Growth Deficiency in Cystic Fibrosis Is Observable at Birth and Predictive of Early Pulmonary Function

Rebecca Darrah; Rebecca Nelson; Elizabeth G Damato; Michael Decker; Anne Matthews; Craig A Hodges

doi:10.1177/1099800416643585

Growth Deficiency in Cystic Fibrosis Is Observable at Birth and Predictive of Early Pulmonary Function

Biol Res Nurs. 2016 Oct;18(5):498-504. doi: 10.1177/1099800416643585. Epub 2016 Apr 13.

Authors

Rebecca Darrah¹, Rebecca Nelson², Elizabeth G Damato³, Michael Decker³, Anne Matthews⁴, Craig A Hodges⁵

Affiliations

¹ Department of Genetics, Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland, OH, USA Co-first authors rjm11@case.edu.
² Department of Genetics, Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland, OH, USA Co-first authors.
³ Department of Genetics, Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland, OH, USA Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland, OH, USA.
⁴ Department of Genetics, Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland, OH, USA.
⁵ Department of Genetics, Frances Payne Bolton School of Nursing, Case Western Reserve University, Cleveland, OH, USA Department of Pediatrics, Rainbow Babies and Children's Hospital, Cleveland, OH, USA.

Abstract

Introduction: Cystic fibrosis (CF) is a complex disease that includes both pulmonary and gastrointestinal challenges, resulting in decreased weight. Pulmonary symptoms of CF are extremely variable. Greater body mass at an early age is associated with improved pulmonary function, but it is unknown at what age weight becomes predictive of pulmonary disease severity. The purpose of this study was to investigate the relationship between birth weight and pulmonary function in CF.

Methods: Birth weight and pulmonary data were obtained. Linear regressions were used to examine the relationship between these two variables. A one-tailed t-test was used to compare birth weights between CF patients and the national average.

Results: Birth weight was significantly lower in babies with CF and correlated with pulmonary disease at ages 6 and 10 years but not with age at which Pseudomonas aeruginosa colonization was observed.

Discussion: These data suggest that CF growth deficiency has prenatal origins. Early nutritional intervention for babies with CF and a low birth weight is warranted to maximize pulmonary potential.

Keywords: birth weight; cystic fibrosis; genetics; pediatrics; respiratory.

Publication types

Comparative Study

MeSH terms

Adolescent
Birth Weight*
Child
Child, Preschool
Cystic Fibrosis / physiopathology*
Early Diagnosis*
Female
Growth and Development*
Humans
Infant
Infant, Newborn
Lung / physiopathology*
Male
Predictive Value of Tests
Respiratory Function Tests
Severity of Illness Index

Abstract

Publication types

MeSH terms

Grants and funding