上海交通大学学报(医学版)

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肝豆状核变性患儿长期随访营养评价

鲁晓事1,2,焦先婷1,赵 蕾1,刘晓青1,陈 笋1,杨健萍1   

  1. 1.上海交通大学 医学院附属新华医院小儿心血管科, 上海 200092; 2.安徽省无为县人民医院儿内科, 无为 238300
  • 出版日期:2013-09-28 发布日期:2013-09-29
  • 通讯作者: 杨健萍, 电子信箱: yangjianping1965@aliyun.com。
  • 作者简介:鲁晓事(1972—), 男, 主治医师, 学士; 电子信箱: lu-xiaoshi@163.com。

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

摘要:

目的 比较采用不同治疗方案的肝豆状核变性患儿营养状况的长期随访结果。方法 对15例肝豆状核变性患儿进行随访,内容包括人体测量(身高、体质量)、血液生化指标测定(血常规、肝功能、血钙、血磷)和骨密度测定,根据治疗方案分为青霉胺组(n=9)和青霉胺+硫酸锌组(n=6),并进行统计学分析。结果 1例生长迟缓,1例中度营养不良。两组间年龄别身高百分位数和Z-评分(HAZ)、年龄别体质量百分位数和Z-评分(WAZ)以及体质量指数(BMI)百分位数和Z-评分(BMIZ)比较,差异均无统计学意义(P>0.05)。青霉胺组有3例轻度贫血,青霉胺+硫酸锌组1例轻度贫血。在青霉胺组中,除2例碱性磷酸酶正常外,其余均高于正常值;1例血钙升高;1例胫骨中段和桡骨远端的骨密度Z值分别为-3和-3.5,低于该年龄正常预期值范围。两组血红蛋白、总蛋白、白蛋白、血钙、血磷、碱性磷酸酶、胫骨中段和桡骨远端的骨密度Z值比较,差异均无统计学意义(P>0.05)。结论 长期驱铜和低铜饮食后,肝豆状核变性患儿远期营养状况基本正常,青霉胺治疗和青霉胺联合硫酸锌治疗对患儿的体格发育无明显影响。

关键词: 儿童, 肝豆状核变性, 营养评价

Abstract:

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