Decreased energy expenditure is caused by abnormal body composition in infants with Prader-Willi Syndrome

Bekx, M. Tracy; Carrel, Aaron L.; Shriver, Timothy C.; Li, Zhanhai; Allen, David B.

doi:10.1067/s0022-3476(03)00386-x

Cited by 87 publications

(78 citation statements)

References 15 publications

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“…Our finding of decreased muscle thickness in infants with PWS is in line with the reported lower LBM in infants with PWS [17][18][19][20] and the early findings of type 2 muscle fiber atrophy and smaller type 1 muscle fiber size in infants with PWS. 39 Some studies reported that GH increases LBM (mainly determined by muscle mass) [20][21][22]26,29 ; however, in these studies, the interpretation of the reported results is problematic because the increase in LBM was not corrected for changes in height.…”

Section: Discussionsupporting

confidence: 81%

“…[10][11][12][13][14][15][16] It is presumed that the motor problems are related to an increased fat:muscle ratio even in underweight infants with PWS. [17][18][19] In infants with PWS, body fat percentages range from 28% to 32%, increasing to 36% to 55% [20][21][22][23] during childhood; in developmentally normal infants, this percentage is 24%, decreasing to 18% during childhood. 17,18,20 In children and infants with PWS, body fat percentages decrease as a result of GH treatment, 18,20,21,[24][25][26] although the fat:muscle ratio does not normalize.…”

mentioning

confidence: 99%

“…[17][18][19] In infants with PWS, body fat percentages range from 28% to 32%, increasing to 36% to 55% [20][21][22][23] during childhood; in developmentally normal infants, this percentage is 24%, decreasing to 18% during childhood. 17,18,20 In children and infants with PWS, body fat percentages decrease as a result of GH treatment, 18,20,21,[24][25][26] although the fat:muscle ratio does not normalize. 27 Dual-energy radiograph absorptiometry revealed a positive GH effect on lean body mass (LBM), which mainly contains muscle tissue.…”

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confidence: 99%

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Growth Hormone Therapy, Muscle Thickness, and Motor Development in Prader-Willi Syndrome: An RCT

Reus

Pillen

Pelzer³

et al. 2014

Pediatrics

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WHAT'S KNOWN ON THIS SUBJECT: Infants with Prader-Willi syndrome suffer from hypotonia, muscle weakness, and motor developmental delay and have increased fat mass combined with decreased muscle mass. Growth hormone improves body composition and motor development.WHAT THIS STUDY ADDS: Ultrasound scans confirmed decreased muscle thickness in infants with Prader-Willi syndrome, which improved as result of growth hormone treatment. Muscle thickness was correlated to muscle strength and motor performance. Catch-up growth in muscle thickness was related to muscle use independent of growth hormone. abstract OBJECTIVE: To investigate the effect of physical training combined with growth hormone (GH) on muscle thickness and its relationship with muscle strength and motor development in infants with Prader-Willi syndrome (PWS). METHODS:In a randomized controlled trial, 22 infants with PWS (12.9 6 7.1 months) were followed over 2 years to compare a treatment group (n = 10) with a waiting-list control group (n = 12). Muscle thickness of 4 muscle groups was measured by using ultrasound. Muscle strength was evaluated by using the Infant Muscle Strength meter. Motor performance was measured with the Gross Motor Function Measurement. Analyses of variance were used to evaluate between-group effects of GH on muscle thickness at 6 months and to compare pre-and posttreatment (after 12 months of GH) values. Multilevel analyses were used to evaluate effects of GH on muscle thickness over time, and multilevel bivariate analyses were used to test relationships between muscle thickness, muscle strength, and motor performance. RESULTS:A significant positive effect of GH on muscle thickness (P , .05) was found. Positive relationships were found between muscle thickness and muscle strength (r = 0.61, P , .001), muscle thickness and motor performance (r = 0.81, P , .001), and muscle strength and motor performance (r = 0.76, P , .001).CONCLUSIONS: GH increased muscle thickness, which was related to muscle strength and motor development in infants with PWS. Catch-up growth was faster in muscles that are most frequently used in early development. Because this effect was independent of GH, it suggests a training effect.

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Section: Discussionsupporting

confidence: 81%

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confidence: 99%

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confidence: 99%

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Growth Hormone Therapy, Muscle Thickness, and Motor Development in Prader-Willi Syndrome: An RCT

Reus

Pillen

Pelzer³

et al. 2014

Pediatrics

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“…high fat mass and low lean tissue mass, which is already present in infancy [7]. Due to an increase in appetite (hyperphagia), which follows the early feeding difficulties and is already present in up to 25% of children with PWS before their third birthday [8], the child may rapidly become obese if access to food is not restricted and physical activity not encouraged.…”

Section: Accepted Manuscript Introductionmentioning

confidence: 99%

Low dose growth hormone treatment in infants and toddlers with Prader-Willi syndrome is comparable to higher dosage regimens

Scheermeyer

Harris

Hughes

et al. 2017

Growth Hormone & IGF Research

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“…Children with PWS have an abnormal body composition with a relatively high body fat percentage and a low lean body mass. Even in infants, who are underweight, body fat percentage is high (1,2). Sleep-related breathing disorders (SRBD) may also occur (3).…”

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confidence: 99%

Psychomotor Development in Infants with Prader-Willi Syndrome and Associations with Sleep-Related Breathing Disorders

et al. 2007

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Prader-Willi syndrome (PWS) is a neurogenetic disorder with hypotonia, psychomotor delay, obesity, short stature, and sleep-related breathing disorders. The aim of this study was to evaluate the association between psychomotor development and sleep-related breathing disorders in PWS infants. Bayley Scales of Infant Development were performed in 22 PWS infants, with a median (interquartile range, IQR) age of 1.8 (1.1-3.4) y, and a body mass index SD score (BMISDS) of -0.5 (-1.3 to 1.6). We evaluated psychomotor development in relation to results of polysomnography. Median (IQR) mental and motor development was 73.1% (64.3-79.6%) and 55.2% (46.5-63.1%) of normal children, respectively. All infants had sleep-related breathing disorders, mostly of central origin. The apnea hypopnea index was not associated with psychomotor development. Only four infants had obstructive sleep apnea syndrome (OSAS). They had a significantly delayed mental development of 65.5% (60.0 -70.3%) of normal. They had a median BMISDS of 1.4 (0.1-1.6), which tended to be higher than in those without OSAS. Our data indicate that psychomotor development in PWS infants is not related to central sleep-related breathing disorders, but infants with OSAS have more severely delayed mental development, suggesting that PWS infants should be screened for OSAS. (Pediatr Res 62: 221-224, 2007)

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Decreased energy expenditure is caused by abnormal body composition in infants with Prader-Willi Syndrome

Cited by 87 publications

References 15 publications

Growth Hormone Therapy, Muscle Thickness, and Motor Development in Prader-Willi Syndrome: An RCT

Growth Hormone Therapy, Muscle Thickness, and Motor Development in Prader-Willi Syndrome: An RCT

Low dose growth hormone treatment in infants and toddlers with Prader-Willi syndrome is comparable to higher dosage regimens

Psychomotor Development in Infants with Prader-Willi Syndrome and Associations with Sleep-Related Breathing Disorders

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