Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: the Israeli Multicenter Study.
OBJECTIVE
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).


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.
Efrati
Ori
O
Pediatric Pulmonary Unit, The Edmond and Lily Safra Children's Hospital, Chaim Sheba Medical Center, Tel HaShomer, Ramat-Gan, Israel. ori.efrati@sheba.health.gov.il
Nir
Judith
J
Fraser
Drora
D
Cohen-Cymberknoh
Malena
M
Shoseyov
David
D
Vilozni
Daphna
D
Modan-Moses
Dalit
D
Levy
Ran
R
Szeinberg
Amir
A
Kerem
Eitan
E
Rivlin
Joseph
J
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D015992:Body Mass Index; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D003974:Diatrizoate Meglumine / Q000627:therapeutic use; D018450:Disease Progression; D004733:Enema; D005260:Female; D006801:Humans; D045823:Ileus / Q000150:complications* / Q000628:therapy; D007223:Infant; D007557:Israel; D008168:Lung; D008297:Male; D008470:Meconium; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
D003974:Diatrizoate Meglumine