婴幼儿时期Prader-Willi syndrome喂养困难的研究进展

doi:10.11852/zgetbjzz2018-1533

中国儿童保健杂志 ›› 2019, Vol. 27 ›› Issue (3): 273-276.DOI: 10.11852/zgetbjzz2018-1533

婴幼儿时期Prader-Willi syndrome喂养困难的研究进展

廉文君¹,段培¹,梁瑞星¹,牛冬鹤¹,陆相朋²,郑宏²

1 河南中医药大学,河南郑州 450008;
2 河南中医药大学第一附属医院,河南郑州 450000

收稿日期:2018-10-26 修回日期:2018-12-07 发布日期:2019-03-20 出版日期:2019-03-20
通讯作者: 郑宏,E-mail:drzhenghs@126.com
作者简介:廉文君(1992-),女,河南人,硕士研究生,主要研究方向儿科神经系统疾病。
基金资助:
国家重点研发计划(2017YFC1001704),区域金磊儿科内分泌中青年医师成长科研基金(PEGRF-CR-20171007)

Research advance on feeding difficulties of Prader-Willi syndrome in infants

LIAN Wen-jun¹,DUAN Pei¹,LIANG Rui-xing¹,NIU Dong-he¹,LU Xiang-peng²,ZHENG Hong²

1 Henan University of Traditional Chinese Medicine,Zhengzhou,Henan 450008,China;
2 the First Affiliated Hospital of Henan University of Chinese Medicine,Zhengzhou,Henan 450000,China

Received:2018-10-26 Revised:2018-12-07 Online:2019-03-20 Published:2019-03-20
Contact: ZHENG Hong,E-mail:drzhenghs@126.com

摘要/Abstract

摘要： Prader-Willi综合征(PWS)是由于印记基因缺陷所导致的遗传病,该病伴有中枢性肌张力低下,喂养困难,进行性肥胖等问题。喂养困难所导致的营养问题是PWS患儿婴幼儿时期死亡的原因之一。本文对PWS患儿婴幼儿时期产生喂养困难的原因及改善喂养的方法进行了综述,提示PWS患儿染色体片段缺失所造成的功能异常和患儿体内饥饿激素水平的变化是导致喂养困难的主要原因,通过鼻内输注催产素、皮下注射生长激素结合口运动练习,鼻饲胃管等喂养方法可以改善婴幼儿时期PWS患儿的喂养状况,提高营养状况。

关键词: Prader-Willi syndrome, 喂养困难, 肌张力低下

Abstract: Objective Prader-Willi syndrome(PWS) is a genetic disease caused by imprinting genetic defects,which is accompanied by problems such as central hypotonia,feeding difficulties and progressive obesity. Nutritional problems caused by feeding difficulties are one of the causes of infant mortality in PWS children. This review summarizes the reasons of feeding difficulties in infants and young children with PWS and the methods of improving feeding. All the evidence that the dysfunction of chromosomal deficiencies in PWS children and the change of the level of hunger hormones in children are the main causes of feeding difficulty. Intranasal infusion of oxytocin,subcutaneous injection of growth hormone,oral exercise and nasogastric feeding tube feeding method can help infants and young children with PWS feeding difficulties,thereby improving nutritional status.

Key words: Prader-Willi syndrome, feeding difficulties, hypotonia

中图分类号:

R179

廉文君,段培,梁瑞星,牛冬鹤,陆相朋,郑宏. 婴幼儿时期Prader-Willi syndrome喂养困难的研究进展[J]. 中国儿童保健杂志, 2019, 27(3): 273-276.

LIAN Wen-jun,DUAN Pei,LIANG Rui-xing,NIU Dong-he,LU Xiang-peng,ZHENG Hong. Research advance on feeding difficulties of Prader-Willi syndrome in infants[J]. journal1, 2019, 27(3): 273-276.

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婴幼儿时期Prader-Willi syndrome喂养困难的研究进展

Research advance on feeding difficulties of Prader-Willi syndrome in infants

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