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巨大膈肌缺损:骨骼畸形可预防吗?

Large diaphragmatic defect: are skeletal deformities preventable?

作者信息

Kuklová P, Zemková D, Kyncl M, Pycha K, Straňák Z, Melichar J, Snajdauf J, Rygl M

机构信息

Department of Pediatric Surgery, 2nd Faculty of Medicine and University Hospital Motol, Charles University in Prague, V úvalu 84, 15000, Prague 5, Czech Republic.

出版信息

Pediatr Surg Int. 2011 Dec;27(12):1343-9. doi: 10.1007/s00383-011-2973-6. Epub 2011 Sep 20.

DOI:10.1007/s00383-011-2973-6
PMID:21932166
Abstract

PURPOSE

The aim of study was to compare growth, nutritional status and incidence of chest wall deformities and scoliosis in survivors of large congenital diaphragmatic hernia (CDH) defect (Gore-Tex patch reconstruction) with survivors with smaller defects and primary reconstruction.

MATERIALS AND METHODS

An anthropometric study of 53 children who underwent CDH repair in neonatal period was carried out. Weight, height, and skin-fold thickness were measured, scoliosis and chest wall deformity were evaluated. Body mass index (BMI) and thoracic index (TI) were calculated using standard rules. The measured data were compared with national population standard with the use of standard deviation score (SDS). According to the type of diaphragmatic reconstruction, the patients were divided into two groups [Gore-Tex patch (10) versus primary repair (43)]. Student t test and Fisher exact tests were used for statistical analysis.

RESULTS

Pectus excavatum was found in 25 (47%) patients, poor posture in 33% and significant scoliosis in 5%. Compared with the population norm, CDH children had a significantly lower body height SDS (mean -0.39, p < 0.05), weight SDS (mean -0.75, p < 0.001), BMI (mean SDS -0.68, p < 0.001) and lower TI (mean SDS -0.62, p < 0.01). Gore-Tex versus primary repair group significantly differed in incidence of pectus excavatum and BMI (PE: p = 0.027, BMI SDS: p = 0.016). A majority of anthropometric parameters (weight, height, thoracic index, and thorax circumference) and incidence of scoliosis and poor posture in children after Gore-Tex patch reconstruction did not significantly differ from children after primary repair.

CONCLUSION

The differences in some anthropometric parameters (weight, BMI, and TI) and in the skeletal deformity suggest that the CDH not only disturbs normal lung growth, but also seems to have implications on some other aspects of somatic development. Whether these changes could be related to the type of diaphragmatic reconstruction or rather to the size of the defect remains uncertain.

摘要

目的

本研究旨在比较大型先天性膈疝(CDH)缺损(戈尔泰克斯补片重建)幸存者与较小缺损及一期重建幸存者的生长发育、营养状况、胸壁畸形和脊柱侧弯的发生率。

材料与方法

对53例新生儿期接受CDH修补术的儿童进行人体测量学研究。测量体重、身高和皮褶厚度,评估脊柱侧弯和胸壁畸形情况。使用标准规则计算体重指数(BMI)和胸廓指数(TI)。将测量数据与全国人口标准进行比较,采用标准差评分(SDS)。根据膈重建类型,将患者分为两组[戈尔泰克斯补片组(10例)与一期修补组(43例)]。采用学生t检验和Fisher精确检验进行统计分析。

结果

25例(47%)患者发现漏斗胸,33%姿势不良,5%有明显脊柱侧弯。与人群标准相比,CDH患儿的身高SDS显著更低(平均-0.39,p<0.05),体重SDS(平均-0.75,p<0.001),BMI(平均SDS -0.68,p<0.001),TI更低(平均SDS -0.62,p<0.01)。戈尔泰克斯补片组与一期修补组在漏斗胸发生率和BMI方面有显著差异(漏斗胸:p = 0.027,BMI SDS:p = 0.016)。戈尔泰克斯补片重建术后儿童的大多数人体测量参数(体重、身高、胸廓指数和胸围)以及脊柱侧弯和姿势不良的发生率与一期修补术后儿童相比无显著差异。

结论

一些人体测量参数(体重、BMI和TI)以及骨骼畸形的差异表明,CDH不仅干扰正常肺生长,而且似乎对身体发育的其他方面也有影响。这些变化是与膈重建类型有关还是与缺损大小有关仍不确定。

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