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A review of newborn screening results and anthropometric measurements in infants diagnosed with cystic fibrosis in the West Midlands

Patterson, Katie Denise

A review of newborn screening results and anthropometric measurements in infants diagnosed with cystic fibrosis in the West Midlands Thumbnail


Authors

Katie Denise Patterson



Abstract

INTRODUCTION:
Newborn screening (NBS) for cystic fibrosis (CF) was implemented nationally in July 2007 and has reduced the age of diagnosis and produced better nutritional and survival outcomes.

AIM:
To assess demographic data of a screened cohort and their anthropometric and microbiology results from birth to 2 years. We undertook cluster analysis of the raw data and developed a model to predict weight and length z scores.

METHODS:
Data were collected from the West Midlands NBS Laboratory on babies screened between November 2007 and October 2014. A retrospective case notes review was performed on all confirmed cases.

RESULTS:
507,608 infants were screened. 200 had a positive CF NBS and 144 were subsequently diagnosed with CF (birth prevalence: 1/3525). In those with CF, 11.8% isolated Staphylococcus aureus within 2 years and 34.5% isolated Pseudomonas aeruginosa. The median birth weight (BW) of the children with CF was 3.09kg with no difference between pancreatic sufficient/insufficient patients. The median time for infants to reach a z score of 0 for weight was 65 weeks and 90 weeks to achieve the same z score for length. Cluster analysis identified 2 distinct groups. We have developed robust models to predict weight and length z scores at 1 and 2 years of age using data available at first clinic visit.

CONCLUSION:
The birth prevalence of CF in the West Midlands is lower than the UK, which is likely to reflect its ethnic diversity. Babies with CF had a normal birth weight but lost weight by their first clinic visit. Infants with CF achieve normal weight before length. Our models to predict future length and weight have the potential to identify children at risk of poor growth in the first 2 years of life.

Publicly Available Date Mar 28, 2024

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