Assessing the growth of children with spinal muscular atrophy using specific curves: A retrospective cohort study
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View abstract on PubMed
Summary
This summary is machine-generated.Spinal muscular atrophy (SMA) growth charts reveal better nutritional status and body composition in children compared to WHO standards. Disease-specific curves improve assessment of growth and body fat in pediatric SMA patients.
Area Of Science
- Pediatric Endocrinology
- Neuromuscular Disorders
- Growth and Development
Background
- Children with spinal muscular atrophy (SMA) exhibit atypical growth patterns.
- Standard World Health Organization (WHO) growth charts may not accurately reflect growth in SMA.
- Disease-specific growth curves for SMA were established in 2021.
Purpose Of The Study
- To evaluate the application of SMA-specific growth curves in pediatric patients.
- To compare growth and body composition in SMA children using SMA-specific versus WHO growth standards.
- To assess nutritional status more accurately in children with SMA.
Main Methods
- Retrospective cohort study of 19 pediatric SMA patients.
- Monthly anthropometric measurements (length, weight) recorded from admission to discharge.
- Calculation of body mass index-for-age z scores (BMIz) and length-for-age z scores (LAzs) using WHO and SMA-specific curves.
- Assessment of triceps skinfold (TSFz) and mid-upper arm circumference z scores (MUACz) using WHO references.
Main Results
- SMA-specific curves indicated significantly different BMIz at admission compared to WHO curves (0.77 vs. -1.31, P < 0.01).
- Using SMA curves, 42.1% of children were classified as overweight, versus 0% with WHO curves.
- Triceps skinfold z scores correlated better with BMIz derived from SMA curves (R²=0.46) than WHO curves (R²=0.27).
- Longitudinal analysis showed stable BMIz, slight increases in height-for-age z scores, declining MUACz, and increasing TSFz over time.
Conclusions
- SMA-specific growth curves provide a more accurate assessment of nutritional status and body composition in children with SMA.
- The study cohort demonstrated increased fat accretion and stable BMI over the follow-up period.
- Disease-specific growth charts are crucial for monitoring growth in pediatric SMA.
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