Molecular Classes and Growth Hormone Treatment Effects on Behavior and Emotion in Patients with Prader–Willi Syndrome
Abstract
:1. Introduction
2. Materials and Methods
3. Results
3.1. Study Participants
3.2. Comparison by Molecular Genetic Class (DEL vs. UPD)
3.3. Comparison of Growth Hormone Treatment
3.4. Effect of Growth Hormone (GH) Treatment after Adjusting for Age in Patients with PWS
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Total | Age Groups | ||||||||
---|---|---|---|---|---|---|---|---|---|
n | Mean ± SD | n | Age < 11 years Mean ± SD | n | Age 11–18 years Mean ± SD | n | Age > 18 years Mean ± SD | p-Value | |
Age (years) | 330 | 13.4 ± 11.5 | 226 | 6 ± 2.5 | 84 | 14.3 ± 0.3 | 20 | 20.8 ± 0.5 | <0.001 |
Age started rhGH (years) | 219 | 4.6 ± 7.2 | 187 | 1 ± 2 | 22 | 4 ± 7.5 | 10 | 20.1 ± 0.5 | 0.001 |
BMI percentile for age and gender | 330 | 82 ± 23 | 226 | 80 ± 21 | 84 | 85 ± 22 | 92.5 ± 2.5 | 0.421 | |
BASC Results Statistics | |||||||||
Parent-reported behaviors: | |||||||||
Attention problems | 212 | 56.9 ± 9.3 | 159 | 54.6 ± 2.6 | 33 | 56.4 ± 2.3 | 20 | 67.33 ± 4.3 | 0.030 |
Hyperactivity | 213 | 56.8 ± 12.6 | 160 | 53.3 ± 3.3 | 33 | 64.6 ± 2.9 | 20 | 72.3 ± 2.3 | <0.001 |
Aggression | 213 | 50.8 ± 9.5 | 159 | 52.5 ± 3 | 34 | 55.3 ± 3 | 20 | 42.6 ± 2.6 | 0.012 |
Leadership | 133 | 39.3 ± 10 | 92 | 45.3 ± 1.9 | 26 | 42.1 ± 2.4 | 15 | 40.3 ± 7.5 | 0.229 |
Anger control | 100 | 56.7 ± 10.8 | 82 | 55.8 ± 3.4 | 10 | 64.8 ± 2.4 | 8 | 64 ± 2.6 | 0.064 |
Teacher-reported behaviors: | |||||||||
Attention problems | 153 | 56.8 ± 9.9 | 108 | 55.7 ± 3.4 | 30 | 52.8 ± 3.5 | 15 | 52.6 ± 7.3 | 0.477 |
Hyperactivity | 155 | 56.8 ± 11 | 56 | 54 ± 2.5 | 84 | 62.2 ± 3.3 | 15 | 56.3 ± 8 | <0.001 |
Aggression | 155 | 55.5 ± 10.4 | 110 | 53.1 ± 2.5 | 36 | 58.7 ± 3.3 | 9 | 52.3 ± 4.6 | 0.156 |
Leadership | 95 | 42.5 ± 7.7 | 63 | 46.7 ± 2.5 | 20 | 41.4 ± 2.3 | 12 | 48.3 ± 10.5 | 0.042 |
Anger control | 71 | 57.4 ± 10.2 | 52 | 54.1 ± 2.3 | 12 | 62.9 ± 2.5 | 7 | 55 ± 10.5 | 0.064 |
Self-reported: | |||||||||
Attention problems | 46 | 51.5 ± 11.2 | 26 | 55.7 ± 3.4 | 12 | 52.8 ± 3.8 | 8 | 52.6 ± 7.3 | 0.157 |
Hyperactivity | 63 | 50.0 ± 11.8 | 26 | 54.2 ± 3.8 | 29 | 48.2 ± 3 | 8 | 52 ± 4.3 | 0.377 |
Deletion | UPD | p-Value | |||||
---|---|---|---|---|---|---|---|
n | Mean | SD | n | Mean | SD | ||
Age (years) | 209 | 14.4 | 12.1 | 118 | 11.6 | 10.1 | |
BMI percentile for age and gender | 209 | 85 | 34 | 116 | 83 | 36 | 0.24 |
Parent-reported: | |||||||
Attention problems | 134 | 56.8 | 9.7 | 78 | 57.1 | 8.7 | 0.82 |
Hyperactivity | 135 | 57.2 | 13.3 | 78 | 55.9 | 11.1 | 0.48 |
Aggression | 135 | 52.2 | 9.8 | 78 | 48.5 | 8.7 | 0.007 |
Leadership | 87 | 39.4 | 10.4 | 46 | 39.2 | 9.4 | 0.93 |
Anger control | 63 | 57.7 | 10.4 | 37 | 54.8 | 11.4 | 0.20 |
Teacher-reported: | |||||||
Attention problems | 92 | 55.7 | 10.7 | 61 | 58.4 | 8.3 | 0.09 |
Hyperactivity | 92 | 57.8 | 11.7 | 63 | 55.3 | 9.6 | 0.17 |
Aggression | 92 | 56.1 | 10.8 | 63 | 54.5 | 9.9 | 0.34 |
Leadership | 58 | 43.3 | 8.4 | 37 | 41.4 | 6.3 | 0.26 |
Anger control | 43 | 57.0 | 11.5 | 28 | 57.9 | 8.1 | 0.72 |
Self-reported: | |||||||
Attention problems | 32 | 52.2 | 12.2 | 14 | 49.9 | 8.6 | 0.53 |
Hyperactivity | 43 | 51.7 | 12.3 | 20 | 46.2 | 9.8 | 0.08 |
GH | No GH | p-Value | |||||
---|---|---|---|---|---|---|---|
n | Mean | SD | n | Mean | SD | ||
Age (years) | 219 | 9.3 | 7.7 | 101 | 21.8 | 13.4 | <0.00 |
BMI percentile for age and gender | 276 | 84 | 35 | 57 | 85 | 42 | 0.51 |
Parent-reported behaviors: | |||||||
Attention problems | 168 | 57.0 | 9.6 | 40 | 56.5 | 8.1 | 0.78 |
Hyperactivity | 168 | 55.8 | 12.4 | 41 | 60.3 | 13.1 | 0.04 |
Aggression | 168 | 50.1 | 9.0 | 41 | 53.5 | 11.5 | 0.04 |
Leadership | 94 | 38.7 | 9.4 | 37 | 41.2 | 11.5 | 0.21 |
Anger control | 87 | 56.0 | 10.5 | 10 | 62.9 | 13.1 | 0.06 |
Teacher-reported behaviors: | |||||||
Attention problems | 130 | 55.8 | 9.9 | 21 | 61.9 | 8.8 | 0.01 |
Hyperactivity | 132 | 56.2 | 11.1 | 21 | 60.6 | 10.4 | 0.09 |
Aggression | 132 | 54.5 | 10.3 | 21 | 61.5 | 9.9 | 0.004 |
Leadership | 75 | 42.5 | 7.9 | 19 | 43.4 | 6.8 | 0.66 |
Anger control | 67 | 57.3 | 10.3 | 3 | 60.3 | 13.5 | 0.62 |
Self-reported: | |||||||
Attention problems | 33 | 51.8 | 11.8 | 12 | 51.3 | 9.9 | 0.89 |
Hyperactivity | 50 | 50.3 | 12.4 | 12 | 49.3 | 9.5 | 0.78 |
GH | No GH | p-Value | |||||
---|---|---|---|---|---|---|---|
Age Groups | <11 Years | 11–18 Years | >18 Years | <11 Years | 11–18 Years | >18 Years | |
Parent-reported behaviors: | |||||||
Attention problems | 57 ± 10 | 60 ± 8.6 | 56 ± 14 | 57.5 ± 9 | 57.6 ± 7.6 | 49 ± 8.7 | 0.150 |
Hyperactivity | 53 ± 10 | 66.3 ± 14.5 | 68 ± 7.4 | 56 ± 10 | 61 ± 9 | 60.7 ± 9 | 0.231 |
Aggression | 48.9 ± 9 | 54.3 ± 11 | 52.2 ± 12.5 | 55.6 ± 16 | 53 ± 11 | 50 ± 5 | 0.638 |
Anger control | 56 ± 10.5 | 59 ± 7 | 64 ± 1.1 | 60 ± 14 | 62 ± 14 | 61.5 ± 9 | 0.697 |
Teacher-reported behaviors: | |||||||
Attention problems | 56.5 ± 9.1 | 57.4 ± 10.5 | 52 ± 12 | 56.7 ± 12 | 60 ± 11.3 | 50 ± 11.4 | 0.942 |
Hyperactivity | 54 ± 9 | 64 ± 13 | 70 ± 10 | 56.5 ± 11 | 58 ± 10 | 57 ± 10 | 0.133 |
Aggression | 51.6 ± 10 | 50 ± 12 | 53 ± 12 | 58.4 ± 13.5 | 59 ± 10 | 61.5 ± 7 | 0.033 |
Anger control | 57 ± 9 | 56 ± 12 | 57 ± 10 | 57 ± 11 | 60 ± 8 | 59 ± 7 | 0.715 |
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Mahmoud, R.; Swanson, H.D.; Butler, M.G.; Flodman, P.; Gold, J.-A.; Miller, J.L.; Roof, E.; Osann, K.; Dykens, E.; Driscoll, D.J.; et al. Molecular Classes and Growth Hormone Treatment Effects on Behavior and Emotion in Patients with Prader–Willi Syndrome. J. Clin. Med. 2022, 11, 2572. https://doi.org/10.3390/jcm11092572
Mahmoud R, Swanson HD, Butler MG, Flodman P, Gold J-A, Miller JL, Roof E, Osann K, Dykens E, Driscoll DJ, et al. Molecular Classes and Growth Hormone Treatment Effects on Behavior and Emotion in Patients with Prader–Willi Syndrome. Journal of Clinical Medicine. 2022; 11(9):2572. https://doi.org/10.3390/jcm11092572
Chicago/Turabian StyleMahmoud, Ranim, Heidi D. Swanson, Merlin G. Butler, Pamela Flodman, June-Anne Gold, Jennifer L. Miller, Elizabeth Roof, Kathryn Osann, Elisabeth Dykens, Daniel J. Driscoll, and et al. 2022. "Molecular Classes and Growth Hormone Treatment Effects on Behavior and Emotion in Patients with Prader–Willi Syndrome" Journal of Clinical Medicine 11, no. 9: 2572. https://doi.org/10.3390/jcm11092572