benefits. Am J Med Genet A. 2007;143:443–8.CrossRef Myers SE, Whitman BY, Carrel AL, Moerchen V, Bekx MT, Allen DB. Two years of growth hormone therapy in young children with Prader-Willi syndrome: physical and neurodevelopmental benefits. Am J Med Genet A. 2007;143:443–8.CrossRef
11.
Zurück zum Zitat Mogul HR, Lee PD, Whitman BY, Zipf WB, Frey M, Myers S, Cahan M, Pinyerd B, Southren AL. Growth hormone treatment of adults with Prader-Willi syndrome and growth hormone deficiency improves lean body mass, fractional body fat, and serum triiodothyronine without glucose impairment: results from the United States multicenter trial. J Clin Endocinol Metab. 2008;93:1238–45.CrossRef Mogul HR, Lee PD, Whitman BY, Zipf WB, Frey M, Myers S, Cahan M, Pinyerd B, Southren AL. Growth hormone treatment of adults with Prader-Willi syndrome and growth hormone deficiency improves lean body mass, fractional body fat, and serum triiodothyronine without glucose impairment: results from the United States multicenter trial. J Clin Endocinol Metab. 2008;93:1238–45.CrossRef
12.
Zurück zum Zitat Nakamura Y, Nagai T, Iida T, Ozeki S. Nohara Y Growth hormone supplement treatment reduces the surgical risk for Prader-Willi syndrome patients. Eur Spine J. 2012;21(Suppl 4):483–91.PubMedCentralCrossRef Nakamura Y, Nagai T, Iida T, Ozeki S. Nohara Y Growth hormone supplement treatment reduces the surgical risk for Prader-Willi syndrome patients. Eur Spine J. 2012;21(Suppl 4):483–91.PubMedCentralCrossRef
13.
Zurück zum Zitat Glastre Catherine, Braillon Pierre, David Louis. Pierre cochat, Pierre J. Meunier, Pierre D. Delmas mesurement of bone mineral content of the lumbar spine by dual energy x-ray absorptionmetry in normal children: correlations with growth parameters. J Endocrinol Metab. 1990;70(5):1330–3.CrossRef Glastre Catherine, Braillon Pierre, David Louis. Pierre cochat, Pierre J. Meunier, Pierre D. Delmas mesurement of bone mineral content of the lumbar spine by dual energy x-ray absorptionmetry in normal children: correlations with growth parameters. J Endocrinol Metab. 1990;70(5):1330–3.CrossRef
14.
Zurück zum Zitat Hiroyuki Tanaka. Bone mineral density. Pediatr Jpn. 2005;46:17–9 (in Japanese). Hiroyuki Tanaka. Bone mineral density. Pediatr Jpn. 2005;46:17–9 (in Japanese).
15.
Zurück zum Zitat Orito S, Kuroda T, Onoe Y, Sato Y, Ohta H. Age-related distribution of bone and skeletal parameters in 1322 Japanese young women. J Bone Miner Metab. 2009;27:698–704.PubMedCrossRef Orito S, Kuroda T, Onoe Y, Sato Y, Ohta H. Age-related distribution of bone and skeletal parameters in 1322 Japanese young women. J Bone Miner Metab. 2009;27:698–704.PubMedCrossRef
16.
Zurück zum Zitat Orimo H, Sugioka Y, Fukunaga H, Mutou H, Hotokebuti T, Gomei I, Nakamura T, Kushida K, Tanaka H, Ikai T, Ohashi Y. Guidline on the primary osteoporosis of Japanese Society for Bone and Mineral Research. Osteoporosis. 1996;4:643–53 (in Japanese). Orimo H, Sugioka Y, Fukunaga H, Mutou H, Hotokebuti T, Gomei I, Nakamura T, Kushida K, Tanaka H, Ikai T, Ohashi Y. Guidline on the primary osteoporosis of Japanese Society for Bone and Mineral Research. Osteoporosis. 1996;4:643–53 (in Japanese).
17.
Zurück zum Zitat Nagai T, Ogata T, Murakami N. Guideline for the human Growth hormone treatment of Prader-Willi syndrome. Foundation for growth science. 2009; 5 (in Japanese). Nagai T, Ogata T, Murakami N. Guideline for the human Growth hormone treatment of Prader-Willi syndrome. Foundation for growth science. 2009; 5 (in Japanese).
18.
Zurück zum Zitat Van Mil EG, Westerterp KR, Gerver WJ. Van Marken Lichtenbelt WD, Kester AD, Saris WH. Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: relationship to physical activity and growth hormone function. J Pediatr. 2001;139:708–14.PubMedCrossRef Van Mil EG, Westerterp KR, Gerver WJ. Van Marken Lichtenbelt WD, Kester AD, Saris WH. Body composition in Prader-Willi syndrome compared with nonsyndromal obesity: relationship to physical activity and growth hormone function. J Pediatr. 2001;139:708–14.PubMedCrossRef
19.
Zurück zum Zitat Höybye C, Hilding A, Jacobsson H, Thorén M. Metabolic profile and body composition in adults with Prader-Willi syndrome and severe obesity. J Clin Endocinol Metab. 2002;87:3590–7.CrossRef Höybye C, Hilding A, Jacobsson H, Thorén M. Metabolic profile and body composition in adults with Prader-Willi syndrome and severe obesity. J Clin Endocinol Metab. 2002;87:3590–7.CrossRef
20.
Zurück zum Zitat Muscatelli F, Abrous DN, Massacrier A, Boccaccio I, Le Moal M, Cau P, Cremer H. Disruption of the mouse Necdin gene results in hypothalamic and behavioral alterations reminiscent of the human Prader-Willi syndrome. Hum Mol Genet. 2000;9:3101–10.PubMedCrossRef Muscatelli F, Abrous DN, Massacrier A, Boccaccio I, Le Moal M, Cau P, Cremer H. Disruption of the mouse Necdin gene results in hypothalamic and behavioral alterations reminiscent of the human Prader-Willi syndrome. Hum Mol Genet. 2000;9:3101–10.PubMedCrossRef
21.
Zurück zum Zitat Hiroyuki Tanaka J. Growth hormone and bone. Clin calcium. 2009;19(7):984 (in Japanese).PubMed Hiroyuki Tanaka J. Growth hormone and bone. Clin calcium. 2009;19(7):984 (in Japanese).PubMed
22.
Zurück zum Zitat Casey RW, Barkin J. Testosterone replacement therapy for the primary care physician. Can J Urol. 2008;15(Suppl 1):71–7.PubMed Casey RW, Barkin J. Testosterone replacement therapy for the primary care physician. Can J Urol. 2008;15(Suppl 1):71–7.PubMed
23.
Zurück zum Zitat Carrel AL, Myers SE, Whitman BY, Allen DB. Benefits of long-term GH therapy in Prader-Willi syndrome: a 4-year study. J Clin Endocrinol Metab. 2002;87(4):1581–5.PubMedCrossRef Carrel AL, Myers SE, Whitman BY, Allen DB. Benefits of long-term GH therapy in Prader-Willi syndrome: a 4-year study. J Clin Endocrinol Metab. 2002;87(4):1581–5.PubMedCrossRef
24.
Zurück zum Zitat de Lind van Wijngaarden RF, Festen DA, Otten BJ, van Mil EG, Rotteveel J, Odink RJ, van Leeuwen M, Haring DA, Bocca G, Mieke Houdijk EC, Hokken-Koelega AC. Bone mineral density and effects of growth hormone treatment in prepubertal children with Prader-Willi syndrome: a randomized controlled trial. J Clin Endocrinol Metab. 2009;94(10):3763–71.PubMedCrossRef de Lind van Wijngaarden RF, Festen DA, Otten BJ, van Mil EG, Rotteveel J, Odink RJ, van Leeuwen M, Haring DA, Bocca G, Mieke Houdijk EC, Hokken-Koelega AC. Bone mineral density and effects of growth hormone treatment in prepubertal children with Prader-Willi syndrome: a randomized controlled trial. J Clin Endocrinol Metab. 2009;94(10):3763–71.PubMedCrossRef
25.
Zurück zum Zitat Docquier PL, Mousny M, Jouret M, Bastin C, Rombouts JJ. Orthopaedic concerns in children with growth hormone therapy. Acta Orthop Belg. 2004;70:299–305.PubMed Docquier PL, Mousny M, Jouret M, Bastin C, Rombouts JJ. Orthopaedic concerns in children with growth hormone therapy. Acta Orthop Belg. 2004;70:299–305.PubMed
26.
Zurück zum Zitat Odent T, Accadbled F, Koureas G, Cournot M, Moine A, Diene G, Molinas C, Pinto G, Tauber M, Gomes B. de Gauzy JS, Glorion C Scoliosis in patients with Prader-Willi syndrome. Pediatrics. 2008;122:e499–503.PubMedCrossRef Odent T, Accadbled F, Koureas G, Cournot M, Moine A, Diene G, Molinas C, Pinto G, Tauber M, Gomes B. de Gauzy JS, Glorion C Scoliosis in patients with Prader-Willi syndrome. Pediatrics. 2008;122:e499–503.PubMedCrossRef
Metadaten
Titel
Growth hormone treatment for osteoporosis in patients with scoliosis of Prader-Willi syndrome
verfasst von
Yutaka Nakamura
Nobuyuki Murakami
Takahiro Iida
Satoshi Asano
Satoru Ozeki
Toshiro Nagai
Publikationsdatum
01.11.2014
Verlag
Springer Japan
Erschienen in
Journal of Orthopaedic Science / Ausgabe 6/2014
Print ISSN: 0949-2658
Elektronische ISSN: 1436-2023
DOI
https://doi.org/10.1007/s00776-014-0641-0

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