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Long-term nutrition assessment in children with Wilson's disease

LU Xiao-shi1,2, JIAO Xian-ting1, ZHAO Lei1, LIU Xiao-qing1, CHEN Sun1, YANG Jian-ping1   

  1. 1.Department of Pediatric Cardiology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200092, China; 2.Department of Pediatric Internal Medicine, Wuwei People's Hospital, Wuwei 238300, China
  • Online:2013-09-28 Published:2013-09-29


Objective To investigate the long-term nutrition status of children with Wilson's disease treated by different methods. Methods Fifteen children with Wilson's disease were followed up for anthropometric measurements (height and body weight), blood biochemical parameters detection (blood routine, liver function, blood calcium and blood phosphorus) and bone density determination. Children were divided into penicillamine group and penicillamine+zinc sulfate group based on the therapies, and comparisons were made between two groups. Results Among the 15 children, 1 had growth retardation, and the other 1 had midrange malnutrition. There was no significant difference in weight for age percentile and Z-score (WAZ), height for age percentile and Z-score (HAZ), and body mass index (BMI) percentile and Z-score (BMIZ) between two groups (P>0.05). There were 3 children with mild anaemia in penicillamine group, and there was 1 child with mild anaemia in penicillamine+zinc sulfate group. The values of alkaline phosphatase in children in penicillamine group were higher than the normal value except for two children. The blood calcium was elevated in 1 child in penicillamine group. The Z-scores of bone density of middle tibia and distal radius were -3 and -3.5 respectively in 1 child in penicillamine group, which were lower than the normal values. There was no significant difference in hemoglobin, total protein, albumin, blood calcium, blood phosphorus, alkaline phosphatase and ratio of bone density of middle tibia to that of distal radius between two groups (P>0.05). Conclusion Children with Wilson's disease have normal nutrition status after long-term copper displacement and low-copper diet. Penicillamine treatment and penicillamine combined with zinc sulfate treatment have no significant effect on children's physical development.

Key words: children, Wilson's disease, nutrition assessment