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Am J Med Genet A. 2018 Jun;176(6):1309-1314. doi: 10.1002/ajmg.a.38705. Epub 2018 Apr 25.

Functional independence of Taiwanese children with Prader-Willi syndrome.

Lee CL1, Lin HY1,2,3,4,5, Tsai LP6, Chiu HC1, Tu RY3, Huang YH1, Chien YH7, Lee NC7, Niu DM8,9, Chao MC10, Tsai FJ11, Chou YY12, Chuang CK3,13, Lin SP1,2,3,14.

Author information

1
Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.
2
Department of Medicine, Mackay Medical College, New Taipei City, Taiwan.
3
Division of Genetics and Metabolism, Department of Medical Research, MacKay Memorial Hospital, Taipei, Taiwan.
4
Mackay Junior College of Medicine, Nursing and Management, Taipei, Taiwan.
5
Department of Medical Research, China Medical University Hospital, China Medical University, Taichung, Taiwan.
6
Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan.
7
Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
8
Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan.
9
Department of Pediatrics, Taipei Veterans General Hospital, Taipei.
10
Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
11
Department of Medical Research, Genetics Center, China Medical University Hospital, Taichung, Taiwan.
12
Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
13
College of Medicine, Fu-Jen Catholic University, Taipei, Taiwan.
14
Department of Infant and Child Care, National Taipei University of Nursing and Health Sciences, Taipei, Taiwan.

Abstract

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.

KEYWORDS:

Prader-Willi syndrome; Taiwan; WeeFIM; independent living; pediatrics

PMID:
29696774
DOI:
10.1002/ajmg.a.38705
[Indexed for MEDLINE]

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