Body composition in individuals with cystic fibrosis

Soltman, Sarah; Hicks, Rebecca; Khan, Farah; Kelly, Andrea

doi:10.1016/j.jcte.2021.100272

Cited by 10 publications

(16 citation statements)

References 45 publications

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“…In adults, fat‐free mass (FFM) was found to correlate negatively with CF disease severity, inflammatory state, and pulmonary function. FFM was also found to correlate with better BMD, while greater FM was associated with greater loss of spine BMD; in youth, LBM was associated with pulmonary function 26 . Additionally, patients with CFRD were found to have a lower FMI z‐score, weight z‐score and leptin levels compared to the control group 27 …”

Section: Discussionmentioning

confidence: 95%

Effect of Trikafta on bone density, body composition and exercise capacity in CF: A pilot study

Gur

Bar‐Yoseph

Hanna

et al. 2022

Pediatric Pulmonology

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Background While the positive effect of Trikafta on cystic fibrosis (CF) pulmonary disease is well established, there is limited data about its effect on bone mineral density (BMD), body composition and exercise capacity. Methods A pilot single center study. BMD and body composition were measured three months after the initiation of Trikafta (study group) and compared to values obtained 2 years earlier. CF patients not treated with Trikafta, for whom BMD was measured 2 years apart, served as controls. Spirometry, lung clearance index (LCI), sweat test, six‐min walk test (6MWT) and cardio‐pulmonary exercise test (CPET) were performed before and three months after the initiation of Trikafta. Results Nine study patients, aged 18.6 ± 4.7 years, and nine controls. For the study group, BMI and hip and spine BMD increased significantly (19.4 ± 2.6 to 20.3 ± 2.19 BMI, p = 0.05; 0.73 ± 0.098 to 0.81 ± 0.12 gr/cm2 hip, p = 0.017; 0.76 ± 0.14 to 0.82 ± 0.14 gr/cm2 spine, p = 0.025). For the control group, there was no difference in hip or spine BMD. Lean body mass, %fat z‐score and fat mass/height2 z‐score increased significantly (34770.23 ± 10521.21 to 37430.16 ± 10330.09gr, p = 0.017; –0.8 ± 0.75 to 0.46 ± 0.58, p = 0.012; and −0.98 ± 0.66 to −0.04 ± 0.51, p = 0.025, respectively). 6MWT improved from 541.1 ± 48.9 to 592.9 ± 54.5 m (p = 0.046). As expected, FEV1%pred increased (p = 0.008) and sweat chloride decreased significantly (p = 0.017). In CPET, VE/VCO2 improved, indicating better ventilatory efficiency. Conclusions To the best of our knowledge, this is the first study evaluating the metabolic effects of Trikafta. The results are encouraging and offer hope beyond the well‐established effect on pulmonary disease. Larger long‐term studies are warranted to unpin the underlying physiological mechanisms.

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Section: Discussionmentioning

confidence: 95%

Effect of Trikafta on bone density, body composition and exercise capacity in CF: A pilot study

Gur

Bar‐Yoseph

Hanna

et al. 2022

Pediatric Pulmonology

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“…Furthermore, serum KISS levels were significantly higher in obese men as well as in obese/overweight girls compared to the normal weight subjects 31 , 32 . Maldigestion and malabsorption, especially fat and fat-soluble vitamins, poor appetite, and increased energy consumption contribute to the frequent occurrence of malnutrition in the population with CF 33 . Thus, decreased KISS levels may be associated with lower body weight in patients with CF.…”

Section: Discussionmentioning

confidence: 99%

Serum kisspeptin and proopiomelanocortin in cystic fibrosis: a single study

Galiniak

Podgórski

Rachel

et al. 2022

Sci Rep

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The determination of hormonal biomarkers is of increasing interest in many diseases, including cystic fibrosis (CF). Hormones that have not been estimated and described so far in CF include kisspeptin (KISS) and proopiomelanocortin (POMC), which are involved in the regulation of many processes, including appetite and fertility. Therefore, the aim of our study was to estimate the level of KISS and POMC in sera from CF patients and to determine the correlation between these hormones and clinical parameters. For this purpose, we estimated the levels of KISS and POMC in 38 CF patients and 16 healthy participants with enzyme-linked immunosorbent assay. We found significantly reduced levels of KISS and POMC in people with CF compared to healthy subjects (1.76 ± 0.46 vs. 2.27 ± 0.56 ng/mL, p < 0.05 and 6.25 ± 4.36 vs. 14.74 ± 6.24 ng/mL, p < 0.001, respectively). Furthermore, the level of both hormones was negatively correlated with age. The hormones studied did not correlate with the results of spirometry and each other. Thus, decreased KISS and POMC levels may be associated with lower body weight and delayed puberty in patients with CF.

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“…BMI is used to evaluate nutritional status, but it has limitations because it could not differentiate fat mass from lean body mass (LBM). Another 2 indicators of nutritional deficiency are more sensitive than BMI: LBM and bone mineral content (BMC), lower values being associated with impaired pulmonary function in children [ 39 , 40 ]. BMI fails to assess body composition, especially fat free mass, an important indicator of nutritional status.…”

Section: Clinical Considerations Of Exocrine Pancreatic Insufficiencymentioning

confidence: 99%

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

et al. 2023

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This review presents current updates of pancreatic enzyme replacement therapy in children with cystic fibrosis based on literature published in the last decade and some special considerations regarding pancreatic enzyme replacement therapy in the era of new therapies, such as cystic fibrosis transmembrane conductance regulator modulator therapies. Few articles evaluate the efficacy of pancreatic enzyme replacement therapy in the pediatric population, and most studies also included children and adults with cystic fibrosis. Approximately 85% of cystic fibrosis patients have exocrine pancreatic insufficiency and need pancreatic enzyme replacement therapy. Fecal elastase is the most commonly used diagnostic test for exocrine pancreatic insufficiency, although this value can fluctuate over time. While it is used as a diagnostic test, it cannot be used for monitoring the effectiveness of pancreatic enzyme replacement therapy and for adjusting doses. Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis. An important key point in cystic fibrosis treatment is the recent hypothesis that cystic fibrosis transmembrane conductance regulator modulators could improve pancreatic function, further studies being essential. Pancreatic enzyme replacement therapy is addressed a complication of the disease (exocrine pancreatic insufficiency), while modulators target the defective cystic fibrosis transmembrane conductance regulator protein. Exocrine pancreatic insufficiency in cystic fibrosis remains an active area of research in this era of cystic fibrosis transmembrane conductance regulator modulator therapies. This new therapy could represent an example of personalized medicine in cystic fibrosis patients, with each class of modulators being addressed to patients with specific genetic mutations.

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Body composition in individuals with cystic fibrosis

Cited by 10 publications

References 45 publications

Effect of Trikafta on bone density, body composition and exercise capacity in CF: A pilot study

Effect of Trikafta on bone density, body composition and exercise capacity in CF: A pilot study

Serum kisspeptin and proopiomelanocortin in cystic fibrosis: a single study

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

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