ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis

Wilschanski, Michael; Munck, Anne; Carrion, Estefania; Cipolli, Marco; Collins, Sarah; Colombo, Carla; Declercq, Dimitri; Hatziagorou, Elpis; Hulst, Jessie; Kalnins, Daina; Katsagoni, Christina N.; Mainz, Jochen G.; Ribes-Koninckx, Carmen; Smith, Chris; Smith, Thomas; Van Biervliet, Stephanie; Chourdakis, Michael

doi:10.1016/j.clnu.2023.12.017

Cited by 15 publications

(3 citation statements)

References 338 publications

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“…In addition, 35% had insufficient Vit-D levels. Vit-D insufficiency is still a problem in CF patients, even in those receiving supplementations [45]. More than half of our series (53%) had hypovitaminosis D. These results are not surprising because Gupta et al, in 2017, reported that 71% of 52 CF patients (6-18 years) had serum Vit-D levels <15 ng/mL [46].…”

Section: Vitamin Dsupporting

confidence: 48%

“…As mentioned in the literature review, the CFF and the ESPEN-ESPGHAN-ECFS guideline has issued instructions for the treatment of Vit-D deficiency, targeting serum levels of at least 30 ng/mL [18,45]. In our series, no patient had serum vitamin levels of 30 ng/mL.…”

Section: Vitamin Dmentioning

confidence: 68%

“…This inconsistency may be because Vit-D deficiency would affect the intestinal absorption of Ca [68]. The ESPGHAN recommends a periodic annual assessment of Ca intake, as well as encouraging greater consumption of foods rich in Ca in patients with a suboptimal intake [7,45]. Moreover, the European and French guidelines recommend annual monitoring of calciuria [69].…”

Section: Calciummentioning

confidence: 99%

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Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study

Escobedo-Monge,

Marcos-Temprano,

Parodi-Román

et al. 2024

IJMS

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Cystic fibrosis (CF) is a monogenic disease with different types of mutations that mainly affect the respiratory-digestive system. Calcium (Ca), phosphorus (P), and vitamin D (Vit-D) are essential nutrients for maintaining adequate growth and development, as well as key components in crucial metabolic pathways. Proper diagnosis, treatment, and response are decisive components of precision medicine. Therefore, we conducted a cross-sectional study to evaluate Ca, P, and Vit-D levels along with health and nutritional indicators, regarding their non-skeletal functions, in a series of CF patients. Anthropometric and clinical evaluation, biochemical analysis, dietary survey, and respiratory and pancreatic status were performed. Even though the results showed that all patients had normal dietary and serum Ca levels, 47% of patients had deficient Vit-D intake, 53% of patients had hypovitaminosis D, 35% had insufficient Vit-D levels, 18% had hypophosphatemia, 76% had elevated alkaline phosphate levels, 29% had hypercalciuria, and 65% had hyperphosphaturia. There were no significant differences between homozygous and compound heterozygous patients. Ca, P, and Vit-D levels were associated with body mass index; body composition; physical activity; diet; growth hormones; and the immune, liver, and kidney systems. We suggest a periodically evaluation of Ca and P losses.

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Section: Vitamin Dsupporting

confidence: 48%

Section: Vitamin Dmentioning

confidence: 68%

Section: Calciummentioning

confidence: 99%

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Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study

Escobedo-Monge,

Marcos-Temprano,

Parodi-Román

et al. 2024

IJMS

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Codevelopment of a model of care for adults living with cystic fibrosis‐related diabetes

Kumar,

Pallin,

Soldatos

2024

Pediatric Pulmonology

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BackgroundCystic fibrosis (CF) related diabetes affects up to half of all adults with CF and is associated with higher morbidity and mortality. Our aim is to codevelop an ideal model of care that integrates diabetes technology and better meets the needs of adults living with the condition to improve attendance, engagement, service satisfaction, and clinical outcomes.MethodsUsing qualitative research methods, we evaluated disease perceptions, barriers, and enablers to optimal CF‐related diabetes management and service delivery. Integration of continuous glucose monitoring (CGM) was also explored. An initial broad purposive consumer survey was followed by focus groups with end‐users. Grounded theory approach was utilized with major problem areas identified then explored, coded, and grouped into requisites for an “ideal model of care” for adults living with CF‐related diabetes.ResultsTwo key themes emerged (i) an ideal model of care consisted of a dual‐specialty service co‐led by endocrinology and CF physicians and supported by diabetes educator and CF dietitian with a goal to provide consistent and personalized diabetes management and (ii) CGM was acceptable for use in adults with CF‐related diabetes with many perceived benefits and should be integrated into the model of care. Barriers to optimizing glycemic control included diet, finger‐prick testing, reduced access to CGM, and pulmonary exacerbations. End‐user feedback on CGM was overwhelmingly positive with regard to operability. CGM was also identified as a tool that could be used to engage, educate, and empower adults living with CF‐related diabetes and facilitate constructive and personalized clinical decision‐making by healthcare providers.ConclusionFor adults living with CF, a diagnosis of diabetes is associated with increased treatment burden. Our findings suggest an “ideal model of care” for CF‐related diabetes would be co‐led by endocrinology services integrated within a pre‐existing CF service, incorporating CGM.

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Effects on growth, weight and body composition after CFTR modulators in children with cystic fibrosis

López Cárdenes,

Merino Sánchez‐Cañete,

Vicente Santamaría

et al. 2024

Pediatric Pulmonology

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BackgroundCystic Fibrosis (CF) is associated with compromised nutrition status, which is responsible for morbidity and mortality along with lung function decline. This study was designed to examine changes in anthropometric markers and body composition parameters by bioelectrical impedance analysis after CFTR modulator (CFTRm) treatment.MethodsWe compared anthropometric parameters and body composition before and after 6 and 12 months of CFTRm treatment. Results are stratified into subgroups according to the modulator used with dual therapy with lumacaftor + ivacaftor or tezacaftor + ivacaftor (LUMA/TEZ + IVA) or triple therapy with elexacaftor + tezacaftor + ivacaftor (ELE + TEZ + IVA). Body composition data are available in patients treated with ELE + TEZ + IVA.ResultsTwo hundred and thirty‐four children (55.1% male) were recruited. The median age was 13.6 years (inter‐quartile range [IQR] 10.7–16.1). We can observe a statistically significant increase in the weight Z score and BMI Z score after CFTRm. In terms of changes in body composition, we observe a significant increase in fat mass (FM) expressed both in kilograms and as a percentage at 6 months (p < .05; Wilcoxon‐test), with no such differences found at 12 months. We also observe a statistically significant increase in fat‐free‐mass (FFM), expressed in kilograms at 6 and 12 months (p < .05; Wilcoxon‐test).ConclusionWeight status improved and changes in body composition occurred in children after CFTRm therapy, including an increase of fat mass. Further studies are needed to confirm these changes in body composition and their impact on disease progression.

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ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis

Cited by 15 publications

References 338 publications

Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study

Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study

Codevelopment of a model of care for adults living with cystic fibrosis‐related diabetes

Effects on growth, weight and body composition after CFTR modulators in children with cystic fibrosis

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