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Prader-Willi syndrome: a single center's experience in Korea.

Kim YJ, Cheon CK - Korean J Pediatr (2014)

Bottom Line: Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age.There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment.Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Pusan National University School of Medicine, Yangsan, Korea.

ABSTRACT

Purpose: Prader-Willi syndrome (PWS) is a complex genetic disorder that results from the lack of paternally expressed genes in the chromosome 15q11-q13 region. This study was performed to delineate the clinical features of PWS infants and toddlers and the effects of two-year growth hormone (GH) treatment according to gender and age at the start of treatment.

Methods: The clinical characteristics and the results of the GH treatment were reviewed retrospectively for 30 PWS patients diagnosed by molecular genetic testing and clinical manifestations.

Results: The mean age at diagnosis with PWS was 13.7 months (2-47 months of age). All patients showed the characteristics of facial dysmorphism, including brown hair and almond-shaped eyes. Most patients showed developmental delays/mental retardation (93.3%), cryptorchidism (75%), feeding problems in infancy (73.3%), and neonatal or infantile hypotonia (66.7%). Among 30 patients, 14 PWS infants and toddlers had been treated with GH for more than two years. Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age. There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment.

Conclusion: Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

No MeSH data available.


Related in: MedlinePlus

Effect of growth hormone (GH) treatment on gross motor development in Prader-Willi syndrome by age at the start of GH treatment.
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Figure 1: Effect of growth hormone (GH) treatment on gross motor development in Prader-Willi syndrome by age at the start of GH treatment.

Mentions: We compared the results of GH treatment for children under two years (n=6) (10.66±5.76 months) and older than two years of age (n=8) (43.65±19.34 months) at the start of treatment. There were significant increases in IGFBP-3 SDS among individuals under two years of age compared with individuals older than two years of age after GH treatment (P=0.014) (Table 3). Psychomotor data were obtained using the DDST in GH-treated patients with PWS according to the age of GH start (Table 4). The changes in both gross and fine motor development were observed during the second year of study among individuals younger than two years of age who obtained GH therapy: Change was from 62.37%±13.68% to 77.02%±14.97% (P=0.028) in gross motor development and from 63.00%±10.96% to 77.11%±11.63% (P=0.028) in fine motor development, respectively (Figs.1, 2).


Prader-Willi syndrome: a single center's experience in Korea.

Kim YJ, Cheon CK - Korean J Pediatr (2014)

Effect of growth hormone (GH) treatment on gross motor development in Prader-Willi syndrome by age at the start of GH treatment.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4127393&req=5

Figure 1: Effect of growth hormone (GH) treatment on gross motor development in Prader-Willi syndrome by age at the start of GH treatment.
Mentions: We compared the results of GH treatment for children under two years (n=6) (10.66±5.76 months) and older than two years of age (n=8) (43.65±19.34 months) at the start of treatment. There were significant increases in IGFBP-3 SDS among individuals under two years of age compared with individuals older than two years of age after GH treatment (P=0.014) (Table 3). Psychomotor data were obtained using the DDST in GH-treated patients with PWS according to the age of GH start (Table 4). The changes in both gross and fine motor development were observed during the second year of study among individuals younger than two years of age who obtained GH therapy: Change was from 62.37%±13.68% to 77.02%±14.97% (P=0.028) in gross motor development and from 63.00%±10.96% to 77.11%±11.63% (P=0.028) in fine motor development, respectively (Figs.1, 2).

Bottom Line: Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age.There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment.Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Pusan National University School of Medicine, Yangsan, Korea.

ABSTRACT

Purpose: Prader-Willi syndrome (PWS) is a complex genetic disorder that results from the lack of paternally expressed genes in the chromosome 15q11-q13 region. This study was performed to delineate the clinical features of PWS infants and toddlers and the effects of two-year growth hormone (GH) treatment according to gender and age at the start of treatment.

Methods: The clinical characteristics and the results of the GH treatment were reviewed retrospectively for 30 PWS patients diagnosed by molecular genetic testing and clinical manifestations.

Results: The mean age at diagnosis with PWS was 13.7 months (2-47 months of age). All patients showed the characteristics of facial dysmorphism, including brown hair and almond-shaped eyes. Most patients showed developmental delays/mental retardation (93.3%), cryptorchidism (75%), feeding problems in infancy (73.3%), and neonatal or infantile hypotonia (66.7%). Among 30 patients, 14 PWS infants and toddlers had been treated with GH for more than two years. Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age. There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment.

Conclusion: Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

No MeSH data available.


Related in: MedlinePlus