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Nutritional Status in the First 2 Years of Life in Cystic Fibrosis Diagnosed by Newborn Screening

Anne Munck¹, Rym Boulkedid², Laurence Weiss³

¹Service des maladies digestives et respiratoires de l'enfant, CRCM, Hôpital Robert Debré, AP-HP, University Paris Diderot.

Journal of Pediatric Gastroenterology and Nutrition

|March 16, 2018

Insights

Nutritional challenges in cystic fibrosis (CF) infants are linked to pancreatic insufficiency and early pulmonary symptoms. Early intervention and breastfeeding may improve outcomes for these children.

Area of Science:

Pediatrics
Nutritional Science
Genetics

Background:

Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, with nutritional status being a key determinant of health outcomes.
Newborn screening allows for early diagnosis and intervention in CF patients.
Understanding factors influencing nutritional status in early childhood CF is crucial for optimizing growth and development.

Purpose of the Study:

To evaluate the nutritional status of infants diagnosed with CF via newborn screening up to 24 months of age.
To identify factors associated with nutritional status, including pancreatic status, feeding methods, and pulmonary outcomes.

Main Methods:

A prospective, longitudinal, multicenter study followed 105 infants with CF.
Nutritional status was assessed based on pancreatic status, feeding modalities, medication, pulmonary status, and biological markers.
Logistic regression analysis was used to identify associated factors.

Main Results:

Infants with exocrine pancreatic insufficiency (EPI) had higher rates of undernutrition (15%) and stunting (24%) compared to those with exocrine pancreatic sufficiency (EPS).
Early pulmonary symptoms were associated with increased risk of poor weight and length z-scores at 24 months.
Stunting was linked to high-calorie intake and Staphylococcus aureus infection; earlier first visit (<1.2 months) reduced symptom frequency.

Conclusions:

Stunting is more prevalent than undernutrition in CF infants, primarily affecting those with EPI.
Feeding modalities were not significantly associated with nutritional status, but breastfeeding may delay Pseudomonas aeruginosa acquisition.
Despite supplementation, significant proportions of infants had suboptimal sodium and vitamin D levels, indicating ongoing nutritional management challenges.

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Abstract

Objective:

To evaluate nutritional status and associated factors in a cystic fibrosis (CF) cohort diagnosed by newborn screening and followed up to month 24.

Methods:

A prospective longitudinal multicenter study assessing nutritional status according to pancreatic status, feeding modalities, prescriptions, pulmonary outcome, and biological nutritional parameters.

Results:

One hundred and five infants were recruited and 99 completed the study. Nutritional care management prevented undernutrition and stunting in those with exocrine pancreatic sufficiency (EPS), but affected (13/87) 15% and (21/86) 24%, respectively, of infants with exocrine pancreatic insufficiency (EPI). The logistic regression model found a positive association between both weight and length z scores "at risk" at month 24, and initial pulmonary symptoms (odds ratio [OR] 0.06, P < 0.01 and OR 0.08, P < 0.01, respectively); these symptoms were less frequent when age at first visit was earlier than 1.2 months (33% vs 67%, P = 0.02); stunting was also associated with high-calorie density intake and Staphylococcus aureus (OR 0.05, P = 0.01 and OR 0.17, P < 0.01). Pulmonary outcome did not differ according to pancreatic status; breast-feeding for at least 3 months delayed first acquisition of Pseudomonas aeruginosa. Despite sodium and fat-soluble vitamin supplementation, half of both cohorts had low urinary sodium output and half of the EPI cohort had low vitamin D levels.

Conclusions:

Our data shed light on the fact that stunting was more frequent than undernutrition, while both parameters involved only patients with pancreatic insufficiency. Modalities of feeding were not associated with nutritional status; breast-feeding may provide some protection against acquisition of P aeruginosa.

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