Growth patterns of Chinese patients with Prader-Willi syndrome

Area of Science:

• Pediatrics
• Genetics
• Endocrinology

Background:

• Prader-Willi syndrome (PWS) is a complex genetic disorder affecting multiple systems.
• Understanding PWS growth patterns in diverse populations is crucial for effective management.
• Previous studies on PWS growth have primarily focused on Caucasian populations.

Purpose of the Study:

• To investigate the spontaneous growth and weight gain patterns in patients with Prader-Willi syndrome (PWS) in Mainland China.
• To compare the observed growth data with standardized growth curves and Caucasian PWS data.
• To identify the optimal timing for potential interventions.

Main Methods:

• Retrospective analysis of 120 PWS cases diagnosed between 1994 and 2014 in Mainland China.
• Comparison of patient growth data (height, weight, BMI) with standardized growth charts.
• Analysis of growth patterns relative to age and comparison with established PWS growth data.

Main Results:

• Birth length was similar to the normal population, but birth weight was below the 50th centile.
• After age 5, 43% of patients fell below the 3rd centile for height.
• By age 3, 65.9% exceeded the 97th centile for weight, with 92.9% showing obesity (BMI > 97th centile) by age 2-3.
• Final mean adult heights were significantly below average Chinese heights for both males and females.
• The observed growth pattern in Chinese PWS patients is comparable to that of Caucasian PWS patients.

Conclusions:

• PWS patients in Mainland China exhibit significant growth retardation and early-onset obesity.
• The growth trajectory aligns with that observed in Caucasian PWS populations.
• Early intervention with recombinant human growth hormone is recommended due to early growth issues and obesity onset.