Functional independence of Taiwanese children with Prader–Willi syndrome

Chung Lin Lee, Hsiang Yu Lin, Li Ping Tsai, Huei Ching Chiu, Ru Yi Tu, You Hsin Huang, Yin Hsiu Chien, Ni Chung Lee, Dau Ming Niu, Mei Chyn Chao, Fuu Jen Tsai, Yen Yin Chou, Chih Kuang Chuang, Shuan Pei Lin

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)1309-1314
Number of pages6
JournalAmerican Journal of Medical Genetics, Part A
Volume176
Issue number6
DOIs
Publication statusPublished - 2018 Jun

All Science Journal Classification (ASJC) codes

  • Genetics
  • Genetics(clinical)

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  • Cite this

    Lee, C. L., Lin, H. Y., Tsai, L. P., Chiu, H. C., Tu, R. Y., Huang, Y. H., Chien, Y. H., Lee, N. C., Niu, D. M., Chao, M. C., Tsai, F. J., Chou, Y. Y., Chuang, C. K., & Lin, S. P. (2018). Functional independence of Taiwanese children with Prader–Willi syndrome. American Journal of Medical Genetics, Part A, 176(6), 1309-1314. https://doi.org/10.1002/ajmg.a.38705