The impact of meconium ileus on the clinical course of children with cystic fibrosis

The present study was designed to compare the clinical course of children d iagnosed with cystic fibrosis (CF) in infancy due to the presence of meconi um ileus (MI) with children diagnosed by way of a newborn screening program me (non-Ml). A matched case-control study design was used. Matching was performed on the basis of sex and date of birth. All children born in New South Wales, Aust ralia after 1980 and who had attended the CF clinic at The Children's Hospi tal at Westmead since diagnosis were included as possible cases or controls . Parameters pertaining to the clinical course were compared in 39 matched pairs. MI children had a significantly worse pulmonary status. The forced expirato ry volume in one second was 16.3 +/-5.2% higher (p <0.001, n=21 pairs) and the forced vital capacity value 10.5 +/-4.7% higher (p <0.05, n=21 pairs) i n non-Ml children. The difference between the pairs (18.6 +/-4.4 MI and 20. 5 +/-3.4 non-MI) in the Shwachman chest radiograph score was statistically significant (p <0.05, n=39 pairs). There were no significant differences in any other assessed parameters, such as height, weight, the presence of liv er function abnormalities, the frequency of hospitalization or airway micro bial colonization. Meconium ileus may be an early indication of a more severe phenotype of cys tic fibrosis. This was suggested by the significantly lower pulmonary funct ion found in children with a history of meconium ileus compared to age- and sex-matched children who did not have meconium ileus.