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

¹ Children's Chest Research Centre, Dept of Respiratory Medicine, The Children's Hospital at Westmead (Royal Alexandra Hospital for Children), Westmead, New South Wales, Australia and ² Dept of Paediatrics and Child Health, The University of Sydney, New South Wales, Australia

CORRESPONDENCE: K.O. McKay, Dept of Respiratory Medicine, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, New South Wales, 2145, Australia. Fax: 61 298453396

Keywords: cystic fibrosis, meconium ileus, neonatal screening, nutrition, pulmonary function

Received: June 15, 2000
Accepted February 20, 2001

The present study was designed to compare the clinical course of children diagnosed with cystic fibrosis (CF) in infancy due to the presence of meconium ileus (MI) with children diagnosed by way of a newborn screening programme (non-MI).

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, Australia after 1980 and who had attended the CF clinic at The Children's Hospital 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 expiratory 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) in non-MI 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 liver function abnormalities, the frequency of hospitalization or airway microbial colonization.

Meconium ileus may be an early indication of a more severe phenotype of cystic fibrosis. This was suggested by the significantly lower pulmonary function found in children with a history of meconium ileus compared to age- and sex-matched children who did not have meconium ileus.

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