小儿囊性纤维化患者有无胎粪性肠梗阻的临床结局差异。

Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus.

机构信息

Department of Gastroenterology, Sydney Children's Hospital, High Street, Randwick NSW 2031, Australia.

Discipline of Paediatrics, School of Women's and Children's Health, University of New South Wales, Sydney NSW 2052, Australia.

出版信息

J Cyst Fibros. 2019 Nov;18(6):857-862. doi: 10.1016/j.jcf.2019.09.008. Epub 2019 Oct 28.

DOI:10.1016/j.jcf.2019.09.008

PMID:31672555

Abstract

BACKGROUND

Meconium ileus (MI) affects up to 20% of newborns with cystic fibrosis (CF). We compared clinical outcomes between Australian paediatric CF patients with and without meconium ileus (non-MI).

METHODS

This was a retrospective case-control study of MI and non-MI patients in New South Wales, Australia, from 1988 to 2010. MI patients were matched 1:1 with pancreatic insufficient non-MI patients for age, sex and CF clinic. Clinical measurements, nutrition and gastrointestinal outcomes over this period were compared between groups using linear mixed models for continuous variables to account for age.

RESULTS

There were 162 matched pairs (N=324, 52% female) with mean (SD) age of 15.3 (8.2) and 14.9 (7.9) years for MI and non-MI patients respectively (P=0.6). MI patients aged 5-23 had poorer FEV1% compared to non-MI patients (estimate -0.070 SE [0.02], P=0.003). There were no significant differences in P. aeruginosa isolation rates; however S. aureus isolation rates were lower in MI patients (72%) compared to non-MI (82%) (OR 0.6 [0.3-1.0], P=0.03). Chronic colonisation rates for P. aeruginosa and S. aureus were not significantly different between groups. MI patients aged 2-20 had significantly lower BMI Z-scores over time (estimate -0.25 SE [0.1], P=0.02). MI patients were more likely to receive oral feed supplements (OR 2.8 [1.4-6.1], P=0.003) and gastrostomy formation (OR 4.4 [1.1-24.6], P=0.02).

CONCLUSIONS

CF patients with MI may have worse lung function, growth and nutrition than non-MI patients over time. Meconium ileus may be an early poor prognostic factor for CF.

摘要

背景

胎粪性肠梗阻（MI）影响高达 20%的囊性纤维化（CF）新生儿。我们比较了澳大利亚儿科 CF 患者中伴有和不伴有胎粪性肠梗阻（非-MI）的临床结局。

方法

这是 1988 年至 2010 年澳大利亚新南威尔士州 MI 和非-MI 患者的回顾性病例对照研究。MI 患者与胰腺功能不全的非-MI 患者按年龄、性别和 CF 诊所进行 1:1 匹配。使用线性混合模型对两组患者在该期间的临床测量、营养和胃肠道结果进行比较，以考虑年龄因素。

结果

共有 162 对匹配的患者（N=324，52%为女性），MI 和非-MI 患者的平均（SD）年龄分别为 15.3（8.2）和 14.9（7.9）岁（P=0.6）。5-23 岁的 MI 患者的 FEV1%较非-MI 患者差（估计值-0.070 SE[0.02]，P=0.003）。两组患者的铜绿假单胞菌分离率无显著差异；然而，MI 患者金黄色葡萄球菌分离率（72%）低于非-MI 患者（82%）（OR 0.6[0.3-1.0]，P=0.03）。两组患者铜绿假单胞菌和金黄色葡萄球菌的慢性定植率无显著差异。2-20 岁的 MI 患者的 BMI Z 评分随时间明显降低（估计值-0.25 SE[0.1]，P=0.02）。MI 患者更可能接受口服喂养补充剂（OR 2.8[1.4-6.1]，P=0.003）和胃造口术（OR 4.4[1.1-24.6]，P=0.02）。