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
[21]   Visceral adipose tissue and metabolic complications of obesity are reduced in Prader-Willi syndrome female adults: Evidence for novel influences on body fat distribution [J].
Goldstone, AP ;
Thomas, EL ;
Brynes, AE ;
Bell, JD ;
Frost, G ;
Saeed, N ;
Hajnal, JV ;
Howard, JK ;
Holland, A ;
Bloom, SR .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2001, 86 (09) :4330-4338
[22]   Growth hormone therapy improves exercise capacity in adult patients with Prader-Willi syndrome [J].
Gondoni, L. A. ;
Vismara, L. ;
Marzullo, P. ;
Vettor, R. ;
Liuzzi, A. ;
Grugni, G. .
JOURNAL OF ENDOCRINOLOGICAL INVESTIGATION, 2008, 31 (09) :765-772
[23]   European guidelines on cardiovascular disease prevention in clinical practice: Full text [J].
Graham, Ian ;
Atar, Dan ;
Borch-Johnsen, Knut ;
Boysen, Gudrun ;
Burell, Gunilla ;
Cifkova, Renata ;
Dallongeville, Jean ;
De Backer, Guy ;
Ebrahim, Shah ;
Gjelsvik, Bjorn ;
Herrmann-Lingen, Christoph ;
Hoes, Arno ;
Humphries, Steve ;
Knapton, Mike ;
Perk, Joep ;
Priori, Silvia G. ;
Pyorala, Kalevi ;
Reiner, Zeljko ;
Ruilope, Luis ;
Sans-Menendez, Susana ;
Reimer, Wilma Scholte Op ;
Weissbergh, Peter ;
Wood, David ;
Yarnell, John ;
Zamorano, Jose Luis .
EUROPEAN JOURNAL OF CARDIOVASCULAR PREVENTION & REHABILITATION, 2007, 14 :S1-S113
[24]   Impairment of GH responsiveness to combined GH-releasing hormone and arginine administration in adult patients with Prader-Willi syndrome [J].
Grugni, G. ;
Marzullo, P. ;
Ragusa, L. ;
Sartorio, A. ;
Trifiro, G. ;
Liuzzi, A. ;
Crino, A. .
CLINICAL ENDOCRINOLOGY, 2006, 65 (04) :492-499
[25]   Effects of growth hormone on pulmonary function, sleep quality, behavior, cognition, growth velocity, body composition, and resting energy expenditure in Prader- Willi syndrome [J].
Haqq, AM ;
Stadler, DD ;
Jackson, RH ;
Rosenfeld, RG ;
Purnell, JQ ;
LaFranchi, SH .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2003, 88 (05) :2206-2212
[26]   Five-years growth hormone (GH) treatment in adults with Prader-Willi syndrome [J].
Hoybye, Charlotte .
ACTA PAEDIATRICA, 2007, 96 (03) :410-413
[27]   Growth hormone treatment completely normalizes adult height and improves body composition in Prader-Willi syndrome: Experience from KIGS (Pfizer international growth database) [J].
Lindgren, A. C. ;
Lindberg, A. .
HORMONE RESEARCH, 2008, 70 (03) :182-187
[28]   Conditional cardiovascular response to growth hormone therapy in adult patients with Prader-Willi syndrome [J].
Marzullo, Paolo ;
Marcassa, Claudio ;
Campini, Riccardo ;
Eleuteri, Ermanno ;
Minocci, Alessandro ;
Sartorio, Alessandro ;
Vettor, Roberto ;
Liuzzi, Antonio ;
Grugni, Graziano .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2007, 92 (04) :1364-1371
[29]   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].
Mogul, Harriette R. ;
Lee, Phillip D. K. ;
Whitman, Barbara Y. ;
Zipf, William B. ;
Frey, Michael ;
Myers, Susan ;
Cahan, Mindy ;
Pinyerd, Belinda ;
Southren, A. Louis .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2008, 93 (04) :1238-1245
[30]   Adult patients with Prader-Willi syndrome:: clinical characteristics, life circumstances and growth hormone secretion [J].
Partsch, CJ ;
Lämmer, C ;
Gillessen-Kaesbach, G ;
Pankau, R .
GROWTH HORMONE & IGF RESEARCH, 2000, 10 :81-85