Evaluation of increased arterial stiffness in pediatric patients with cystic fibrosis by augmentation index and pulse wave velocity analysis

Öztürk, Gökçen Kartal; Çonkar, Seçil; Eşki, Aykut; Gülen, Figen; Keskınoğlu, Ahmet; Demir, Esen

doi:10.1002/ppul.24688

Cited by 11 publications

(6 citation statements)

References 23 publications

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“… 23 Cardiovascular studies in patients with CF reported a lower aortic distensibility and higher aortic stiffness index, in line with the CDACD study findings. 25 , 26 Arterial abnormalities in adolescents with CoA have also been reported. Children with CoA showed a higher carotid and femoral IMT and higher aortic stiffness.…”

Section: Discussionmentioning

confidence: 89%

Preclinical Aortic Atherosclerosis in Adolescents With Chronic Disease

Ververs

Eikendal

Kofink

et al. 2022

JAHA

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Background Adolescents with chronic disease are often exposed to inflammatory, metabolic, and hemodynamic risk factors for early atherosclerosis. Since postmortem studies have shown that atherogenesis starts in the aorta, the CDACD (Cardiovascular Disease in Adolescents with Chronic Disease) study investigated preclinical aortic atherosclerosis in these adolescents. Methods and Results The cross‐sectional CDACD study enrolled 114 adolescents 12 to 18 years old with chronic disorders including juvenile idiopathic arthritis, cystic fibrosis, obesity, corrected coarctation of the aorta, and healthy controls with a corrected atrial septal defect. Cardiovascular magnetic resonance was used to assess aortic pulse wave velocity and aortic wall thickness, as established aortic measures of preclinical atherosclerosis. Cardiovascular magnetic resonance showed a higher aortic pulse wave velocity, which reflects aortic stiffness, and higher aortic wall thickness in all adolescent chronic disease groups, compared with controls ( P <0.05). Age (β=0.253), heart rate (β=0.236), systolic blood pressure (β=−0.264), and diastolic blood pressure (β=0.365) were identified as significant predictors for aortic pulse wave velocity, using multivariable linear regression analysis. Aortic wall thickness was predicted by body mass index (β=0.248) and fasting glucose (β=0.242), next to aortic lumen area (β=0.340). Carotid intima‐media thickness was assessed using ultrasonography, and was only higher in adolescents with coarctation of the aorta, compared with controls ( P <0.001). Conclusions Adolescents with chronic disease showed enhanced aortic stiffness and wall thickness compared with controls. The enhanced aortic pulse wave velocity and aortic wall thickness in adolescents with chronic disease could indicate accelerated atherogenesis. Our findings underscore the importance of the aorta for assessment of early atherosclerosis, and the need for tailored cardiovascular follow‐up of children with chronic disease.

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Section: Discussionmentioning

confidence: 89%

Preclinical Aortic Atherosclerosis in Adolescents With Chronic Disease

Ververs

Eikendal

Kofink

et al. 2022

JAHA

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“…Significant weight gain is seen in pwCF on modulating drug which should be aware of the problem of obesity. Increased risk for intestinal cancer and cardiovascular complications will ask to develop preventive screenings programs and early interventions when pwCF grows older in the near future [61,62,63].…”

Section: Prognosismentioning

confidence: 99%

A new era for people with cystic fibrosis

et al. 2021

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Cystic fibrosis is the most prevalent inherited disease caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The impaired electrolyte homeostasis caused by the mutated or absent protein leads to symptoms in multiple organ systems. However, the pulmonary manifestation with chronic infections and eventually respiratory failure remains the most important threat. Until one decade ago, only symptomatic treatment was available. However, since 2012, different combinations of CFTR modulators are available for people with cystic fibrosis (pwCF) that carry different mutations. The advent of these drugs has impressively changed life expectancy and quality of life in people with cystic fibrosis and raised new challenges regarding long-term complications and tapering of conventional therapies.Conclusion: In this review, we provide an update on the latest developments around diagnostics, treatment, and prognosis of pwCF. What is Known:• Cystic fibrosis is an incurable and life-shortening disease asking for life-long symptomatic treatment.• Three combination CFTR modulating drugs has gained marked approval over the last 10 years. What is New:• The emerge of new (modulating) therapies contribute to the increasing life expectancy.• A high unmet need to develop new therapies for people with CF who cannot access or benefit from these drugs remains. This review gives an update on the current status.

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“…The risk of CVD in pwCF is still considered low because of its historically low prevalence, which is likely related to decreased survival rates. 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%

“…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 Conventional evaluation of lipids in children and adults with CF have revealed changes associated with increased CVD risk, including a significantly lower concentration of high-density lipoprotein cholesterol (HDL-c) and higher triglycerides compared with controls or population means.…”

Section: Essential Fatty Acidsmentioning

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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Evaluation of increased arterial stiffness in pediatric patients with cystic fibrosis by augmentation index and pulse wave velocity analysis

Cited by 11 publications

References 23 publications

Preclinical Aortic Atherosclerosis in Adolescents With Chronic Disease

Preclinical Aortic Atherosclerosis in Adolescents With Chronic Disease

A new era for people with cystic fibrosis

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

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