TY - JOUR
T1 - Beneficial Effects of Growth Hormone Treatment on Cognition in Children with Prader-Willi Syndrome
T2 - A Randomized Controlled Trial and Longitudinal Study
AU - Siemensma, Elbrich P. C.
AU - van Wijngaarden, Roderick F. A. Tummers-de Lind
AU - Festen, Dederieke A. M.
AU - Troeman, Zyrhea C. E.
AU - van Alfen-van der Velden, A. A. E. M. (Janielle)
AU - Otten, Barto J.
AU - Rotteveel, Joost
AU - Odink, Roelof J. H.
AU - Bindels-de Heus, G. C. B. (Karen)
AU - van Leeuwen, Mariette
AU - Haring, Danny A. J. P.
AU - Oostdijk, Wilma
AU - Bocca, Gianni
AU - Houdijk, E. C. A. Mieke
AU - van Trotsenburg, A. S. Paul
AU - Hoorweg-Nijman, J. J. Gera
AU - van Wieringen, Hester
AU - Vreuls, Rene C. F. M.
AU - Jira, Petr E.
AU - Schroor, Eelco J.
AU - van Pinxteren-Nagler, Evelyn
AU - Pilon, Jan Willem
AU - Lunshof, L. (Bert)
AU - Hokken-Koelega, Anita C. S.
PY - 2012/7
Y1 - 2012/7
N2 - Background: Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited.Methods: Fifty prepubertal children aged 3.5 to 14 yr were studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment. Cognitive functioning was measured biennially by short forms of the WPPSI-R or WISC-R, depending on age. Total IQ (TIQ) score was estimated based on two subtest scores.Results: During the randomized controlled trial, mean SD scores of all subtests and mean TIQ score remained similar compared to baseline in GH-treated children with PWS, whereas in untreated controls mean subtest SD scores and mean TIQ score decreased and became lower compared to baseline. This decline was significant for the Similarities (P = 0.04) and Vocabulary (P = 0.03) subtests. After 4 yr of GH treatment, mean SD scores on the Similarities and Block design subtests were significantly higher than at baseline (P = 0.01 and P = 0.03, respectively), and scores on Vocabulary and TIQ remained similar compared to baseline. At baseline, children with a maternal uniparental disomy had a significantly lower score on the Block design subtest (P = 0.01) but a larger increment on this subtest during 4 yr of GH treatment than children with a deletion. Lower baseline scores correlated significantly with higher increases in Similarities (P = 0.04) and Block design (P <0.0001) SD scores.Conclusions: Our study shows that GH treatment prevents deterioration of certain cognitive skills in children with PWS on the short term and significantly improves abstract reasoning and visuospatial skills during 4 yr of GH treatment. Furthermore, children with a greater deficit had more benefit from GH treatment. (J Clin Endocrinol Metab 97: 2307-2314, 2012)
AB - Background: Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited.Methods: Fifty prepubertal children aged 3.5 to 14 yr were studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment. Cognitive functioning was measured biennially by short forms of the WPPSI-R or WISC-R, depending on age. Total IQ (TIQ) score was estimated based on two subtest scores.Results: During the randomized controlled trial, mean SD scores of all subtests and mean TIQ score remained similar compared to baseline in GH-treated children with PWS, whereas in untreated controls mean subtest SD scores and mean TIQ score decreased and became lower compared to baseline. This decline was significant for the Similarities (P = 0.04) and Vocabulary (P = 0.03) subtests. After 4 yr of GH treatment, mean SD scores on the Similarities and Block design subtests were significantly higher than at baseline (P = 0.01 and P = 0.03, respectively), and scores on Vocabulary and TIQ remained similar compared to baseline. At baseline, children with a maternal uniparental disomy had a significantly lower score on the Block design subtest (P = 0.01) but a larger increment on this subtest during 4 yr of GH treatment than children with a deletion. Lower baseline scores correlated significantly with higher increases in Similarities (P = 0.04) and Block design (P <0.0001) SD scores.Conclusions: Our study shows that GH treatment prevents deterioration of certain cognitive skills in children with PWS on the short term and significantly improves abstract reasoning and visuospatial skills during 4 yr of GH treatment. Furthermore, children with a greater deficit had more benefit from GH treatment. (J Clin Endocrinol Metab 97: 2307-2314, 2012)
KW - FOR-GESTATIONAL-AGE
KW - PREPUBERTAL CHILDREN
KW - BREATHING DISORDERS
KW - FACTOR-I
KW - GH
KW - POPULATION
KW - EXPRESSION
KW - VELOCITY
KW - BEHAVIOR
KW - THERAPY
U2 - 10.1210/jc.2012-1182
DO - 10.1210/jc.2012-1182
M3 - Article
VL - 97
SP - 2307
EP - 2314
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
SN - 0021-972X
IS - 7
ER -