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Functional independence of Taiwanese children with Prader–Willi syndrome
Author(s) -
Lee ChungLin,
Lin HsiangYu,
Tsai LiPing,
Chiu HueiChing,
Tu RuYi,
Huang YouHsin,
Chien YinHsiu,
Lee NiChung,
Niu DauMing,
Chao MeiChyn,
Tsai FuuJen,
Chou YenYin,
Chuang ChihKuang,
Lin ShuanPei
Publication year - 2018
Publication title -
american journal of medical genetics part a
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.064
H-Index - 112
eISSN - 1552-4833
pISSN - 1552-4825
DOI - 10.1002/ajmg.a.38705
Subject(s) - functional independence measure , medicine , pediatrics , short stature , cognition , physical therapy , activities of daily living , psychiatry
Prader–Willi syndrome (PWS) is a genetic disorder with obesity, developmental delay, short stature, and behavioral abnormalities. The study aimed to assess the functional independence in children with PWS. The Functional Independence Measure for Children (WeeFIM) was used to evaluate 81 children with PWS (44 boys and 37 girls) with a median age of 11 years 1 month (range 2 years 8 months to 20 years 2 months) were recruited between January 2013 and December 2016. The mean total WeeFIM score was 103.8 (maximum 126). Sixty‐five patients (80%) had deletion type PWS, 16 (20.0%) had nondeletion type. The scores were 103.6 ± 18.5 for deletion and 104.8 ± 18.3 for nondeletion type ( p = .405), 104.8 ± 19.3 in boys and 102.6 ± 17.3 in girls ( p = .293). The mean self‐care, mobility, and cognition scores were 47 (maximum 56), 33 (maximum 35), and 24 (maximum 35), respectively. All total scores and 18 subscores in the three functional domains were positively correlated with age ( p < .05). Most children required assistance in problem‐solving, comprehension, and expression. The WeeFIM identified the strengths and limitations of children with PWS and confirmed that support and supervision were needed in cognitive and self‐care tasks.