Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a matched cohort study

J Pediatr Surg. 2006 Sep;41(9):1556-60. doi: 10.1016/j.jpedsurg.2006.05.014.

Abstract

Objective: 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).

Material and method: 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.

Results: 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.

Conclusion: 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.

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Cathartics / therapeutic use
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cystic Fibrosis / complications*
  • Diatrizoate Meglumine / therapeutic use
  • Digestive System Surgical Procedures
  • Enema
  • Female
  • Humans
  • Ileus / etiology*
  • Ileus / therapy*
  • Infant
  • Intestinal Obstruction / etiology
  • Intestinal Obstruction / therapy
  • Male
  • Meconium
  • Nutrition Assessment
  • Pseudomonas Infections / microbiology
  • Pseudomonas aeruginosa
  • Respiratory Function Tests
  • Treatment Outcome

Substances

  • Cathartics
  • Diatrizoate Meglumine