Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study

J Cyst Fibros. 2019 Nov;18(6):863-868. doi: 10.1016/j.jcf.2019.07.003. Epub 2019 Jul 25.

Abstract

Background: 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.

Methods: 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).

Results: 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).

Conclusions: 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.

Keywords: Breastfeeding; Cystic fibrosis; Faltering growth; Meconium ileus; Risk factors.

Publication types

  • Multicenter Study
  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Breast Feeding / statistics & numerical data*
  • Critical Care / statistics & numerical data
  • Cystic Fibrosis* / epidemiology
  • Cystic Fibrosis* / genetics
  • Cystic Fibrosis* / physiopathology
  • Cystic Fibrosis* / therapy
  • Digestive System Surgical Procedures* / adverse effects
  • Digestive System Surgical Procedures* / methods
  • Female
  • Humans
  • Infant, Newborn
  • Intestinal Atresia / diagnosis*
  • Intestinal Obstruction* / diagnosis
  • Intestinal Obstruction* / etiology
  • Intestinal Obstruction* / surgery
  • Italy / epidemiology
  • Liver Diseases* / diagnosis
  • Liver Diseases* / etiology
  • Male
  • Meconium Ileus* / diagnosis
  • Meconium Ileus* / epidemiology
  • Meconium Ileus* / physiopathology
  • Meconium Ileus* / surgery
  • Outcome and Process Assessment, Health Care
  • Postoperative Complications / diagnosis*
  • Prenatal Diagnosis / methods*
  • Prognosis
  • Protective Factors
  • Risk Factors