Sustained benefit after 2 years of growth hormone on body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome

Myers, Susan E.; Carrel, Aaron L.; Whitman, Barbara Y.; Allen, David B.

doi:10.1067/mpd.2000.105369

Cited by 142 publications

(146 citation statements)

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“…Preliminary data also suggest that it may have a beneficial effect on weight gain, and possibly appetite, in individuals with PWS. 33,35 Infants with PWS treated with GH therapy have improvements in head circumference, height, BMI, body composition (with improvement of lean muscle mass and delay of fat tissue accumulation), body proportions, acquisition of gross motor skills, language acquisition, and cognitive scores. [36][37][38][39][40][41][42][43] Older children and adolescents treated with GH therapy not only have the aforementioned physical benefits of the treatment but are also reported to have improvements in behaviors with lack of behavioral deterioration during adolescence.…”

Section: Hypogonadismmentioning

confidence: 99%

“…GH treatment normalizes height, increases lean body mass, decreases fat mass, and increases mobility, which are beneficial to weight management. 4,[35][36][37][38][39][41][42][43] Dose recommendations in children are generally similar to those for individuals with isolated GH deficiency, i.e., approximately 1 mg/m2. 42,147 Educational planning should be instigated, and speech therapy is often needed for articulation abnormalities and delays.…”

Section: Paternal Deletionmentioning

confidence: 99%

“…GH replacement therapy to normalize height, increase lean body mass, mobility and activity level, and decrease fat mass is standard of care and can be started at any age. 4,[35][36][37][38][39][41][42][43] Studies thus far have indicated that no age is too early for GH treatment, and the sooner it is started the more benefit it has. 42 It is recommended that a sleep study be performed before initiation of GH therapy to assess for and treat obstructive apnea.…”

Section: Managementmentioning

confidence: 99%

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Prader-Willi syndrome

Cassidy

Schwartz

Miller

et al. 2012

Genetics in Medicine

1,074

1,155

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Section: Hypogonadismmentioning

confidence: 99%

Section: Paternal Deletionmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

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Prader-Willi syndrome

Cassidy

Schwartz

Miller

et al. 2012

Genetics in Medicine

1,074

1,155

View full text Add to dashboard Cite

“…Fasting and 2-hour postprandial glucose and insulin, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, free fatty acid, triglyceride, free thyroxine, and thyroid-stimulating hormone levels were monitored at baseline and (with the exception of GH) at 12 and 24 months. These data are presented in Myers et al 21 Height was measured on a Harpenden stadiometer at Ϫ6, 0, 6, 12, 18, and 24 months. Bone age was determined annually by a single pediatric radiologist and spine films screening for scoliosis were obtained at baseline and, if indicated, at 6-month intervals.…”

Section: Patientsmentioning

confidence: 99%

The Behavioral Impact of Growth Hormone Treatment for Children and Adolescents With Prader-Willi Syndrome: A 2-Year, Controlled Study

et al. 2002

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ABSTRACT. Introduction. Prader-Willi syndrome (PWS) is characterized by obesity, hypotonia, hypogonadism, hyperphagia, short stature, and a neurobehavioral profile that includes cognitive deficits, learning problems, and behavioral difficulties that increase in both quantity and severity over time. PWS results from an alteration in the molecular composition of a critical region of C#15q. Morbid obesity resulting from hyperphagia is amplified by decreased energy expenditure and reduced physical activity. The hyperphagia has proven refractory to all psychopharmocologic intervention; the behavioral components are equally resistant to psychotropic intervention. PWS patients' body composition resembles that of individuals with growth hormone (GH) deficiency, including short stature and reduced lean body mass with concomitant increased fat mass. We hypothesized that GH administration to children with PWS, in addition to stimulating linear growth, would improve body composition, increase energy expenditure and fat utilization, and improve muscle strength, physical agility, and pulmonary function. Two recent reports from this study document significant positive effects of GH treatment on these children's physical parameters measured in a 2-year, controlled study. However, the behavioral impact of GH treatment in this population remains incompletely described.A psychosocial burden, including emotional, behavioral, and cognitive disturbances associated with short stature, has been previously described in a non-PWS population with GH deficiency and idiopathic short stature. An impaired quality of life and psychosocial status is also documented in otherwise normal adults with GH deficiency. In both populations, growth hormone replacement therapy (GHRT) is reported to improve alertness, activity level, endurance, irritability, tendency to worry, and extroversion resulting in better personal relationships with fewer conflicts. This report focuses on that portion of the study investigating the behavioral and psychosocial outcomes accompanying increased stature and improved physical status for persons with PWS treated with GHRT. We hypothesized that, as in other populations, GHRT for persons with PWS would have a significant positive effect on their psychosocial status as well as an improvement in their growth parameters. Methods.A 2-year, controlled study with control group crossover in the second year was used. Fifty-four consecutive children with genetically confirmed PWS were enrolled. Patients were 4 to 16 years of age at time of enrollment, had skeletal maturation <13 for girls and <15 for boys; all but 3 participants remained prepubertal (Tanner stage 1) throughout the study. Children who had previous therapy with GH were excluded, as were children with a scoliosis >20°. The SDI contains a second section measuring behavior functioning in the school environment, in the family, and in personal and social relationships. A wider scoring range is used and is question-specific. Parallel forms of this measure are available for ...

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“…또한 최근의 논 문들은 PWS에서 성장호르몬 치료가 신체활동 및 정신사회적인 기능을 향상시킬 뿐만 아니라 [16][17][18] , 호흡근의 근력을 강화하여 호 흡기능에도 긍적적인 영향을 준다고 보고하고 있다 16,19) . …”

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Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study

et al. 2008

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Purpose : The objective of this study was to evaluate the effects and adverse side-effects of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). Methods : Forty-one patients who had been treated with GH for more than two years (24 boys and 17 girls, mean age 7.3 ±3.3 years during treatment initiation) were enrolled for this study. Results : After 2 years of GH therapy, the height and weight standard deviation scores (SDS) increased significantly (1.19± 1.37 vs. 0.02±1.45, and 1.02±2.42 vs. 1.63±2.22, P<0.002); however the percentage body fat decreased (44.6±9.9% vs. 38.1±10.5%, P<0.001). Further, no change was observed in the thyroid and serum glucose levels, but the total cholesterol level decreased. GH therapy did not impact glucose control in the patients with diabetes. The most common adverse effects of GH therapy were the progression of scoliosis and adenoid hypertrophy. Conclusion : GH therapy improved the height SDS and body composition in patients with PWS. However, GH should be used with caution in patients with scoliosis and adenoid hypertrophy. (Korean J Pediatr 2008;51:742-746)

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Sustained benefit after 2 years of growth hormone on body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome

Cited by 142 publications

References 20 publications

Prader-Willi syndrome

Prader-Willi syndrome

The Behavioral Impact of Growth Hormone Treatment for Children and Adolescents With Prader-Willi Syndrome: A 2-Year, Controlled Study

Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study

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