The role of neonatal screening in nutritional evolution in the first 12 months after diagnosis of cystic fibrosis

Janine Pruinelli Martins,Miriam Isabel Souza Dos Santos Simon,Paulo José Cauduro Marostica,Matias Epifanio,Leonardo Araújo Pinto,Gabriele Carra Forte

doi:10.1590/1806-9282.64.11.1032

Abstract

to assess the progression of pediatric cystic fibrosis (CF) patients' nutritional status during the first 12 months after diagnosis and to establish its association with neonatal screening and clinical variables. Patients were recruited from two reference centers in Southern Brazil. Retrospective cohort study was carried out with all the patients diagnosed between 2009 and 2014. Anthropometric, clinic and neonatal screening were collected from medical files. Analysis of anthropometric markers over time was performed by generalized estimating equations. A multivariate regression analysis model to predict the Δ percentile body mass index (BMI) (BMI percentile difference between one year after the treatment and BMI percentile at diagnosis) was done. Forty-seven patients were included in the study. Analysis of nutritional data over the period between six months and one year after diagnosis showed significant improvement of BMI, weight/age and weight/height percentiles and Z scores. The neonatal screening was associated with a significant increase of 31.2 points in ΔBMI percentile at the one-year evaluation (p<0.05). On the other hand, a one-point increase of initial BMI percentile was associated with a reduction of 0.6 points in ΔBMI percentile. This study demonstrated the role of neonatal screening in the nutritional status of patients diagnosed with CF in the first year after diagnosis. Early diagnosis can significantly contribute to the achievement of appropriate anthropometric indicators and important nutritional recovery of CF patients.

Highlights

Cystic Fibrosis (CF) is accompanied by chronic obstructive pulmonary disease, pancreatic insufficiency with poor digestion and poor absorption of nutrients, leading to the worsening of the nutritional status[1]
The neonatal screening was associated with a significant increase of 31.2 points in ΔBMI percentile at the one-year evaluation (p
A one-point increase of initial body mass index (BMI) percentile was associated with a reduction of 0.6 points in ΔBMI percentile

Summary

Introduction

Cystic Fibrosis (CF) is accompanied by chronic obstructive pulmonary disease, pancreatic insufficiency with poor digestion and poor absorption of nutrients, leading to the worsening of the nutritional status[1]. The neonatal screening for CF with early treatment and identification of the pancreatic disease and proper nutritional management has been associated with the reduction of nutritional deficits and improvement of growth parameters over the years[2,3]. ET AL sion of the pulmonary disease and survival, the studies show an advantage in the pulmonary function up to at least 8 to 10 years old[4]. The strong association of the nutritional status and pulmonary function[5,6] and the increase of survival[7] indicate a possible improvement of morbimortality in the patients diagnosed by neonatal screening

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