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Time to change course and tackle CF related obesity

Published:August 12, 2022DOI:https://doi.org/10.1016/j.jcf.2022.08.004
      Poor nutrition in people with Cystic Fibrosis (CF) is associated with poor clinical outcomes and early intervention is essential for normal growth, development and to ensure clinical stability. Over the past few decades, the nutritional status and survival of people with CF (pwCF) has dramatically improved, a far cry from the early descriptions of CF in the 1930s, where malnutrition was a distinctive feature and life expectancy was limited to early childhood [
      • Andersen D.
      Cystic fibrosis of the pancreas and its relation to celiac disease - A clinical and pathologic study.
      ]. Inadvertently, the pendulum has swung the other way, and resulted in an increase prevalence of overweight and obese individuals with CF. These changes have developed over several decades [
      • Peckham D.
      • Whitaker P.
      Drug induced complications; can we do more?.
      ] and been further augmented by the introduction of highly effective CFTR modulators.
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      References

        • Andersen D.
        Cystic fibrosis of the pancreas and its relation to celiac disease - A clinical and pathologic study.
        Am J Dis Child. 1938; 56: 344-399
        • Peckham D.
        • Whitaker P.
        Drug induced complications; can we do more?.
        J Cyst Fibros. 2013; 12: 547-558
        • Hanssens L.S.
        • Duchateau J.
        • Casimir G.J.
        CFTR Protein: not Just a Chloride Channel?.
        Cells. 2021; 10
        • Shapiro B.L.
        Evidence for a mitochondrial lesion in cystic fibrosis.
        Life Sci. 1989; 44: 1327-1334
        • García R.
        • Falduti C.
        • Clauzure M.
        • et al.
        CFTR chloride channel activity modulates the mitochondrial morphology in cultured epithelial cells.
        Int J Biochem Cell Biol. 2021; 135105976
        • Braccia C.
        • Christopher J.A.
        • Crook O.M.
        • et al.
        CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium.
        Cells. 2022; 11
        • Lukasiak A.
        • Zajac M.
        The Distribution and Role of the CFTR Protein in the Intracellular Compartments.
        Membranes (Basel). 2021; 11
        • Jarosz-Griffiths H.H.
        • Scambler T.
        • Wong C.H.
        • et al.
        Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis.
        Elife. 2020; 9
        • Scambler T.
        • Jarosz-Griffiths H.H.
        • Lara-Reyna S.
        • et al.
        ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in cystic fibrosis.
        Elife. 2019; 8: e49248
        • Gabillard-Lefort C.
        • Casey M.
        • Glasgow A.M.A.
        • et al.
        Trikafta Rescues CFTR and Lowers Monocyte P2×7R-induced Inflammasome Activation in Cystic Fibrosis.
        Am J Respir Crit Care Med. 2022; 205: 783-794
        • Peckham D.
        • Holland E.
        • Range S.
        • Knox A.J.
        Na+/K+ ATPase in lower airway epithelium from cystic fibrosis and non-cystic-fibrosis lung.
        Biochem Biophys Res Commun. 1997; 232: 464-468
        • Dellschaft N.S.
        • Ng C.
        • Hoad C.
        • et al.
        Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls.
        J Cyst Fibros. 2022; 21: 502-505
        • Li L.
        • Somerset S.
        Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy.
        Dig Liver Dis. 2014; 46: 865-874
        • Enaud R.
        • Hooks K.B.
        • Barre A.
        • et al.
        Intestinal Inflammation in Children with Cystic Fibrosis Is Associated with Crohn's-Like Microbiota Disturbances.
        J Clin Med. 2019; 8
        • Culhane S.
        • George C.
        • Pearo B.
        • Spoede E.
        Malnutrition in cystic fibrosis: a review.
        Nutr Clin Pract. 2013; 28: 676-683
        • Stephenson A.L.
        • Mannik L.A.
        • Walsh S.
        • et al.
        Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study.
        Am J Clin Nutr. 2013; 97: 872-877
        • Liou T.G.
        • Adler F.R.
        • Fitzsimmons S.C.
        • Cahill B.C.
        • Hibbs J.R.
        • Marshall B.C.
        Predictive 5-year survivorship model of cystic fibrosis.
        Am J Epidemiol. 2001; 153: 345-352
        • Stallings V.A.
        • Stark L.J.
        • Robinson K.A.
        • et al.
        Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review.
        J Am Diet Assoc. 2008; 108: 832-839
        • Corey M.
        • McLaughlin F.J.
        • Williams M.
        • Levison H.
        A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto.
        J Clin Epidemiol. 1988; 41: 583-591
        • Yen E.H.
        • Quinton H.
        • Borowitz D.
        Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis.
        J Pediatr. 2013; 162 (e1): 530-535
        • White H.
        • Pollard K.
        • Etherington C.
        • et al.
        Nutritional decline in cystic fibrosis related diabetes: the effect of intensive nutritional intervention.
        J Cyst Fibros. 2009; 8: 179-185
        • Bellissimo M.P.
        • Zhang I.
        • Ivie E.A.
        • et al.
        Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis.
        J Cyst Fibros. 2019; 18: 430-435
        • Scully K.J.
        • Jay L.T.
        • Freedman S.
        • et al.
        The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis.
        Nutrients. 2022; 14
        • McBennett K.A.
        • Davis P.B.
        • Konstan M.W.
        Increasing life expectancy in cystic fibrosis: advances and challenges.
        Pediatr Pulmonol. 2022; 57 (Suppl): S5-S12
        • Szentpetery S.
        • Fernandez G.S.
        • Schechter M.S.
        • Jain R.
        • Flume P.A.
        • Fink A.K.
        Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.
        J Cyst Fibros. 2022;
        • Petersen M.C.
        • Begnel L.
        • Wallendorf M.
        • Litvin M.
        Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis.
        J Cyst Fibros. 2022; 21: 265-271
        • Bailey J.
        • Krick S.
        • Fontaine K.R.
        The Changing Landscape of Nutrition in Cystic Fibrosis: the Emergence of Overweight and Obesity.
        Nutrients. 2022; 14
        • Madde A.
        • Okoniewski W.
        • Sanders D.B.
        • Ren C.L.
        • Weiner D.J.
        • Forno E.
        Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis.
        J Cyst Fibros. 2021;
        • Lean M.E.
        • Han T.S.
        • Seidell J.C.
        Impairment of health and quality of life in people with large waist circumference.
        Lancet. 1998; 351: 853-856
        • Ramsey B.W.
        • Davies J.
        • McElvaney N.G.
        • et al.
        A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
        N Engl J Med. 2011; 365: 1663-1672
        • Sutharsan S.
        • McKone E.F.
        • Downey D.G.
        • et al.
        Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial.
        Lancet Respir Med. 2022; 10: 267-277
        • Middleton P.G.
        • Mall M.A.
        • Dřevínek P.
        • et al.
        Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
        N Engl J Med. 2019; 381: 1809-1819
        • Heijerman H.G.M.
        • McKone E.F.
        • Downey D.G.
        • et al.
        Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.
        Lancet. 2019; 394: 1940-1948
        • Taylor-Cousar J.L.
        • Munck A.
        • McKone E.F.
        • et al.
        Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.
        N Engl J Med. 2017; 377: 2013-2023
        • Nichols A.L.
        • Davies J.C.
        • Jones D.
        • Carr S.B.
        Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor.
        Paediatr Respir Rev. 2020; 35: 99-102
        • Munce D.
        • Lim M.
        • Akong K.
        Persistent recovery of pancreatic function in patients with cystic fibrosis after ivacaftor.
        Pediatr Pulmonol. 2020; 55: 3381-3383
        • Bellin M.D.
        • Laguna T.
        • Leschyshyn J.
        • et al.
        Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study.
        Pediatr Diabetes. 2013; 14: 417-421
        • Gramegna A.
        • De Petro C.
        • Leonardi G.
        • et al.
        Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: a case series.
        J Cyst Fibros. 2022;
        • Crozier D.N.
        Cystic fibrosis: a not-so-fatal disease.
        Pediatr Clin North Am. 1974; 21: 935-950
        • Maisonneuve P.
        • Marshall B.C.
        • Knapp E.A.
        • Lowenfels A.B.
        Cancer risk in cystic fibrosis: a 20-year nationwide study from the United States.
        J Natl Cancer Inst. 2013; 105: 122-129
        • Soltman S.
        • Hicks R.A.
        • Naz Khan F.
        • Kelly A
        Body composition in individuals with cystic fibrosis.
        J Clin Transl Endocrinol. 2021; 26100272
        • Alvarez J.A.
        • Ziegler T.R.
        • Millson E.C.
        • Stecenko A.A.
        Body composition and lung function in cystic fibrosis and their association with adiposity and normal-weight obesity.
        Nutrition. 2016; 32: 447-452
        • Ritchie H.
        • Nahikian-Nelms M.
        • Roberts K.
        • Gemma S.
        • Shaikhkhalil A.
        The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations.
        J Cyst Fibros. 2021; 20: 837-842