Are Angelman and Prader-Willi syndromes more similar than we thought? Food-related behavior problems in Angelman, Cornelia de Lange, Fragile X, Prader-Willi and 1p36 deletion syndromes

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@article{22603207f6924e3cadf3f476aa284b49,
title = "Are Angelman and Prader-Willi syndromes more similar than we thought?: Food-related behavior problems in Angelman, Cornelia de Lange, Fragile X, Prader-Willi and 1p36 deletion syndromes",
abstract = "Food-related behavior problems are well documented in Prader-Willi syndrome (PWS), with impaired satiety, preoccupation with food and negative food-related behaviors (such as taking and storing food) frequently reported as part of the behavioral phenotype of older children and adults. Food-related behavior problems in other genetic neurodevelopmental syndromes remain less well studied, including those seen in Angelman Syndrome (AS), the 'sister imprinted disorder' of PWS. Food-related behavior problems were assessed in 152 participants each with one of five genetic neurodevelopmental syndromes - PWS, AS, 1p36 deletion, Cornelia de Lange, and fragile X. Predictably, levels of food-related behavior problems reported in participants with PWS significantly exceeded those of at least one other groups in most areas (impaired satiety; preoccupation with food; taking and storing food; composite negative behavior). However, in some areas people with AS were reported to display food-related problems at least as severe as those with PWS, with the AS group reported to display significantly more food-related behavior problems than at least one comparison group on measures of taking and storing food, composite negative behaviors, impaired satiety and preoccupation with food. Over 50% of participants in the AS group scored above the median point of the distribution of PWS scores on a measure of taking and storing food. These findings indicate further investigation of eating problems in AS are warranted and have implications for current theoretical interpretations of the behavioral differences between AS and PWS. {\textcopyright} 2015 Wiley Periodicals, Inc.",
keywords = "1p36 deletion syndrome, Angelman syndrome, Behavior, Cornelia de Lange syndrome, Eating inedible, Eating, food, Fragile X syndrome, Prader-Willi syndrome, age distribution, Article, child, chromosome 1p, chromosome analysis, chromosome deletion, clinical feature, compulsion, controlled study, de Lange syndrome, differential diagnosis, disease association, disease severity, feeding behavior, feeding disorder, female, follow up, Food Related Problems Questionnaire, fragile X syndrome, happy puppet syndrome, human, intellectual impairment, longitudinal study, major clinical study, male, negative syndrome, Prader Willi syndrome, psychologic assessment, psychophysiology, school child, scoring system, self help, Wessex Scale",
author = "Alice Welham and Johnny Lau and Joanna Moss and Jenny Cullen and Suzanne Higgs and Gemma Warren and Lucy Wilde and Abby Marr and Faye Cook and Christopher Oliver",
note = "{\textcopyright} 2015 Wiley Periodicals, Inc.",
year = "2015",
month = mar
doi = "10.1002/ajmg.a.36923",
language = "English",
volume = "167",
pages = "572--578",
journal = "American Journal of Medical Genetics. Part A",
issn = "1552-4825",
publisher = "Wiley",
number = "3",

}

RIS

TY - JOUR

T1 - Are Angelman and Prader-Willi syndromes more similar than we thought?

T2 - Food-related behavior problems in Angelman, Cornelia de Lange, Fragile X, Prader-Willi and 1p36 deletion syndromes

AU - Welham, Alice

AU - Lau, Johnny

AU - Moss, Joanna

AU - Cullen, Jenny

AU - Higgs, Suzanne

AU - Warren, Gemma

AU - Wilde, Lucy

AU - Marr, Abby

AU - Cook, Faye

AU - Oliver, Christopher

N1 - © 2015 Wiley Periodicals, Inc.

PY - 2015/3

Y1 - 2015/3

N2 - Food-related behavior problems are well documented in Prader-Willi syndrome (PWS), with impaired satiety, preoccupation with food and negative food-related behaviors (such as taking and storing food) frequently reported as part of the behavioral phenotype of older children and adults. Food-related behavior problems in other genetic neurodevelopmental syndromes remain less well studied, including those seen in Angelman Syndrome (AS), the 'sister imprinted disorder' of PWS. Food-related behavior problems were assessed in 152 participants each with one of five genetic neurodevelopmental syndromes - PWS, AS, 1p36 deletion, Cornelia de Lange, and fragile X. Predictably, levels of food-related behavior problems reported in participants with PWS significantly exceeded those of at least one other groups in most areas (impaired satiety; preoccupation with food; taking and storing food; composite negative behavior). However, in some areas people with AS were reported to display food-related problems at least as severe as those with PWS, with the AS group reported to display significantly more food-related behavior problems than at least one comparison group on measures of taking and storing food, composite negative behaviors, impaired satiety and preoccupation with food. Over 50% of participants in the AS group scored above the median point of the distribution of PWS scores on a measure of taking and storing food. These findings indicate further investigation of eating problems in AS are warranted and have implications for current theoretical interpretations of the behavioral differences between AS and PWS. © 2015 Wiley Periodicals, Inc.

AB - Food-related behavior problems are well documented in Prader-Willi syndrome (PWS), with impaired satiety, preoccupation with food and negative food-related behaviors (such as taking and storing food) frequently reported as part of the behavioral phenotype of older children and adults. Food-related behavior problems in other genetic neurodevelopmental syndromes remain less well studied, including those seen in Angelman Syndrome (AS), the 'sister imprinted disorder' of PWS. Food-related behavior problems were assessed in 152 participants each with one of five genetic neurodevelopmental syndromes - PWS, AS, 1p36 deletion, Cornelia de Lange, and fragile X. Predictably, levels of food-related behavior problems reported in participants with PWS significantly exceeded those of at least one other groups in most areas (impaired satiety; preoccupation with food; taking and storing food; composite negative behavior). However, in some areas people with AS were reported to display food-related problems at least as severe as those with PWS, with the AS group reported to display significantly more food-related behavior problems than at least one comparison group on measures of taking and storing food, composite negative behaviors, impaired satiety and preoccupation with food. Over 50% of participants in the AS group scored above the median point of the distribution of PWS scores on a measure of taking and storing food. These findings indicate further investigation of eating problems in AS are warranted and have implications for current theoretical interpretations of the behavioral differences between AS and PWS. © 2015 Wiley Periodicals, Inc.

KW - 1p36 deletion syndrome

KW - Angelman syndrome

KW - Behavior

KW - Cornelia de Lange syndrome

KW - Eating inedible

KW - Eating, food

KW - Fragile X syndrome

KW - Prader-Willi syndrome

KW - age distribution

KW - Article

KW - child

KW - chromosome 1p

KW - chromosome analysis

KW - chromosome deletion

KW - clinical feature

KW - compulsion

KW - controlled study

KW - de Lange syndrome

KW - differential diagnosis

KW - disease association

KW - disease severity

KW - feeding behavior

KW - feeding disorder

KW - female

KW - follow up

KW - Food Related Problems Questionnaire

KW - fragile X syndrome

KW - happy puppet syndrome

KW - human

KW - intellectual impairment

KW - longitudinal study

KW - major clinical study

KW - male

KW - negative syndrome

KW - Prader Willi syndrome

KW - psychologic assessment

KW - psychophysiology

KW - school child

KW - scoring system

KW - self help

KW - Wessex Scale

U2 - 10.1002/ajmg.a.36923

DO - 10.1002/ajmg.a.36923

M3 - Article

C2 - 25691410

VL - 167

SP - 572

EP - 578

JO - American Journal of Medical Genetics. Part A

JF - American Journal of Medical Genetics. Part A

SN - 1552-4825

IS - 3

ER -