Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis

Borowitz, Drucy; Robinson, Karen A.; Rosenfeld, Margaret; Davis, Stephanie D.; Sabadosa, Kathryn A.; Spear, Steven J.; Michel, Suzanne H.; Parad, Richard B.; White, Terry B.; Farrell, Philip M.; Marshall, Bruce C.; Accurso, Frank J.

doi:10.1016/j.jpeds.2009.09.001

Cited by 376 publications

(442 citation statements)

References 133 publications

(157 reference statements)

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“…Jeśli karmienie piersią nie zapewnia odpowiedniej podaży składników pokarmowych, należy wzbogacić dietę dziecka o mieszanki dla niemowląt lub dodatek tłuszczu czy węglowodanów. Pokarmy stałe wprowadza się w 4-6 miesiącu życia [37].…”

Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

“…Jeśli ich zawartość w diecie jest niewystarczająca, zalecana jest suplementacja tymi składnikami. Rekomendowana jest również suplementacja chlorkiem sodu (szczególnie w czasie lata), witamin rozpuszczalnych w tłuszczach oraz enzymów trzustkowych [37,38]. U dzieci i młodzieży z CF zapotrzebowanie energe tyczne szybko ulega zmianie.…”

Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

“…Aby zapewnić utrzymanie prawidłowego rozwoju, ważne jest systematyczne monito rowanie podaży kalorii z dietą oraz wdrażanie odpowiednich nawyków żywieniowych. Należy unikać podawania pac jentom produktów niskokalorycznych lub o obniżonej zawartości tłuszczu, a preferować te wysokokaloryczne [37,38].…”

Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

“…Zaleca się, aby podaż energii dla chorych na mukowiscydozę wynosiła 120-150% dziennego zapotrzebowania energetycznego zdrowej osoby. Niektóre zalecenia proponują nawet 110-200% dziennego zapotrzebowania energetycznego [3,37,39].…”

Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

“…W przypadku współwystępowania cukrzycy konieczna jest modyfikacja spożycia węglowodanów [3,[36][37][38][39][40].…”

Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

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Nutritional status disorders in cystic fibrosis – dietary recommendations and supplementation. A review

2017

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Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

“…W przypadku współwystępowania cukrzycy konieczna jest modyfikacja spożycia węglowodanów [3,[36][37][38][39][40].…”

Section: Leczenie Dietetyczne I Zalecenia żYwienioweunclassified

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Nutritional status disorders in cystic fibrosis – dietary recommendations and supplementation. A review

2017

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Cystic Fibrosis

Ong

Ramsey

2023

JAMA

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ImportanceCystic fibrosis, a genetic disorder defined by variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, affects more than 30 000 individuals in the US and approximately 89 000 worldwide. Absent or decreased function of the CFTR protein is associated with multiorgan dysfunction and shortened life expectancy.ObservationsCFTR is an anion channel in the apical membrane of epithelial cells. Loss of function leads to obstructed exocrine glands. Of people with cystic fibrosis in the US, approximately 85.5% have the gene variant F508del. Manifestations of cystic fibrosis in patients with the F508del gene variant begin in infancy with steatorrhea, poor weight gain, and respiratory symptoms (coughing, wheezing). As people with cystic fibrosis age, chronic respiratory bacterial infections cause loss of lung function and bronchiectasis. With the availability of universal newborn screening in multiple countries including the US, many people with cystic fibrosis are asymptomatic at diagnosis. With multidisciplinary care teams that included dietitians, respiratory therapists, and social workers, treatment of cystic fibrosis can slow disease progression. Median survival has improved from 36.3 years (95% CI, 35.1-37.9) in 2006 to 53.1 years (95% CI, 51.6-54.7) in 2021. Pulmonary therapies for patients with cystic fibrosis consist of mucolytics (eg, dornase alfa), anti-inflammatories (eg, azithromycin), and antibiotics (such as tobramycin delivered by a nebulizer). Four small molecular therapies, termed CFTR modulators, that facilitate CFTR production and/or function have received regulatory approval. Examples are ivacaftor and elexacaftor-tezacaftor-ivacaftor. For example, in patients with 1 F508del variant, the combination of ivacaftor, tezacaftor, and elexacaftor improved lung function from −0.2% in the placebo group to 13.6% (difference, 13.8%; 95% CI, 12.1%-15.4%) and decreased the annualized estimated rate of pulmonary exacerbations from 0.98 to 0.37 (rate ratio, 0.37; 95% CI, 0.25-0.55). Improved respiratory function and symptoms have lasted up to 144 weeks in postapproval observational studies. An additional 177 variants are eligible for treatment with the elexacaftor-tezacaftor-ivacaftor combination.ConclusionCystic fibrosis affects approximately 89 000 people worldwide and is associated with a spectrum of disease related to exocrine dysfunction, including chronic respiratory bacterial infections and reduced life expectancy. First-line pulmonary therapies consist of mucolytics, anti-inflammatories, and antibiotics, and approximately 90% of people with cystic fibrosis who are 2 years or older may benefit from a combination of ivacaftor, tezacaftor, and elexacaftor.

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Behavioral and Nutritional Treatment for Preschool-Aged Children With Cystic Fibrosis

et al. 2015

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IMPORTANCE Evidence-based treatments that achieve optimal energy intake and improve growth in preschool-aged children with cystic fibrosis (CF) are a critical need. OBJECTIVE To test whether behavioral and nutritional treatment (intervention) was superior to an education and attention control treatment in increasing energy intake, weight z (WAZ) score, and height z (HAZ) score. DESIGN, SETTING, AND PARTICIPANTS This randomized clinical trial included 78 children aged 2 to 6 years (mean age, 3.8 years) with CF and pancreatic insufficiency (intervention, n = 36 and control, n = 42). The study was conducted at 7 CF centers between January 2006 and November 2012; all 78 participants who met intent-to-treat criteria completed through follow-up. INTERVENTIONS Behavioral intervention combined individualized nutritional counseling targeting increased energy intake and training in behavioral child management skills. The control arm provided education and served as a behavioral placebo controlling for attention and contact frequency. Both treatments were delivered in person or telehealth (via telephone). Sessions occurred weekly for 8 weeks then monthly for 4 months (6 months). Participants then returned to standard care for 1 year, with 12-month follow-up thereafter. MAIN OUTCOMES AND MEASURES Changes in energy intake and WAZ score were examined from pretreatment to posttreatment (6 months) and change in HAZ score was assessed pretreatment to follow-up (18 months). Covariates included sex, Pseudomonas aeruginosa status at baseline, and treatment modality (in person vs telehealth). RESULTS At baseline, mean (SD) energy intake was 1462 (329) kcals/d, WAZ score was −0.44 (0.81), and HAZ score was −0.55 (0.84). From pretreatment to posttreatment, the intervention increased daily energy intake by 485 calories vs 58 calories for the control group (adjusted difference, 431 calories; 95% CI, 282 to 581; P < .001) and increased the WAZ score by 0.12 units vs 0.06 for the control (adjusted difference, 0.09; 95% CI, −0.06 to 0.24; P = .25). From pretreatment to follow-up, the intervention increased the HAZ score by 0.09 units vs −0.02 for the control (adjusted difference, 0.14 units; 95% CI, 0.001 to 0.27; P = .049). Measured treatment integrity and credibility were high for both groups. CONCLUSIONS AND RELEVANCE Behavioral and nutritional intervention improved energy intake and HAZ score outcomes but not WAZ score outcomes. Our results provide evidence that behavioral and nutritional treatment may be efficacious as a nutritional intervention for preschoolers aged 2 to 6 years with CF and pancreatic insufficiency. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00241969

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Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis

Cited by 376 publications

References 133 publications

Nutritional status disorders in cystic fibrosis – dietary recommendations and supplementation. A review

Nutritional status disorders in cystic fibrosis – dietary recommendations and supplementation. A review

Cystic Fibrosis

Behavioral and Nutritional Treatment for Preschool-Aged Children With Cystic Fibrosis

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