Cognitive and Adaptive Effects of Early Growth Hormone Treatment in Prader-Willi Syndrome Patients: A Cohort Study

doi:10.3390/jcm11061592

. 2022 Mar 14;11(6):1592.

doi: 10.3390/jcm11061592.

Cognitive and Adaptive Effects of Early Growth Hormone Treatment in Prader-Willi Syndrome Patients: A Cohort Study

Aitana Ayet-Roger^{1

2}, Lorena Joga-Elvira^{2

3}, Assumpta Caixàs^{3

4

5}, Raquel Corripio^{3

4

6}

Affiliations

¹ Clinical and Health Psychology Department, Universitat Autònoma de Barcelona, 08193 Bellaterra, Spain.
² Pediatric Neuropediatric Department, Parc Tauli Sabadell Hospital Universitari, 08208 Sabadell, Spain.
³ Institut d'Investigació i Innovació Parc Taulí (I3PT), Centre CERCA, 08208 Sabadell, Spain.
⁴ Department of Medicine, Universitat Autònoma de Barcelona, 08208 Sabadell, Spain.
⁵ Endocrinology and Nutrition Department, Hospital Universitari Parc Taulí, 08208 Sabadell, Spain.
⁶ Pediatric Endocrine Department, Parc Tauli Sabadell Hospital Universitari, 08208 Sabadell, Spain.

PMID: 35329918
PMCID: PMC8955812
DOI: 10.3390/jcm11061592

Cognitive and Adaptive Effects of Early Growth Hormone Treatment in Prader-Willi Syndrome Patients: A Cohort Study

Aitana Ayet-Roger et al. J Clin Med. 2022.

. 2022 Mar 14;11(6):1592.

doi: 10.3390/jcm11061592.

Authors

Aitana Ayet-Roger^{1

2}, Lorena Joga-Elvira^{2

3}, Assumpta Caixàs^{3

4

5}, Raquel Corripio^{3

4

6}

Affiliations

¹ Clinical and Health Psychology Department, Universitat Autònoma de Barcelona, 08193 Bellaterra, Spain.
² Pediatric Neuropediatric Department, Parc Tauli Sabadell Hospital Universitari, 08208 Sabadell, Spain.
³ Institut d'Investigació i Innovació Parc Taulí (I3PT), Centre CERCA, 08208 Sabadell, Spain.
⁴ Department of Medicine, Universitat Autònoma de Barcelona, 08208 Sabadell, Spain.
⁵ Endocrinology and Nutrition Department, Hospital Universitari Parc Taulí, 08208 Sabadell, Spain.
⁶ Pediatric Endocrine Department, Parc Tauli Sabadell Hospital Universitari, 08208 Sabadell, Spain.

PMID: 35329918
PMCID: PMC8955812
DOI: 10.3390/jcm11061592

Abstract

Background: Prader-Willi Syndrome (PWS) is a genetically based neurodevelopmental disease characterized by obesity, hyperphagia, and mild to moderate intellectual disability. Treatment with growth hormone (GH) could provide cognitive benefits. The objective of the present study was to compare the cognitive and adaptive performance of 31 patients with genetically confirmed PWS grouped in two cohorts, one treated with GH before 2 years old (Group 1) and the other receiving the treatment later (Group 2).

Method: We compared two variables necessary to diagnose intellectual disability: intellectual performance, using the Weschler scales, and adaptive behavior, using the DABS scale. The scores were analyzed by means of non-parametric statistical tests.

Results: Group 1 (n = 10) obtained higher and statistically significant scores in Total Intelligence Quotient (TIQ), General Ability Index (GAI), and General Adaptive Behavior (GAB), implying better cognitive and adaptive performance compared to Group 2.

Conclusions: Treatment with GH should be administered in the early stage of development (before 2 years old) to obtain greater benefits at the cognitive and adaptive levels.

Keywords: Prader–Willi Syndrome; adaptive behavior; cognition; growth hormone treatment.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

**Figure 1**
Boxplot of comparison of differences in processing speed for the two groups. (°) and (*): outlier values.

See this image and copyright information in PMC

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References

1. Gabau E., Aguilera C., Baena N., Ruiz A., Guitart M. Enfermedades por alteración de la impronta genética. Síndrome Prader-Willi y de Angelman. Pediatr. Integral. 2019;XXIII:249–257.
1. Rice L., Einfeld S.L. Cognitive and behavioural aspects of Prader–Willi syndrome. Curr. Opin. Psychiatry. 2015;28:102–106. doi: 10.1097/YCO.0000000000000135. - DOI - PubMed
1. Dimitropoulos A., Ferranti A., Lemler M. Expressive and receptive language in Prader–Willi syndrome: Report on genetic subtype differences. J. Commun. Disord. 2013;46:193–201. doi: 10.1016/j.jcomdis.2012.12.001. - DOI - PubMed
1. Famelart N., Diene G., Çabal-Berthoumieu S., Glattard M., Molinas C., Guidetti M., Tauber M. Equivocal expression of emotions in children with Prader-Willi syndrome: What are the consequences for emotional abilities and social adjustment? Orphanet J. Rare Dis. 2020;15:55. doi: 10.1186/s13023-020-1333-9. - DOI - PMC - PubMed
1. Dykens E.M., Roof E., Hunt-Hawkins H., Daniell C., Jurgensmeyer S. Profiles and trajectories of impaired social cognition in people with Prader-Willi syndrome. PLoS ONE. 2019;14:e0223162. doi: 10.1371/journal.pone.0223162. - DOI - PMC - PubMed

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[1] Gabau E., Aguilera C., Baena N., Ruiz A., Guitart M. Enfermedades por alteración de la impronta genética. Síndrome Prader-Willi y de Angelman. Pediatr. Integral. 2019;XXIII:249–257.

[2] Gabau E., Aguilera C., Baena N., Ruiz A., Guitart M. Enfermedades por alteración de la impronta genética. Síndrome Prader-Willi y de Angelman. Pediatr. Integral. 2019;XXIII:249–257.

[3] Rice L., Einfeld S.L. Cognitive and behavioural aspects of Prader–Willi syndrome. Curr. Opin. Psychiatry. 2015;28:102–106. doi: 10.1097/YCO.0000000000000135. - DOI - PubMed

[4] Rice L., Einfeld S.L. Cognitive and behavioural aspects of Prader–Willi syndrome. Curr. Opin. Psychiatry. 2015;28:102–106. doi: 10.1097/YCO.0000000000000135. - DOI - PubMed

[5] Dimitropoulos A., Ferranti A., Lemler M. Expressive and receptive language in Prader–Willi syndrome: Report on genetic subtype differences. J. Commun. Disord. 2013;46:193–201. doi: 10.1016/j.jcomdis.2012.12.001. - DOI - PubMed

[6] Dimitropoulos A., Ferranti A., Lemler M. Expressive and receptive language in Prader–Willi syndrome: Report on genetic subtype differences. J. Commun. Disord. 2013;46:193–201. doi: 10.1016/j.jcomdis.2012.12.001. - DOI - PubMed

[7] Famelart N., Diene G., Çabal-Berthoumieu S., Glattard M., Molinas C., Guidetti M., Tauber M. Equivocal expression of emotions in children with Prader-Willi syndrome: What are the consequences for emotional abilities and social adjustment? Orphanet J. Rare Dis. 2020;15:55. doi: 10.1186/s13023-020-1333-9. - DOI - PMC - PubMed

[8] Famelart N., Diene G., Çabal-Berthoumieu S., Glattard M., Molinas C., Guidetti M., Tauber M. Equivocal expression of emotions in children with Prader-Willi syndrome: What are the consequences for emotional abilities and social adjustment? Orphanet J. Rare Dis. 2020;15:55. doi: 10.1186/s13023-020-1333-9. - DOI - PMC - PubMed

[9] Dykens E.M., Roof E., Hunt-Hawkins H., Daniell C., Jurgensmeyer S. Profiles and trajectories of impaired social cognition in people with Prader-Willi syndrome. PLoS ONE. 2019;14:e0223162. doi: 10.1371/journal.pone.0223162. - DOI - PMC - PubMed

[10] Dykens E.M., Roof E., Hunt-Hawkins H., Daniell C., Jurgensmeyer S. Profiles and trajectories of impaired social cognition in people with Prader-Willi syndrome. PLoS ONE. 2019;14:e0223162. doi: 10.1371/journal.pone.0223162. - DOI - PMC - PubMed

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Cognitive and Adaptive Effects of Early Growth Hormone Treatment in Prader-Willi Syndrome Patients: A Cohort Study

Affiliations

Cognitive and Adaptive Effects of Early Growth Hormone Treatment in Prader-Willi Syndrome Patients: A Cohort Study

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Conflict of interest statement

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