Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: The israeli multicenter study

Ori Efrati, Judith Nir, Drora Fraser, Malena Cohen-Cymberknoh, David Shoseyov, Daphna Vilozni, Dalit Modan-Moses, Ran Levy, Amir Szeinberg, Eitan Kerem, Joseph Rivlin

Research output: Contribution to journalArticlepeer-review

Abstract

Objectives: Patients with cystic fibrosis (CF) presenting with meconium ileus (MI) tend to have worse outcomes than those without MI. We evaluated the clinical characteristics and survival rates among Israeli patients with CF with and without MI after a prolonged follow-up (15-30 years). Patients and Methods: A multicenter retrospective study. Forty-nine patients with CF, representing 13.8% of all patients with CF in Israel, presented with MI (current age 17.4 ± 7.9 years) between 1975 and 2006. They were compared with 38 patients with CF (current age 19.3 ± 6.5 years) without MI matched by sex and CF transmembrane conductance regulator mutation. Results: A total of 66.2% of patients with MI and 73.6% without MI were followed for a prolonged period (24.9 ± 2.7 years). Of the patients with MI, 31 were managed operatively, whereas 18 were treated successfully with gastrograffin enema, with similar clinical outcomes. Five patients in the MI group and 3 in the control group died during the study period. Bacterial colonization, z score of body mass index, and pulmonary function tests were similar in patients with and without MI in the long term. In younger patients, many clinical parameters were more prevalent in patients with MI (P = 0.004). However, these differences disappeared after the long-term follow-up (up to 31-years). Conclusions: Patients with CF presenting with MI had similar pulmonary function and nutritional status, as well as survival rates as did the control patients without MI. The distinct genetic mutation found in our population may explain in part the favorable results compared with other studies. In addition, it seems that early diagnosis and treatment of MI in patients with CF may be beneficial, subsequently lowering morbidity, and increasing survival.

Original languageEnglish
Pages (from-to)173-178
Number of pages6
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume50
Issue number2
DOIs
StatePublished - Feb 2010
Externally publishedYes

Keywords

  • Cystic fibrosis
  • Meconium ileus
  • Nutritional status
  • Pulmonary function
  • Survival

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