Growth Standards of Infants With Prader-Willi Syndrome

Abstract

WHAT'S KNOWN ON THIS SUBJECT:Standardized growth curves do not exist for infants with Prader-Willi syndrome. Syndromespecific growth charts are required for monitoring growth and nutritional status, particularly during growth hormone treatment and follow-up for this disorder during infancy. WHAT THIS STUDY ADDS:Standardized growth curves are reported to be used in monitoring growth and development of infants with Prader-Willi syndrome of both genders from 0 to 36 months of age. abstract OBJECTIVE: To generate and report standardized growth curves for weight, length, head circumference, weight/length, and BMI for nongrowth hormone-treated white infants (boys and girls) with PraderWilli syndrome (PWS) between 0 and 36 months of age. The goal was to monitor growth and compare data with other infants with PWS. METHODS:Anthropometric measures (N ϭ 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 , , and ) 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. RESULTS: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. CONCLUSIONS: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.

Growth Charts for Non-Growth Hormone Treated Prader-Willi Syndrome

“…Growth charts for weight, height, head circumference, and BMI were developed for each gender by using the LMS method 13,14 as used previously in the development and reporting of standardized growth curves. 7 The LMS method is a standard technique for constructing age-related growth references and summarizes the distribution of an anthropometric variable Y at each value of a covariate (age) in terms of 3 parameters: BoxCox power l (L), median m (M), and coefficient of variation s (S):…”

Prader-Willi syndrome

“…[1][2][3] Prenatal hypotonia usually results in decreased fetal movement, abnormal fetal position at delivery, and increased incidence of assisted delivery or cesarean section. 1 …”

“…The 15q11.2-q13 region can be roughly divided into four distinct regions that are delineated by three common deletion breakpoints, 92 which lie within segmental duplications 93 (Figure 3): (1) a proximal nonimprinted region between the two common proximal breakpoints (BP1 and BP2) containing four biparentally expressed genes, NIPA1, NIPA2, CYF1P1, and GCP5. 94 (2) The "PWS paternal-only expressed region" containing five polypeptide coding genes (MKRN3, MAGEL2, NECDIN, and the bicistronic SNURF-SNRPN); C15orf2 (an intronless gene that is biallelically expressed in testis but only expressed from the paternal allele in brain); a cluster of C/D box small nucleolar RNA genes (snoRNAs); and several antisense transcripts (including the The following additional descriptions pertain to the diagnostic criteria. The hypotonia is central and improves with age.…”

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

“…Improvements in current neonatal nutritional intervention may explain the slightly positive value at baseline of both age groups (approximately 0.5 SDS PWS ) and updated PWS specific growth charts as currently available are valuable for future use in children up to 3 years of age [27].…”