Nutritional management of the infant with cystic fibrosis.

Identification of the infant with cystic fibrosis by neonatal screening of blood immunoreactive trypsin has been undertaken in some regions for many years,' and molecular methods are increasingly being used for the genetic detection of affected children.2Several retrospective studies suggest that early detection of cystic fibrosis is beneficial in the long term3 4: improved nutritional status,5 and decreased morbidity from respiratory infection follow treatment that begins soon after diagnosis.6Detection of the cystic fibrosis gene7 has not only made possible genetic screening of affected fetuses and carriers,8 but may also offer a cure for this disease.9While exciting therapeutic prospects, such as manipulation of the abnormal gene product10 and pharmaco- logical interventions to correct defective chloride transport across epithelial cell mem- branes 1 are explored, there remains the chal- lenge of caring for the infant with cystic fibrosis identified in the neonatal period.The major objectives of treatment are to prevent pulmonary infection and to ensure optimal growth and nutrition.Physiotherapy from diagnosis, a The Children's full course of immunisations, and prophylactic Hospital, Leicester oral flucloxacillin6 are all indicated.Royal 1nfirmary, In this review we discuss the nutritional man-Leicester agement of the infant with cystic fibrosis identi- fied soon after birth, in the light of our growing Department of understanding of the pathophysiology of the Hospital for Sick disease in early life.There are three major Children, Glasgow reasons why nutrition of the infant with cystic E Buchanan fibrosis requires special attention (table 1).