BackgroundPeople with Prader-Willi syndrome (PWS) typically have mild to moderate intellectual deficits, compulsivity, hyperphagia, obesity, and growth hormone deficiencies. Growth hormone treatment (GHT) in PWS has well-established salutatory effects on linear growth and body composition, yet cognitive benefits of GHT, seen in other patient groups, have not been well studied in PWS. MethodsStudy 1 included 96 children and youth with PWS aged 4-21years who naturalistically varied in their exposures to GHT. Controlling for socioeconomic status, analyses compared cognitive and adaptive behavior test scores across age-matched treatment naive versus growth hormone treated children. Study II assessed if age of treatment initiation or treatment duration was associated with subsequent cognition or adaptive behavior in 127, 4- to 21-year olds with PWS. Study III longitudinally examined cognitive and adaptive behavior in 168 participants who were either consistently on versus off GHT for up to 4-5years. ResultsCompared to the treatment naive group, children receiving GHT had significantly higher Verbal and Composite IQs, and adaptive communication and daily living skills. Children who began treatment before 12months of age had higher Nonverbal and Composite IQs than children who began treatment between 1 and 5years of age. Longitudinally, the groups differed in their intercepts, but not slopes, with each group showing stable IQ and adaptive behavior scores over time. ConclusionsCognitive and adaptive advantages should be considered an ancillary benefit and additional justification for GHT in people with PWS. Future efforts need to target apparent socioeconomic inequities in accessing GHT in the PWS population.
机构:
Univ Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Hop Univ Necker Enfants Malades, Ctr Reference Pathol Gynecol Rares & Malad Endocr, Pediatr Endocrinol Gynecol & Diabetol, Paris, FranceUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Buff Passone, Caroline de Gouveia
Franco, Ruth Rocha
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Univ Sao Paulo, Sao Paulo, BrazilUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Franco, Ruth Rocha
Ito, Simone Sakura
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Univ Sao Paulo, Sao Paulo, BrazilUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Ito, Simone Sakura
Trindade, Evelinda
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Univ Sao Paulo, Sao Paulo, BrazilUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Trindade, Evelinda
Polak, Michel
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Hop Univ Necker Enfants Malades, Ctr Reference Pathol Gynecol Rares & Malad Endocr, Pediatr Endocrinol Gynecol & Diabetol, Paris, FranceUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Polak, Michel
Damiani, Durval
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Univ Sao Paulo, Sao Paulo, BrazilUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
Damiani, Durval
Bernardo, Wanderley Marques
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Univ Sao Paulo, Sao Paulo, BrazilUniv Sao Paulo, Pediat Endocrinol Unit, Sao Paulo, Brazil
机构:
Univ Zagreb, Sch Dent Med, Vinogradska C 29, HR-10000 Zagreb, Croatia
Sestre Milosrdnice Univ Hosp Ctr, Dept Pediat, Vinogradska C 29, HR-10000 Zagreb, CroatiaUniv Zagreb, Sch Dent Med, Vinogradska C 29, HR-10000 Zagreb, Croatia
Stipancic, Gordana
Sepec, Marija Pozgaj
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Sestre Milosrdnice Univ Hosp Ctr, Dept Pediat, Vinogradska C 29, HR-10000 Zagreb, CroatiaUniv Zagreb, Sch Dent Med, Vinogradska C 29, HR-10000 Zagreb, Croatia
Sepec, Marija Pozgaj
Sabolic, Lavinia La Grasta
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Sestre Milosrdnice Univ Hosp Ctr, Dept Pediat, Vinogradska C 29, HR-10000 Zagreb, CroatiaUniv Zagreb, Sch Dent Med, Vinogradska C 29, HR-10000 Zagreb, Croatia