Growth Charts for Prader-Willi Syndrome During Growth Hormone Treatment

Merlin G Butler1, Jaehoon Lee2, Devin M Cox3

  • 1University of Kansas Medical Center, Kansas City, KS, USA mbutler4@kumc.edu.

Clinical Pediatrics
|February 5, 2016
PubMed

Insights

Standardized growth curves were developed for children with Prader-Willi syndrome (PWS) undergoing growth hormone treatment. These new charts show growth hormone therapy improves height and weight in PWS individuals.

Area of Science:

  • Pediatrics
  • Endocrinology
  • Genetics

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder affecting multiple body systems, often leading to growth abnormalities.
  • Growth hormone deficiency is common in PWS, necessitating therapeutic interventions to optimize growth and development.
  • Existing growth charts may not accurately reflect growth patterns in PWS individuals receiving growth hormone treatment.

Purpose of the Study:

  • To develop syndrome-specific, standardized growth curves for individuals with Prader-Willi syndrome (PWS) aged 0-18 years receiving growth hormone (GH) therapy.
  • To provide clinicians with accurate tools for monitoring growth in GH-treated PWS patients.
  • To compare growth patterns in GH-treated PWS individuals against non-GH-treated PWS populations.

Main Methods:

  • Development of syndrome-specific growth curves using the LMS method for 171 PWS subjects (0-18 years) treated with GH.
  • Collection of anthropometric data including weight, height, head circumference, weight/length, and BMI.
  • Comparison with normative data and growth charts for non-GH-treated PWS individuals.

Main Results:

  • Standardized growth curves for PWS patients treated with growth hormone were established for 7 percentile ranges.
  • Growth hormone treatment demonstrated a normalization of stature and significant improvement in weight compared to non-GH-treated PWS individuals.
  • The developed charts allow for better tracking of growth trajectories in GH-treated PWS patients.

Conclusions:

  • Syndrome-specific growth charts are essential for accurately assessing growth in children with PWS receiving growth hormone therapy.
  • Growth hormone treatment significantly improves key growth parameters, including height and weight, in individuals with PWS.
  • These standardized growth curves provide valuable clinical guidance for managing GH therapy in PWS.

Related Concept Videos

Signs of Puberty01:27

Signs of Puberty

Puberty is a critical phase, typically beginning between the ages of 8 and 13 in girls and 9 and 14 in boys, though timing can vary based on genetics, environmental factors, and overall health. This period is characterized by the development of secondary sexual characteristics and the attainment of reproductive potential. Endocrine changes underpin puberty, with hormonal surges of Luteinizing Hormone (LH) and Follicle-Stimulating Hormone (FSH) instigated by Gonadotropin-Releasing Hormone (GnRH)...
1.9K
Nature and Nurture01:10

Nature and Nurture

Many human characteristics, like height, are shaped by both nature—in other words, by our genes—and by nurture, or our environment. For example, chronic stress during childhood inhibits the production of growth hormones and consequently reduces bone growth and height. Scientists estimate that 70-90% of variation in height is due to genetic differences among individuals, and 10-30% of variation in height is due to differences in the environments that individuals experience,...
22.8K
Growth of Cartilage and Bone Tissue01:27

Growth of Cartilage and Bone Tissue

Chondrocytes form a temporary cartilaginous model by dividing and secreting a thick gel-like extracellular matrix. Once the chondrocytes undergo programmed cell death, osteoblasts enter the site of the cartilaginous model. The process of replacing the temporary cartilaginous model with bone in an ordered manner is called endochondral ossification. In endochondral ossification, not all of the cartilage is replaced by bone tissue. Some cartilage that performs a protective and supportive function...
4.7K