Literature DB >> 24001750

Eight years of growth hormone treatment in children with Prader-Willi syndrome: maintaining the positive effects.

N E Bakker1, R J Kuppens, E P C Siemensma, R F A Tummers-de Lind van Wijngaarden, D A M Festen, G C B Bindels-de Heus, G Bocca, D A J P Haring, J J G Hoorweg-Nijman, E C A M Houdijk, P E Jira, L Lunshof, R J Odink, W Oostdijk, J Rotteveel, E J Schroor, A A E M Van Alfen, M Van Leeuwen, E Van Pinxteren-Nagler, H Van Wieringen, R C F M Vreuls, N Zwaveling-Soonawala, M A J de Ridder, A C S Hokken-Koelega.   

Abstract

BACKGROUND: The most important reason for treating children with Prader-Willi syndrome (PWS) with GH is to optimize their body composition.
OBJECTIVES: The aim of this ongoing study was to determine whether long-term GH treatment can counteract the clinical course of increasing obesity in PWS by maintaining the improved body composition brought during early treatment.
SETTING: This was a multicenter prospective cohort study.
METHODS: We have been following 60 prepubertal children for 8 years of continuous GH treatment (1 mg/m(2)/d ≈ 0.035 mg/kg/d) and used the same dual-energy x-ray absorptiometry machine for annual measurements of lean body mass and percent fat.
RESULTS: After a significant increase during the first year of GH treatment (P < .0001), lean body mass remained stable for 7 years at a level above baseline (P < .0001). After a significant decrease in the first year, percent fat SD score (SDS) and body mass index SDS remained stable at a level not significantly higher than at baseline (P = .06, P = .14, resp.). However, body mass index SDSPWS was significantly lower after 8 years of GH treatment than at baseline (P < .0001). After 8 years of treatment, height SDS and head circumference SDS had completely normalized. IGF-1 SDS increased to +2.36 SDS during the first year of treatment (P < .0001) and remained stable since then. GH treatment did not adversely affect glucose homeostasis, serum lipids, blood pressure, and bone maturation.
CONCLUSION: This 8-year study demonstrates that GH treatment is a potent force for counteracting the clinical course of obesity in children with PWS.

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Year:  2013        PMID: 24001750     DOI: 10.1210/jc.2013-2012

Source DB:  PubMed          Journal:  J Clin Endocrinol Metab        ISSN: 0021-972X            Impact factor:   5.958


  38 in total

Review 1.  Review of Prader-Willi syndrome: the endocrine approach.

Authors:  Ryan Heksch; Manmohan Kamboj; Kathryn Anglin; Kathryn Obrynba
Journal:  Transl Pediatr       Date:  2017-10

Review 2.  Growth hormone treatment in non-growth hormone-deficient children.

Authors:  Sandro Loche; Luisanna Carta; Anastasia Ibba; Chiara Guzzetti
Journal:  Ann Pediatr Endocrinol Metab       Date:  2014-03-31

Review 3.  Update on Diabetes Mellitus and Glucose Metabolism Alterations in Prader-Willi Syndrome.

Authors:  Antonino Crinò; Graziano Grugni
Journal:  Curr Diab Rep       Date:  2020-02-06       Impact factor: 4.810

4.  Behavior in children with Prader-Willi syndrome before and during growth hormone treatment: a randomized controlled trial and 8-year longitudinal study.

Authors:  Sin T Lo; Elbrich P C Siemensma; Dederieke A M Festen; Philippe J L Collin; Anita C S Hokken-Koelega
Journal:  Eur Child Adolesc Psychiatry       Date:  2014-12-19       Impact factor: 4.785

Review 5.  Prader- Willi syndrome: An uptodate on endocrine and metabolic complications.

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Journal:  Rev Endocr Metab Disord       Date:  2019-06       Impact factor: 6.514

Review 6.  Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach.

Authors:  Silvia Savastano; Giovanna Muscogiuri; Luigi Barrea; Claudia Vetrani; Danilo Fintini; Giulia de Alteriis; Filippo Maria Panfili; Sarah Bocchini; Ludovica Verde; Annamaria Colao
Journal:  Curr Obes Rep       Date:  2022-09-05

7.  Progression of Obstructive Sleep Apnea Syndrome in Pediatric Patients with Prader-Willi Syndrome.

Authors:  Shi-Bing Wong; Mei-Chen Yang; I-Shiang Tzeng; Wen-Hsin Tsai; Chou-Chin Lan; Li-Ping Tsai
Journal:  Children (Basel)       Date:  2022-06-17

Review 8.  Prader Willi Syndrome: Genetics, Metabolomics, Hormonal Function, and New Approaches to Therapy.

Authors:  Krystal A Irizarry; Mark Miller; Michael Freemark; Andrea M Haqq
Journal:  Adv Pediatr       Date:  2016-08

9.  Pediatric Obesity-Assessment, Treatment, and Prevention: An Endocrine Society Clinical Practice Guideline.

Authors:  Dennis M Styne; Silva A Arslanian; Ellen L Connor; Ismaa Sadaf Farooqi; M Hassan Murad; Janet H Silverstein; Jack A Yanovski
Journal:  J Clin Endocrinol Metab       Date:  2017-03-01       Impact factor: 5.958

10.  Correlation of Genotype and Perinatal Period, Time of Diagnosis and Anthropometric Data before Commencement of Recombinant Human Growth Hormone Treatment in Polish Patients with Prader-Willi Syndrome.

Authors:  Agnieszka Lecka-Ambroziak; Marta Wysocka-Mincewicz; Katarzyna Doleżal-Ołtarzewska; Agata Zygmunt-Górska; Teresa Żak; Anna Noczyńska; Dorota Birkholz-Walerzak; Renata Stawerska; Maciej Hilczer; Monika Obara-Moszyńska; Barbara Rabska-Pietrzak; Elżbieta Gołębiowska; Adam Dudek; Elżbieta Petriczko; Mieczysław Szalecki
Journal:  Diagnostics (Basel)       Date:  2021-04-28
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