Cystic Fibrosis

Dickinson, Kimberly M.; Collaco, Joseph M.

doi:10.1542/pir.2019-0212

Cited by 76 publications

(66 citation statements)

References 72 publications

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“…Cystic fibrosis (CF) is a genetic disorder and its phenotype results from mutations in the transmembrane conductance regulatory protein gene (CFTR), which leads to CFTR deficiency or dysfunction, changes that disable the transport of sodium and chloride ions through epithelial cells, and other membranes. 1 As a result, fluid transport is abnormal and mucous secretions become thick, thus impairing the function of organs such as the lungs, pancreas, liver, gallbladder, and intestines. Once in the lungs, thick mucus adheres to airway surfaces epithelium, which leads to impaired mucociliary clearance, an increased risk of inflammation, and infection.…”

Section: Introductionmentioning

confidence: 99%

Effect of Protein Intake on Weight Gain and Growth in Cystic Fibrosis Patients

Epifânio¹,

Ongaratto

Pacheco³

et al. 2022

MRAJ

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Background and aim: despite the knowledge about the importance of adequate nutritional status in the health of patients with cystic fibrosis (CF), achieving protein intake recommendation remains a challenge. The influence of protein on anthropometric parameters in these patients needs to be better studied. The aim of the study was evaluated anthropometric changes after nutritional counselling to increase protein content in CF children and adolescents with low protein intake. Methods: a prospective longitudinal study in 20 children and adolescents with CF. Food record of 3 consecutive days and anthropometric assessment were applied with an interval of 3 months. Patients with a protein intake <20% of the total daily caloric intake received nutritional counselling to increase protein content and patients with protein intake of =20% maintained their routine diet. Data were analyzed using the Generalized Estimating Equations (GEE) model. Results: 15 patients (75%) had a daily protein intake of less than 20% of total daily calories at the first dietary assessment and received nutritional counselling. After 3 months, the protein content of the diet underwent a statistically significant increase, despite the other macronutrients and total daily calories. There was a significant improvement in z scores for weight (p<0.001), height (p<0.001) and body mass index (BMI) (p=0.001) compared to patients with adequate protein intake. Conclusion: most CF patients did not meet the daily recommendation for protein intake. The reach of the proper protein intake allowed an increase in weight, height, and BMI.

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Section: Introductionmentioning

confidence: 99%

Effect of Protein Intake on Weight Gain and Growth in Cystic Fibrosis Patients

Epifânio¹,

Ongaratto

Pacheco³

et al. 2022

MRAJ

View full text Add to dashboard Cite

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“…Cystic Fibrosis (CF) is the most common genetically determined disease among Caucasians. It is inherited in an autosomal recessive manner [ 1 ]. According to the European Cystic Fibrosis Society Patient Registry annual report from the year 2020, there were 52,246 registered CF patients living in 39 of the European and neighboring countries.…”

Section: Introductionmentioning

confidence: 99%

“…Due to the residual thick discharge, it is common for CF patients to suffer from the Pseudomonas aeruginosa pulmonary infections and develop the exocrine gland duct obstruction, which can lead to chronic inflammation. Although respiratory problems are the best-known symptoms, CF is a systemic disease in which the functioning of the pancreas, intestines, salivary, sweat glands and the reproductive system is impaired [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Mental Health of Cystic Fibrosis Patients and the COVID-19 Pandemic in Poland: A Single-Center Comparative Study

Humaj-Grysztar

Rachel

Śmiech-Michalec

et al. 2022

IJERPH

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Research shows that people with cystic fibrosis are more prone to suffer from psychological problems than healthy people; thus, the outbreak of the COVID-19 pandemic in Poland could have had an impact on their mental health. To assess this impact, we examined the mental health of patients before and during the pandemic. Survey participants were asked to fill in questionnaires that consisted of Beck Depression Inventory (BDI), 12-Item General Health Questionnaire (GHQ-12) and Cystic Fibrosis Questionnaire-Revised (CFQ-R; for the purpose of the study, an emotional functioning domain was used) during their hospital visits. A total of 81 patients took part in the study: 39 before the COVID-19 pandemic (BP) and 42 during the COVID-19 pandemic (DP). Patients’ medians were lower for the BDI, GHQ-12 and higher for the emotional domain of CFQ-R during the pandemic (3, 6, 75 vs. 4, 10, 73.33). Fewer patients felt that their mental health had deteriorated during the pandemic (Δχ2 = 7.723; p = 0.005), and GHQ-12 scores were lower in the DP group (Z = −3.044; p = 0.002). No significant differences were found between groups in terms of experiencing depressive symptoms (Δχ2 = 1.036; p = 0.309). It was found that patients with cystic fibrosis from our study group not only maintained but also improved their mental health state during the COVID-19 pandemic.

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“…Cystic fibrosis (CF) is a life‐limiting, autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The clinical characteristics of CF mainly include exocrine pancreatic insufficiency leading to malabsorption and malnutrition, progressive obstructive lung disease, and liver and pancreatic dysfunction 1 . Recently, the incidence of CF was found as 2.9 per 10,000 live births in Central Anatolia 2 .…”

Section: Introductionmentioning

confidence: 99%

Cystic fibrosis newborn screening: Five‐year experience from a tertiary care center

Büyükşahin

Emiralioğlu

Özsezen

et al. 2021

Pediatric Pulmonology

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Background Newborn screening (NBS) for cystic fibrosis (CF) was implemented in our country on January 1, 2015, based on immunoreactive trypsinogen tests (IRT/IRT). Here, we aimed to evaluate the diagnoses of patients and follow‐up process within the first 5 years of NBS from a tertiary care center. Methods This retrospective cohort study was conducted on patients who were admitted to our pediatric pulmonology department for sweat test (ST) via NBS. Patients with CF with negative NBS results and those with CF with positive NBS and joined our follow‐up were also investigated. Clinical outcome measures were compared between patients with CF with positive and negative NBS. Results Six hundred sixty infants who were referred for ST via NBS were included. Across the entire study population (n = 683), 11.4% of patients had CF (14.1% of had negative NBS in this CF group). The sensitivity of NBS was found as 84.9% and the positive predictive value (PPV) was 9.4%. The median age at diagnosis was older (p < 0.001), reluctance for feeding and Pseudobartter syndrome (PBS) were significantly higher at presentation in the negative NBS group. There was no statistically significant difference between the groups regarding weight‐for‐age (p = 0.899) and height‐for‐age (p = 0.491) in the first 2 years' follow‐ups. Conclusions Our findings showed the low sensitivity and PPV of NBS; therefore, further studies based on all patients in our country are necessary for new cut‐off values. PBS and reluctance for feeding should be alarm symptoms for CF even if the infants had negative NBS. Additionally, later diagnosis of patients who had negative NBS did not affect the nutritional outcomes; we need large‐scale prospective studies to optimize nutritional benefits for all infants diagnosed via NBS.

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Cystic Fibrosis

Cited by 76 publications

References 72 publications

Effect of Protein Intake on Weight Gain and Growth in Cystic Fibrosis Patients

Effect of Protein Intake on Weight Gain and Growth in Cystic Fibrosis Patients

Mental Health of Cystic Fibrosis Patients and the COVID-19 Pandemic in Poland: A Single-Center Comparative Study

Cystic fibrosis newborn screening: Five‐year experience from a tertiary care center

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