Cystic Fibrosis Support Centre, Paediatric Department, University of Brescia, ASST Spedali Civili Brescia, Italy.
Cystic Fibrosis Centre, Mother-Child Department, United Hospitals, Ancona, Italy.
J Cyst Fibros. 2019 Nov;18(6):863-868. doi: 10.1016/j.jcf.2019.07.003. Epub 2019 Jul 25.
Meconium ileus (MI) is a risk factor for poor outcomes in cystic fibrosis (CF) patients. The aim of this study was to identify the risk factors for poor 12-month clinical outcomes in MI-CF newborns.
This retrospective, multicentre, observational study of MI-CF infants born 2009-2015 recorded their pre- and neonatal histories, intestinal occlusion treatments, post-surgical history, nutrition, CF diagnosis, and compared the patients with 12-month faltering growth or chronic Pseudomonas aeruginosa respiratory infection (cases) with the others (controls).
About 25% of the 85 patients enrolled by 13 Italian CF centres (24% premature, 18% of low birth weight) had prenatally diagnosed bowel obstruction, and 39% had complex MI. Seventy-one required surgery (the 33 with complex MI and 38 with simple MI), of whom 58 (82%) required post-surgical intensive care, including 25 (35%) needing ventilatory support. Forty-six (54%) were breastfed; exclusively parenteral nutrition was started in 52 (61%). Cholestasis was diagnosed in 21%. Thirty-one (37%) experienced negative outcomes: the only risk factors were prenatally diagnosed intestinal obstruction and a need for intensive care and oxygen therapy. The cases had significantly higher first blood immunoreactive trypsinogen (b-IRT) levels (P = .008). Logistic regression showed that the probability of having negative outcome is decreased in the absence of cholestasis (Odds Ratio = 0.125) and a need for intensive therapy (OR = 0.141), and increased by not having been breastfed (OR = 2.921).
High b-IRT levels, prenatally diagnosed intestinal obstruction, a severe post-surgical clinical picture and early liver disease are risk factors for negative outcomes. Breastfeeding may be protective.
胎粪性肠梗阻（MI）是囊性纤维化（CF）患者预后不良的一个危险因素。本研究旨在确定 MI-CF 新生儿 12 个月临床结局不良的危险因素。
本回顾性、多中心、观察性研究纳入了 2009 年至 2015 年期间出生的 MI-CF 婴儿，记录了他们的产前和新生儿病史、肠闭塞治疗、术后病史、营养状况、CF 诊断，并将 12 个月生长迟缓或慢性铜绿假单胞菌呼吸道感染的患者（病例）与其他患者（对照组）进行比较。
在 13 个意大利 CF 中心纳入的 85 名患者中，约有 25%（24%为早产儿，18%为低出生体重儿）存在产前诊断的肠阻塞，39%存在复杂 MI。71 名患者需要手术（33 名复杂 MI 患者和 38 名简单 MI 患者），其中 58 名（82%）需要术后重症监护，包括 25 名（35%）需要呼吸机支持。46 名（54%）接受母乳喂养；52 名（61%）开始接受完全肠外营养。21%被诊断为胆汁淤积。31 名（37%）出现不良结局：唯一的危险因素是产前诊断的肠梗阻、需要重症监护和氧疗。病例组的首次血免疫反应性胰蛋白酶原（b-IRT）水平显著更高（P=0.008）。Logistic 回归显示，无胆汁淤积（优势比[OR] = 0.125）和需要重症治疗（OR = 0.141）的情况下，发生不良结局的可能性降低，而未接受母乳喂养（OR = 2.921）的情况下，发生不良结局的可能性增加。
高 b-IRT 水平、产前诊断的肠梗阻、严重的术后临床情况和早期肝病是不良结局的危险因素。母乳喂养可能具有保护作用。
