Ann Pediatr Endocrinol Metab.  2012 Sep;17(3):175-178. 10.6065/apem.2012.17.3.175.

Growth Hormone Treatment and Its Effect on Height in Pediatric Patients with Different Genotypes of Prader-Willi Syndrome

Affiliations
  • 1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. jindk@skku.edu

Abstract

PURPOSE
Differences in phenotypes between the two most common subtypes of Prader-Willi syndrome (PWS) indicate that a distinct response to growth hormone (GH) treatment may exist. To test this hypothesis, we compared the results of GH treatment in individuals with PWS due to uniparental disomy (UPD) to those of individuals with deletions.
METHODS
Sixty-five children with PWS who had been treated with GH for more than two years were included in this study. Twenty-one individuals were confirmed as having UPD and 44 individuals had a deletion. Height, body weight, body mass index (BMI), and insulin like growth factor-1 (IGF-I) measurements were recorded before GH treatment and at intervals of 12 months thereafter.
RESULTS
After two years of GH therapy, no significant differences were noted for yearly improvements in height standard deviation scores (SDS) between the groups (second year SDS, 0.93 +/- 0.94; deletion, 0.84 +/- 1.31; UPD, P = 0.717). Body weight SDS, BMI SDS, and IGF-I SDS also showed no differences between the two groups.
CONCLUSION
Our study showed no significant differences in yearly improvements in height SDS between the deletion and UPD groups, at least for the first two years.

Keyword

Prader-Willi syndrome; Growth hormone; Sequence deletion; Uniparental disomy

MeSH Terms

Body Height
Body Weight
Child
Genotype
Growth Hormone
Humans
Insulin
Insulin-Like Growth Factor I
Phenotype
Prader-Willi Syndrome
Sequence Deletion
Uniparental Disomy
Growth Hormone
Insulin
Insulin-Like Growth Factor I
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