Munck Anne, Gérardin Michèle, Alberti Corinne, Ajzenman Catherine, Lebourgeois Muriel, Aigrain Yves, Navarro Jean
Cystic Fibrosis Centre, Hospital Robert Debré 48, bd Serurier, 75019 Paris, France.
J Pediatr Surg. 2006 Sep;41(9):1556-60. doi: 10.1016/j.jpedsurg.2006.05.014.
This matched case-control study compared the nutritional and the pulmonary long-term outcomes of cystic fibrosis (CF) patients presenting a history of meconium ileus (MI) with early-diagnosed symptomatic CF without MI (non-MI).
Twenty-six patients with CF treated for MI between 1980 and 1997 have been matched for sex, birth date, and earliest CF symptomatic diagnosis for the children with non-MI CF. Clinical characteristics, genotype and complications were evaluated as well as the progression of the CF disease from infancy to 15 years old by nutritional status (z score weight, z score height), pulmonary function tests (PFTs) (FVC and FEV1), and Pseudomonas aeruginosa acquisition.
Median duration of the follow-up was 12.5 years (range, 10-17 years). Genotype identification showed no significant difference. Further on, the rate of complications and the occurrence of chronic P. aeruginosa colonization did not differ. At age of 15 years (n = 13), nutritional status and PFTs did not demonstrate any significant difference.
These results suggest that adequate initial nutritional and medical management of MI allows further similar nutritional status and PFTs compared with other early-diagnosed symptomatic CF patients. In this study, MI did not represent an additional risk factor for the patient's life.
本配对病例对照研究比较了有胎粪性肠梗阻（MI）病史的囊性纤维化（CF）患者与早期诊断的无MI症状性CF（非MI）患者的营养和肺部长期预后。
1980年至1997年间接受MI治疗的26例CF患者，已与非MI CF儿童在性别、出生日期和最早的CF症状诊断方面进行了配对。评估了临床特征、基因型和并发症，以及通过营养状况（体重z评分、身高z评分）、肺功能测试（PFTs）（用力肺活量和第一秒用力呼气量）和铜绿假单胞菌感染情况从婴儿期到15岁的CF疾病进展。
随访的中位持续时间为12.5年（范围10 - 17年）。基因型鉴定显示无显著差异。此外，并发症发生率和慢性铜绿假单胞菌定植的发生情况也无差异。在15岁时（n = 13），营养状况和PFTs未显示任何显著差异。
这些结果表明，与其他早期诊断的有症状CF患者相比，对MI进行充分的初始营养和医疗管理可使营养状况和PFTs进一步相似。在本研究中，MI并非患者生命的额外危险因素。
