Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome

Prader-Willi syndrome (PWS) is a rare, multifaceted genetic disorder resulting from the absence of normally active paternally expressed genes from the 15q11-q13 chromosome region. Due to a lack of anthropometric and intellectual data in Taiwan, we attempted these evaluations. Twenty patients (14 mal...

Full description

Bibliographic Details
Main Authors: San-Ging Shu, Shu Chien, Yen-Ching Wu, Pei-Ling Tsai, Joung-Kun Yih
Format: Article
Language:English
Published: Elsevier 2007-01-01
Series:Journal of the Formosan Medical Association
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S092966460960303X
_version_ 1818430218031857664
author San-Ging Shu
Shu Chien
Yen-Ching Wu
Pei-Ling Tsai
Joung-Kun Yih
author_facet San-Ging Shu
Shu Chien
Yen-Ching Wu
Pei-Ling Tsai
Joung-Kun Yih
author_sort San-Ging Shu
collection DOAJ
description Prader-Willi syndrome (PWS) is a rare, multifaceted genetic disorder resulting from the absence of normally active paternally expressed genes from the 15q11-q13 chromosome region. Due to a lack of anthropometric and intellectual data in Taiwan, we attempted these evaluations. Twenty patients (14 males/6 females) aged 7–23 years with molecularly confirmed PWS were enrolled with parental consent. Their mean height standard deviation score (SDS) was −1.26 ± 1.89 (from −4.3 to +2.16); mean weight SDS was +1.77 ± 2.00 (from −0.44 to +5.89); mean body mass index SDS was +3.84 ± 10.54 (from −0.08 to +10.48); and mean body fat tissue SDS was 39.4 ± 10.54% (14.7-57.8%) by an InBody 3.0 analyzer. All were hypogonadal. Nine of them had once been given growth hormone therapy, and were taller and slimmer than the rest. Their in telligence tests showed full intelligence quotient = 52.0 ± 7.6; verbal intelligence quotient = 55.9 ± 8.77; performance intelligence quotient = 53.2 ± 9.0. Chronic health status revealed that diabetes was prevalent among the older population. Their IQ was in the range of those with moderate retardation.
first_indexed 2024-12-14T15:29:55Z
format Article
id doaj.art-7c25c5dc25074f4d99b49228bcdb8c5d
institution Directory Open Access Journal
issn 0929-6646
language English
last_indexed 2024-12-14T15:29:55Z
publishDate 2007-01-01
publisher Elsevier
record_format Article
series Journal of the Formosan Medical Association
spelling doaj.art-7c25c5dc25074f4d99b49228bcdb8c5d2022-12-21T22:55:55ZengElsevierJournal of the Formosan Medical Association0929-66462007-01-01106650951210.1016/S0929-6646(09)60303-XAnthropometric and Intellectual Evaluation of Individuals with Prader-Willi SyndromeSan-Ging Shu0Shu Chien1Yen-Ching Wu2Pei-Ling Tsai3Joung-Kun Yih4Department of PediatricsGenetic CenterDepartment of Psychiatry, Taichung Veterans General HospitalDepartment of Psychiatry, Taichung Veterans General HospitalDepartment of Psychiatry, Taichung Veterans General HospitalPrader-Willi syndrome (PWS) is a rare, multifaceted genetic disorder resulting from the absence of normally active paternally expressed genes from the 15q11-q13 chromosome region. Due to a lack of anthropometric and intellectual data in Taiwan, we attempted these evaluations. Twenty patients (14 males/6 females) aged 7–23 years with molecularly confirmed PWS were enrolled with parental consent. Their mean height standard deviation score (SDS) was −1.26 ± 1.89 (from −4.3 to +2.16); mean weight SDS was +1.77 ± 2.00 (from −0.44 to +5.89); mean body mass index SDS was +3.84 ± 10.54 (from −0.08 to +10.48); and mean body fat tissue SDS was 39.4 ± 10.54% (14.7-57.8%) by an InBody 3.0 analyzer. All were hypogonadal. Nine of them had once been given growth hormone therapy, and were taller and slimmer than the rest. Their in telligence tests showed full intelligence quotient = 52.0 ± 7.6; verbal intelligence quotient = 55.9 ± 8.77; performance intelligence quotient = 53.2 ± 9.0. Chronic health status revealed that diabetes was prevalent among the older population. Their IQ was in the range of those with moderate retardation.http://www.sciencedirect.com/science/article/pii/S092966460960303Xanthropometricbody compositionintellectualPrader-Willi syndrome
spellingShingle San-Ging Shu
Shu Chien
Yen-Ching Wu
Pei-Ling Tsai
Joung-Kun Yih
Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome
Journal of the Formosan Medical Association
anthropometric
body composition
intellectual
Prader-Willi syndrome
title Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome
title_full Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome
title_fullStr Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome
title_full_unstemmed Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome
title_short Anthropometric and Intellectual Evaluation of Individuals with Prader-Willi Syndrome
title_sort anthropometric and intellectual evaluation of individuals with prader willi syndrome
topic anthropometric
body composition
intellectual
Prader-Willi syndrome
url http://www.sciencedirect.com/science/article/pii/S092966460960303X
work_keys_str_mv AT sangingshu anthropometricandintellectualevaluationofindividualswithpraderwillisyndrome
AT shuchien anthropometricandintellectualevaluationofindividualswithpraderwillisyndrome
AT yenchingwu anthropometricandintellectualevaluationofindividualswithpraderwillisyndrome
AT peilingtsai anthropometricandintellectualevaluationofindividualswithpraderwillisyndrome
AT joungkunyih anthropometricandintellectualevaluationofindividualswithpraderwillisyndrome