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...
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Elsevier
2007-01-01
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Series: | Journal of the Formosan Medical Association |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S092966460960303X |
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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. |
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language | English |
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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 |
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