Coronary artery disease in patients with cystic fibrosis – A case series and review of the literature

Sandouk, Zahrae; Nachawi, Noura; Simon, Richard H.; Wyckoff, Jennifer; Putman, Melissa S.; Kiel, Sarah C.; Soltman, Sarah; Moran, Antoinette; Moheet, Amir

doi:10.1016/j.jcte.2022.100308

Cited by 11 publications

(10 citation statements)

References 24 publications

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“…91 These metabolic changes raise concern, especially considering a case series of six PwCF with coronary artery disease and acute myocardial infarction requiring stent placement with no noticeable pattern related to known risk factors. 93 diagnosis of CFLD had a longer median length of stay and two times higher mortality rate. 94 The increase in mortality seems to have some gender bias with a 6.4-fold increased risk of death observed in female PwCF with advanced CFLD compared to males in a single-center study analyzing 27 years of patient follow-up in 55 PwCF.…”

Section: Obesitymentioning

confidence: 95%

“…Negative associations were seen with more overweight/obese PwCF having CF‐related liver disease, elevated cholesterol, and systemic hypertension 91 . These metabolic changes raise concern, especially considering a case series of six PwCF with coronary artery disease and acute myocardial infarction requiring stent placement with no noticeable pattern related to known risk factors 93 …”

Section: Gastrointestinalmentioning

confidence: 99%

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Cystic fibrosis year in review 2022

Kim,

Lyman,

Savant

2023

Pediatric Pulmonology

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Remarkable medical advancements have been made for people with cystic fibrosis (CF) in recent years, with an abundance of research continuing to be conducted worldwide. With concern for limitations in access to highly effective CFTR modulators, as well as the recent Coronavirus Disease‐19 pandemic, there has been a consistent effort to understand and improve CF screening, disease burden, diagnosis, and management. Our aim in this review is to present articles from 2022 with an emphasis on clinically relevant studies. We hope this will serve as a broad overview of the research published in the past year.

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Section: Obesitymentioning

confidence: 95%

Section: Gastrointestinalmentioning

confidence: 99%

Cystic fibrosis year in review 2022

Kim,

Lyman,

Savant

2023

Pediatric Pulmonology

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“…However, recent studies demonstrated increased arterial stiffness, subclinical atherosclerosis, and gradual worsening of endothelial function in pwCF 134,135 . Several cases of myocardial infarction have been reported in pwCF with and without CFRD 136,137 …”

Section: Pathophysiology Of the Cf Gi Tractmentioning

confidence: 99%

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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“…However, isolated cases of coronary artery disease or acute myocardial infarction have been previously described, especially in individuals over 45 [ 48 , 49 ]. Sandouk et al published a mini case series of six patients with CFRD that developed coronary artery disease (non-ST segment elevation myocardial ischemia): two patients were smokers, four were hypertensive, and five patients were not receiving aspirin or statins prior to this event [ 50 ].…”

Section: Cardiovascular Disease In Patients With Cystic Fibrosismentioning

confidence: 99%

Can Bioactive Food Substances Contribute to Cystic Fibrosis-Related Cardiovascular Disease Prevention?

Trandafir

Frăsinariu²,

Ţarcă³

et al. 2023

Nutrients

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Advances in cystic fibrosis (CF) care have significantly improved the quality of life and life expectancy of patients. Nutritional therapy based on a high-calorie, high-fat diet, antibiotics, as well as new therapies focused on CFTR modulators change the natural course of the disease. They do so by improving pulmonary function and growing BMI. However, the increased weight of such patients can lead to unwanted long-term cardiovascular effects. People with CF (pwCF) experience several cardiovascular risk factors. Such factors include a high-fat diet and increased dietary intake, altered lipid metabolism, a decrease in the level of fat-soluble antioxidants, heightened systemic inflammation, therapeutic interventions, and diabetes mellitus. PwCF must pay special attention to food and eating habits in order to maintain a nutritional status that is as close as possible to the proper physiological one. They also have to benefit from appropriate nutritional counseling, which is essential in the evolution and prognosis of the disease. Growing evidence collected in the last years shows that many bioactive food components, such as phytochemicals, polyunsaturated fatty acids, and antioxidants have favorable effects in the management of CF. An important positive effect is cardiovascular prevention. The possibility of preventing/reducing cardiovascular risk in CF patients enhances both quality of life and life expectancy in the long run.

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Coronary artery disease in patients with cystic fibrosis – A case series and review of the literature

Cited by 11 publications

References 24 publications

Cystic fibrosis year in review 2022

Cystic fibrosis year in review 2022

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

Can Bioactive Food Substances Contribute to Cystic Fibrosis-Related Cardiovascular Disease Prevention?

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