Growth Hormone Therapy for Children and Adolescents with Prader-Willi Syndrome Is Associated with Improved Body Composition and Metabolic Status in Adulthood

被引:49
作者
Coupaye, Muriel [1 ,2 ,3 ,4 ]
Lorenzini, Francoise [4 ,5 ]
Lloret-Linares, Celia [1 ,2 ,3 ,4 ]
Molinas, Catherine [4 ,6 ,7 ,8 ]
Pinto, Graziella [4 ,9 ]
Diene, Gwenaelle [4 ]
Mimoun, Emmanuelle [4 ,6 ,7 ]
Demeer, Genevieve [4 ,10 ]
Labrousse, Florence [4 ,5 ]
Jauregi, Joseba [4 ,10 ,11 ]
Laurier, Virginie [4 ,10 ]
Basdevant, Arnaud [1 ,2 ,3 ,4 ,12 ,13 ]
Polak, Michel [4 ,9 ,14 ,15 ]
Thuilleaux, Denise [4 ,10 ]
Tauber, Maithe [4 ,6 ,7 ,8 ]
Poitou, Christine [1 ,2 ,3 ,4 ,12 ,13 ]
机构
[1] Hop La Pitie Salpetriere, Assistance Publ Hop Paris, Nutr & Endocrinol Dept, F-75013 Paris, France
[2] Ctr Rech Nutr Humaine, F-75013 Paris, France
[3] Inst Cardiol Metab & Nutr, F-75013 Paris, France
[4] French Reference Ctr Prader Willi Syndrome, F-31059 Toulouse, France
[5] Hop Rangueil, Dept Endocrinol Metab Dis & Nutr, F-31059 Toulouse, France
[6] Childrens Hosp, Dept Endocrinol Bone Dis Genet & Gynaecol, F-31059 Toulouse, France
[7] Univ Toulouse 3, F-31062 Toulouse, France
[8] INSERM, Team 12 1043, Human Physiopathol Ctr, F-31059 Toulouse, France
[9] Necker Enfants Malades Hosp, Assistance Publ Hop Paris, Paediat Endocrinol & Diabet Unit, F-75015 Paris, France
[10] Hendaye Hosp, Assistance Publ Hop Paris, Prader Willi Unit, F-647000 Hendaye, France
[11] Univ Basque Country, Fac Psychol, San Sebastian, Spain
[12] INSERM, U872, Team Nutri 7, Cordeliers Res Ctr, F-75006 Paris, France
[13] Univ Paris 06, UMR S 872, F-75006 Paris, France
[14] CNRS, UPR 2147, F-75015 Paris, France
[15] Univ Paris 05, F-75015 Paris, France
关键词
ENERGY-EXPENDITURE; ADIPOSE-TISSUE; GH THERAPY; OBESITY; ENDOCRINE; IMPAIRMENT; HEIGHT; SLEEP; RISK; MASS;
D O I
10.1210/jc.2012-2881
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Context: Children with Prader-Willi syndrome (PWS) who receive GH treatment have improved growth and body composition; however, data are lacking for adults when treatment is discontinued after completion of growth. Objectives: Our aim was to compare body composition and metabolic status in adults with PWS according to GH treatment in childhood and adolescence. Design: 64 adults (mean age: 25.4 years) with a genetic diagnosis of PWS were evaluated: 20 received GH in childhood (T), which had been discontinued at the time of this study, and 44 did not receive GH (C). Mean duration of treatment in the T group was 4.4 +/- 2.7 years, age at baseline was 11.8 +/- 2.7 years, mean time between the end of treatment and the current evaluation was 7.0 +/- 4.4 years. Main Outcomes Measures: Dual-energy X-ray absorptiometry was used to assess body composition and fasting biological analyses evaluated metabolic status. Results (mean +/- SD): Body mass index and percentage of fat mass were significantly lower in the T group (32.4 +/- 10.3 vs 41.2 +/- 11.1 kg/m(2), P = 0.05 and 44.0 +/- 9.6 vs 50.1 +/- 7.2%, P = 0.02, respectively). Insulinemia and HOMA-IR in non-diabetic subjects were significantly lower in the T group (5.8 +/- 5.9 vs 13.9 +/- 11.6 mu UI/ml, P = 0.03, and 1.6 +/- 1.3 vs 2.7 +/- 2.1, P = 0.04, respectively). Non-diabetic and diabetic subjects from the T group had a significantly lower HbA1c. Lipid profiles were similar between groups. Conclusions: GH treatment in childhood and adolescence is associated with significantly decreased body mass index and improved body composition and metabolic status in adults with PWS at several years after discontinuing treatment. (J Clin Endocrinol Metab 98: E328-E335, 2013)
引用
收藏
页码:E328 / E335
页数:8
相关论文
共 45 条
[1]   Final adult height in children with Prader-Willi syndrome with and without human growth hormone treatment [J].
Angulo, Moris A. ;
Castro-Magana, Mariano ;
Lamerson, Michele ;
Arguello, Raul ;
Accacha, Siham ;
Khan, Asjad .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2007, 143A (13) :1456-1461
[2]  
[Anonymous], 2007, DIABETES CARE, V30, P4, DOI [DOI 10.2337/DC07-S004, 10.2337/dc07-S004]
[3]   Decreased energy expenditure is caused by abnormal body composition in infants with Prader-Willi syndrome [J].
Bekx, MT ;
Carrel, AL ;
Shriver, TC ;
Li, ZH ;
Allen, DB .
JOURNAL OF PEDIATRICS, 2003, 143 (03) :372-376
[4]  
BRAY GA, 1992, INT J OBESITY, V16, pS13
[5]   Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome: a population-based study [J].
Butler, JV ;
Whittington, JE ;
Holland, AJ ;
Boer, H ;
Clarke, D ;
Webb, T .
DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY, 2002, 44 (04) :248-255
[6]   Energy expenditure and phvsical activity in Prader-Willi syndrome: Comparison with obese subjects [J].
Butler, Merlin G. ;
Theodoro, Mariana F. ;
Bittel, Douglas C. ;
Donnelly, Joseph E. .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2007, 143A (05) :449-459
[7]  
Butler MG, 2011, CURR GENOMICS, V12, P204, DOI 10.2174/138920211795677877
[8]   Long-Term Growth Hormone Therapy Changes the Natural History of Body Composition and Motor Function in Children with Prader-Willi Syndrome [J].
Carrel, Aaron L. ;
Myers, Susan E. ;
Whitman, Barbara Y. ;
Eickhoff, Jens ;
Allen, David B. .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2010, 95 (03) :1131-1136
[9]   Benefits of long-term GH therapy in Prader-Willi syndrome: A 4-year study [J].
Carrel, AL ;
Myers, SE ;
Whitman, BY ;
Allen, DB .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2002, 87 (04) :1581-1585
[10]  
Cassidy SB, 1997, AM J MED GENET, V68, P433