CAJ | 학술논문

目的探讨中国人先天性裂手/裂足畸形(SHFM)的流行病学特征和临床特点.方法通过中国出生缺陷监测网络获得SHFM研究资料,把病例分为单发(仅有SHFM或者伴发其他肢体畸形)和综合征(SHFM伴发其他非肢体畸形)两类,分别按围产儿性别、居住地(城/乡)计算其发生率.并分析患儿的出生孕周、出生体质量、围产期转归和畸形发生部位.结果在4489692例围产儿中诊断出SHFM病例736例,总发生率为1.64/10~4,单发和综合征SHFM的发生率分别为0.64/10~4和1.00/10~4.男性围产儿SHFM发生率为1.79/10~4,女性为1.25/10~4;城镇1.51/10~4,乡村1.86/10~4.SHFM患儿中,未足月分娩和低出生体质量分别占30.20%和43.93%.单发和综合征SHFM的围产期病死率分别为24.74%和66.59%,总病死率为50.27%.SHFM畸形以发生在上肢多见.结论我国SHFM发生率高于国外报道.较高的围产期病死率与伴发畸形的严重程度有关.
목적 탐토중국인선천성렬수/렬족기형(SHFM)적류행병학특정화림상특점.방법 통과중국출생결함감측망락획득SHFM연구자료,파병례분위단발(부유SHFM혹자반발기타지체기형)화종합정(SHFM반발기타비지체기형)량류,분별안위산인성별、거주지(성/향)계산기발생솔.병분석환인적출생잉주、출생체질량、위산기전귀화기형발생부위.결과 재4489692례위산인중진단출SHFM병례736례,총발생솔위1.64/10~4,단발화종합정SHFM적발생솔분별위0.64/10~4화1.00/10~4.남성위산인SHFM발생솔위1.79/10~4,녀성위1.25/10~4;성진1.51/10~4,향촌1.86/10~4.SHFM환인중,미족월분면화저출생체질량분별점30.20%화43.93%.단발화종합정SHFM적위산기병사솔분별위24.74%화66.59%,총병사솔위50.27%.SHFM기형이발생재상지다견.결론 아국SHFM발생솔고우국외보도.교고적위산기병사솔여반발기형적엄중정도유관.
Objective To study the epidemiological and clinical features of congenital split hand/split foot malformation (SHFM) in Chinese population. Methods Data used in this study were provided by the Chinese Birth Defects Monitoring Network. The SHFM cases were categorized into two groups: isolated (SHFM only and SHFM with other limb defects) and syndromic (SHFM with non-limb defects). Prevalence rates were calculated by residential area (urban versus rural) and by gender. Further analyses were conducted to identify the characteristics of SHFM with related to gestational age, birth weight, perinatal outcome and affected limbs. Results A total of 736 newborn babies were identified with SHFM among 4,489,692 births, with a prevalence of 1.64/10,000. The prevalence of isolated SHFM and syndromic SHFM were 0.64 and 1.00 per 10,000 births, respectively. The prevalence of SHFM in male and female babies were 1.79 and 1.25 per 10,000 births, respectively. The prevalence of SHFM in urban and rural areas were 1.51 and 1.86 per 10,000 births, respectively. Preterm birth and low birth weight accounted for 30.20% and 43.93% of the SHFM cases, respectively. The perinatal mortality for the SHFM, isolated SHFM, and syndromic SHFM were 50.27%, 24.74%, and 66.59%, respectively. SHFM occurred more often in upper limbs. Conclusion The prevalence of SHFM in Chinese population is greater than foreign populations. The high perinatal mortality rate of SHFM is associated with the severity of accompanied malformations.