CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention.

Study design (if review, criteria of inclusion for studies)

This study was part of an ongoing, randomized, longitudinal investigation

Participants

infants diagnosed with cystic fibrosis (CF) through neonatal screening

Interventions

energy intake

Outcome measures

nutritional status, energy and macronutrient-consumption patterns

Main results

normal patterns could be achieved with mean energy intake values at ages 6 and 12 mo of 481 and 426 kJ/kg body wt (115 and 102 kcal/kg body wt), respectively. Biochemical assessment demonstrated low alpha-tocopherol and linoleic acid values at diagnosis in the majority of infants whereas one-third had abnormal indices of protein nutriture. Essential fatty acid deficiency was also demonstrated at diagnosis by abnormal triene-tetraene ratio values in 27% of screened infants.

Authors' conclusions

With predigested formula and dietary supplementation, there was improvement in all indices of nutritional status and only a low percentage of patients showed mild biochemical abnormalities at age 12 mo.

Keywords: Neonatal Screening; Newborn; non pharmacological intervention - diagn; screening; diagnostic procedures;