Cognitive function during 3 years of growth hormone in previously growth hormone-treated young adults with Prader-Willi syndrome

Demi J. Trueba-Timmermans*, Lionne N. Grootjen, Alicia F. Juriaans, Eva F. Mahabier, Gerthe F. Kerkhof, Edmond H.H.M. Rings, Anita C.S. Hokken-Koelega

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Context: Most patients with Prader-Willi syndrome (PWS) have mild to moderate cognitive impairment. Growth hormone (GH) treatment has positive short-and long-Term effects on cognition in children with PWS. Few studies, however, have investigated the effects of GH on cognitive functioning in adults with PWS. Objective: To investigate the effects of 3 years of GH treatment on cognitive functioning and behavior in young adults with PWS who were treated with GH during childhood. Design: Open-label, prospective study. Setting: Dutch PWS Reference Center. Methods: Patients were treated with 0.33a mg GH/m²/day (∼0.012a mg/kg/day; 33% of childhood dose). Cognitive functioning was measured by Wechsler Adult Intelligence (WAIS) tests. Behavior was studied by a developmental behavior checklist-parents/caregivers (DBC-P). Results: Forty-six young adults with PWS with a median age of 19 (IQR 17-21) years were investigated. Estimated mean (95% CI) total, verbal, and performance IQ remained stable during 3 years of GH-Treatment. Total IQ being 66 (63-69) at the start and 67 (64-71) after 3 years (P =. 30); Verbal IQ being 65 (62-68) and 66 (62-70), respectively (P =. 31) and performance IQ being 67 (63-70) and 67 (63-72) resp. (P =. 42). Estimated mean Total DBC score did not significantly change during 3 years of GH-Treatment, being 36.3 at start and 36.5 after 3 years (P =. 94) (P50). Conclusions: Three years of GH-Treatment in young adults with PWS with 33% of the pediatric dose, maintains total, verbal, and performance IQ. The emotional and behavioral disturbances remained stable and were similar compared to peers with other intellectual disabilities.

Original languageEnglish
Pages (from-to)132-139
Number of pages8
JournalEuropean Journal of Endocrinology
Issue number1
Publication statusPublished - Jul 2023

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© 2023 The Author(s). Published by Oxford University Press on behalf of European Society of Endocrinology.


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