Nutrition in cystic fibrosis: From the past to the present and into the future

Altman, Kimberly; McDonald, Catherine M.; Michel, Suzanne H.; Maguiness, Karen

doi:10.1002/ppul.24521

Cited by 15 publications

(33 citation statements)

References 107 publications

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“…13 Significant associations were identified between nutritional status and PFTs in some studies. 12 In a multicenter study conducted by Zemel et al,14 z-scores of weight and height of people with CF were positively associated with PFTs, and it was found that growth and nutritional status of people were associated with changes in FEV 1 . Thus, it was suggested that nutritional interventions might slow the impaired pulmonary function in CF.…”

Section: Discussionmentioning

confidence: 97%

“…Management of nutrition, which is a risk factor for morbidity and mortality is important in people with CF as lung function, pulmonary muscle function, and exercise tolerance are associated with nutritional status 11,12 . Although many physicians consider malnutrition as a result of lung disease, it is a contributing factor to lung disease 12 . In some studies, nutritional status has been associated with survival for people with CF, and stabilization has been observed in malnourished people when their nutritional status improves 12 …”

Section: Discussionmentioning

confidence: 99%

“…12 In some studies, nutritional status has been associated with survival for people with CF, and stabilization has been observed in malnourished people when their nutritional status improves. 12 In the ECFSPR, evaluating by age groups, the median z-scores for weight and height in 2017 ranged from −1.3 to −1.0 and from −1.1 to −0.9, respectively. 8 In the CFRT, evaluating by age groups in 2017, the median z-scores of weights, height, and BMI of all patients ranged from −1.2 to −0.6, −1.4 to −0.6, and −0.8 to −0.3, respectively.…”

Section: Discussionmentioning

confidence: 99%

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The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care

Asfuroglu

Eyüboğlu

Aslan

et al. 2022

Pediatric Pulmonology

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Background: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease.Methods: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care

Asfuroglu

Eyüboğlu

Aslan

et al. 2022

Pediatric Pulmonology

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“…Several additional studies also reported associations between imbalanced iron status and other inflammatory lung diseases. Thus, there is evidence that iron deficiency increases airway inflammation and leads to worse lung function in patients with lung cancer, 33 chronic obstructive pulmonary disease, 34 thalassemia, 35 and cystic fibrosis 36 . However, studies of the association of iron deficiency with childhood airway dysfunction have yielded disparate results.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, there is evidence that iron deficiency increases airway inflammation and leads to worse lung function in patients with lung cancer, 33 chronic obstructive pulmonary disease, 34 thalassemia, 35 and cystic fibrosis. 36 However, studies of the association of iron deficiency with childhood airway dysfunction have yielded disparate results. A previous study of adults found that impaired iron stores were associated with worse lung function as indicated by spirometry, but none of the indices of iron were associated with FeNO.…”

Section: Discussionmentioning

confidence: 99%

Abnormal iron status is independently associated with reduced oscillometric lung function in schoolchildren

Kim

Lee

et al. 2021

Clinical Respiratory J

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Background Associations between anemia and allergic diseases have been reported, but the relationship of iron deficiency with airway dysfunction in children remains unclear. We aimed to investigate the relationship between abnormal iron parameters and lung function in schoolchildren. Methods Four hundred and forty‐five children (10–12 years‐old) from 11 elementary schools in were enrolled. The relationships of different iron parameters (hemoglobin, serum iron, transferrin saturation, and serum ferritin) with lung function evaluated by impulse oscillometry (airways resistance at 5 Hz [Rrs5], 10 Hz [Rrs10], and the difference of Rrs5 and Rrs20 Hz [Rrs5‐20]), and with exhaled nitric oxide (FeNO) were evaluated after adjustment for confounders including height, sex, and body mass index z‐score, and for additional covariates that could affect airway function. Results Total airway dysfunction represented by Rrs5 was reduced in participants with low serum iron level (aβ: −0.13, 95% CI: −0.23 to −0.03, p = 0.040) after adjustment for key confounders, but did not correlate with other iron profiles. Reduced oscillometric lung function recorded as Rrs5‐20 was related with low serum iron and high serum ferritin, but the results were inconsistent after multiple comparisons. Associations were not observed with serum hemoglobin. Conclusions Decreased serum iron level was related with airway dysfunction represented as oscillomteric Rrs5. Our results suggest a relationship of reduced lung function with abnormal iron status in children.

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Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

McDonald,

Reid,

Pohl

et al. 2024

Nut in Clin Prac

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Cystic fibrosis (CF) is a progressive, genetic, multi‐organ disease affecting the respiratory, digestive, endocrine, and reproductive systems. CF can affect any aspect of the gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, colon, pancreas, liver, and gall bladder. GI pathophysiology associated with CF results from CF membrane conductance regulator (CFTR) dysfunction. The majority of people with CF (pwCF) experience exocrine pancreatic insufficiency resulting in malabsorption of nutrients and malnutrition. Additionally, other factors can cause or worsen fat malabsorption, including the potential for short gut syndrome with a history of meconium ileus, hepatobiliary diseases, and disrupted intraluminal factors, such as inadequate bile salts, abnormal pH, intestinal microbiome changes, and small intestinal bacterial overgrowth. Signs and symptoms associated with fat malabsorption, such as abdominal pain, bloating, malodorous flatus, gastroesophageal reflux, nausea, anorexia, steatorrhea, constipation, and distal intestinal obstruction syndrome, are seen in pwCF despite the use of pancreatic enzyme replacement therapy. Given the association of poor nutrition status with lung function decline and increased mortality, aggressive nutrition support is essential in CF care to optimize growth in children and to achieve and maintain a healthy body mass index in adults. The introduction of highly effective CFTR modulator therapy and other advances in CF care have profoundly changed the course of CF management. However, GI symptoms in some pwCF may persist. The use of current knowledge of the pathophysiology of the CF GI tract as well as appropriate, individualized management of GI symptoms continue to be integral components of care for pwCF.

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Nutrition in cystic fibrosis: From the past to the present and into the future

Abstract: Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition care.

Cited by 15 publications

References 107 publications

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care

Abnormal iron status is independently associated with reduced oscillometric lung function in schoolchildren

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

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