Growth Standards of Infants With Prader-Willi Syndrome

Merlin G. Butler,Jennifer Sturich,Daniel J. Driscoll,Virginia Kimonis,Susan E. Myers,Ann Scheimann,Norma Terrazas,June-Anne Gold,Jaehoon Lee,Barbara Y. Whitman

doi:10.1542/peds.2010-2736

Abstract

To generate and report standardized growth curves for weight, length, head circumference, weight/length, and BMI for non-growth hormone-treated white infants (boys and girls) with Prader-Willi syndrome (PWS) between 0 and 36 months of age. The goal was to monitor growth and compare data with other infants with PWS. Anthropometric measures (N = 758) were obtained according to standard methods and analyzed from 186 non-growth hormone-treated white infants (108 boys and 78 girls) with PWS between 0 and 36 months of age. Standardized growth curves were developed and the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles were calculated by using the LMS (refers to λ, μ, and σ) smoothing procedure method for weight, length, head circumference, weight/length, and BMI along with the normative 50th percentile using Centers for Disease Control and Prevention national growth data from 2003. The data were plotted for comparison purposes. Five separate standardized growth curves (weight, length, head circumference, weight/length, and BMI) representing 7 percentile ranges were developed from 186 non-growth hormone-treated white male and female infants with PWS aged 0 to 36 months, and the normative 50th percentile was plotted on each standardized infant growth curve. We encourage the use of these growth standards when examining infants with PWS and evaluating growth for comparison purposes, monitoring for growth patterns, nutritional assessment, and recording responses to growth hormone therapy, commonly used in infants and children with PWS.

Highlights

Prader-Willi syndrome (PWS), first reported in 1956,1 is a complex genomic imprinting disorder characterized in infancy by central hypotonia, a poor suck and feeding difficulties with failure to thrive, growth hormone deficiency, and hypogonadism
We developed standardized curves for weight, length, head circumference, weight/length, and BMI for non– growth hormone–treated white infants with PWS aged between 0 and 36 months to be used in the clinical setting for evaluation, management, and care of infants with PWS
The 50th percentile was calculated and plotted from normal infants using growth data collected from year 2003 by the Centers for Disease Control and Prevention (CDC)[12] and plotted on each standardized growth curve

Summary

Introduction

Prader-Willi syndrome (PWS), first reported in 1956,1 is a complex genomic imprinting disorder characterized in infancy by central hypotonia, a poor suck and feeding difficulties with failure to thrive, growth hormone deficiency, and hypogonadism. Hyperphagia occurs in early childhood, leading to obesity if left uncontrolled.[2,3,4,5] Mental deficiency is present with an average IQ of 65 along with behavioral problems (eg, obsessive-compulsive disorder, temper tantrums, skin-picking), hypopigmentation, small hands and feet, short stature, hypogonadism, hypogenitalism, and a characteristic facial appearance.[2,3,5,6,7]

Methods

Discussion

Conclusion