Factors affecting the growth of infants diagnosed with cystic fibrosis by newborn screening

Patterson, K. D.; Kyriacou, Theocharis; Desai, Maya; Carroll, Will; Gilchrist, Francis J

doi:10.1186/s12887-019-1727-9

Cited by 8 publications

(10 citation statements)

References 18 publications

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“…These mean values are the same as in France, Germany, and Denmark, according to an ECFS report. 22 In a study by Patterson et al 23 the rate of malnutrition was mild in cases diagnosed at infancy. Neonatal screening for CF has been performed in Turkey since 2015.…”

Section: Discussionmentioning

confidence: 94%

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

Kılınç¹,

Beşer²,

Ugur

et al. 2021

Pediatrics International

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Background: Cystic fibrosis (CF) causes malabsorption of nutrients that exacerbate pulmonary problems. Nutritional interventions can improve pulmonary functions. We aimed to evaluate the effects of nutritional intervention in CF patients with malnutrition, and to determine if there is a correlation between nutritional status and pulmonary functions. Methods: The study included 143 CF patients (67 females) with a mean 2 year follow-up time. Patients' sociodemographic data, presenting symptoms and history were recorded. Height-forage , weight-forage , weight-for-length/ height (WFL/H), and body mass index (BMI) were calculated in all patients. Patients were grouped as well nourished, mild malnutrition, moderate malnutrition, and severe malnutrition. These four groups were compared in terms of pulmonary function test results, lung infections, and the hospitalization rate. Results: Among the patients with a WFL/H or BMI z-scores that decreased, the frequency of lung infection was 74.1% and the hospitalization rate was 40.7%, versus 34% and 12.3%, respectively among the patients with a WFL/H or BMI z-scores that increased. The difference was significant (P = 0.02 and P = 0.01, respectively). The difference in bacterial lung infections differed significantly between the four nutritional status groups (P = 0.002). Patients in the well-nourished group had significantly higher pulmonary function test scores than the other groups. The forced expiratory volume in the first second differed significantly between the patients with and without an increase in the WFL/H or BMI z-scores (P = 0.001). Conclusions: The appropriate nutritional intervention to pediatric CF patients with malnutrition, decrease the frequency of lung infections, and improve respiratory function.

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Section: Discussionmentioning

confidence: 94%

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

Kılınç¹,

Beşer²,

Ugur

et al. 2021

Pediatrics International

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“…In a prospective longitudinal multicenter study of 99 infant PWCF, Munck et al 24 found an association between WFA and LFA z scores and the presences of initial pulmonary symptoms as well as an association between LFA z score and hospitalization for respiratory symptoms and Staphylococcus aureus infection. In comparison, Patterson et al 25 found an association between a single isolation of Pseudomonas aeruginosa and reduced weight and height in infant PWCF in the first 2 years of life. Similarly, the Baby Observational and Nutrition Study (BONUS) found lower weight or length in the 1st year of life was associated with P. aeruginosa infection, MI, PI, male sex, and histamine blocker use 26 .…”

Section: Discussionmentioning

confidence: 93%

“…Our results suggest that there could be other factors which play a greater role in growth and nutrition outcomes during infancy and toddlerhood in PWCF besides sZn. Multiple studies have reported an association with growth in PWCF and exocrine pancreatic function 24,25 . In a prospective longitudinal multicenter study of 99 infant PWCF, Munck et al 24 found an association between WFA and LFA z scores and the presences of initial pulmonary symptoms as well as an association between LFA z score and hospitalization for respiratory symptoms and Staphylococcus aureus infection.…”

Section: Discussionmentioning

confidence: 99%

“…Multiple studies have reported an association with growth in PWCF and exocrine pancreatic function. 24,25 In a prospective longitudinal multicenter study of 99 infant PWCF, Munck et al 24 found an association between WFA and LFA z sex, and histamine blocker use. 26 Other potential factors include alterations in levels of insulin-like growth factor, growth hormone binding protein, essential fatty acids, and growth hormone receptor gene expression.…”

Section: Discussionmentioning

confidence: 99%

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Zinc status and growth in infants and young children with cystic fibrosis

Bauer

Lai

McDonald

et al. 2021

Pediatric Pulmonology

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Background: Zinc deficiency is associated with poor growth in children without cystic fibrosis (CF), but its impact on growth in children with CF is unknown.Objective: To determine the prevalence of low serum Zn (sZn) and its relationship with growth in the first 3 years of life in children with CF.Methods: We utilized data from infants with CF who were enrolled in a longitudinal study of nutrition and lung health and had sZn measured as part of clinical care.Cross-sectional correlations between sZn levels and growth z scores were assessed by Pearson's correlation coefficient. To identify factors associated with sZn status and its association to longitudinal growth patterns, multiple regression analysis with repeated measures were performed using generalized estimating equations.Results: A total of 106 sZn measurements from 53 infants were identified. Seventeen infants (32%) had intermittent Zn insufficiency, defined as at least one sZn <70 mcg/dl in their first 3 years of life. There were no significant cross-sectional associations between sZn and growth z scores. However, analysis of longitudinal growth patterns revealed that weight-and length-for-age z scores in children with intermittent Zn insufficiency were lower during early infancy and their weight-forlength z scores at age 3 years were also lower compared to those who were always Zn sufficient. Conclusion:Low sZn occurs in one-third of children with CF in the first 3 years of life. Cross-sectional and longitudinal analyses revealed discrepant associations between sZn and growth. Therefore, prospective studies are needed to understand the role of Zn in growth in CF.

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“…It has been shown that even in developed countries, neonates screened positive for CF by newborn screening (NBS) develops growth retardation by the time diagnosis is confirmed by 3 weeks of age. [ 8 ] The challenge for a primary care physician in India is that there is neither NBS for CF in the country nor national guidelines to aid early identification of these infants. [ 9 ] Unless affected infants are diagnosed at the earliest, the therapeutic window for better nutrition is lost.…”

Section: Introductionmentioning

confidence: 99%

Effect of malnutrition in infants with cystic fibrosis in India

Thomas

John

Lionel

et al. 2021

Journal of Family Medicine and Primary Care

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Aims: To assess the clinical profile and nutritional status of infants with cystic fibrosis (CF) and track their nutritional outcomes with treatment. Materials and Methods: This retrospective study was conducted in a tertiary-care institute in South India. Demographic and clinical information were collected. The nutritional status and treatment outcome was assessed by Z-scores for weight-for-age (WAZ), length-for-age (LAZ), and weight-for-length (WLZ) at diagnosis and follow-up. Results: Nineteen infants with CF had mean follow-up duration of 9.7 ± 8.7 months. There was a mean delay of 2.9 ± 2.1 months from symptom onset to diagnosis, by which time infants were severely malnourished (mean WAZ -4.68 ± 1.8). Pneumonia, summer dehydration with electrolyte abnormalities (42.1%), and a combination of anemia, hypoalbuminemia, and malnutrition (42.1%) were the predominant features. Significant weight loss had been recorded in undiagnosed infants by second month of life before symptom onset. At follow-up, there was a remarkable improvement in WAZ (P 0.001), but not LAZ and WLZ. There was a high mortality rate of 37% in these infants. Conclusions: Malnutrition is a significant morbidity in infants with CF in India. There was significant improvement of WAZ with treatment, but it lagged behind the recommended targets. There is an opportunity for identification of CF infants at the time of vaccination at six and ten weeks of age, by the primary care physician and pediatrician. Screening of young infants having failure to thrive in the immunization clinic may be a strategy for early diagnosis of infants with severe CF phenotype.

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Factors affecting the growth of infants diagnosed with cystic fibrosis by newborn screening

Cited by 8 publications

References 18 publications

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

Zinc status and growth in infants and young children with cystic fibrosis

Effect of malnutrition in infants with cystic fibrosis in India

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