Auxological and Endocrinological Features in Children and Adolescents with Cystic Fibrosis.
Vittorio FerrariVito TerlizziStefano StagiPublished in: Journal of clinical medicine (2022)
Cystic fibrosis (CF) is a multisystem autosomal recessive disease caused by mutations that lead to deficient or dysfunctional CF transmembrane conductance regulator ( CFTR ) proteins. Patients typically present malnutrition resulting from the malabsorption of fundamental nutrients and recurring lung infections, with a progressive worsening of the respiratory function. For these reasons, the clinical management of CF requires a multidisciplinary team. From an endocrinological point of view, patients often present major complications, such as diabetes, bone disease, thyroid disorders, delayed growth and puberty, hypogonadism and infertility, which negatively affect their quality of life and, in some cases, significantly reduce life expectancy. These complications can arise as a direct result of CFTR dysfunction and/or as a consequence of a deterioration in the function of the organs affected. The objective of this review is to analyze all the possible endocrinological complications that can occur in patients with CF by evaluating the most recent papers in the literature.
Keyphrases
- cystic fibrosis
- end stage renal disease
- pseudomonas aeruginosa
- ejection fraction
- lung function
- newly diagnosed
- chronic kidney disease
- type diabetes
- peritoneal dialysis
- risk factors
- cardiovascular disease
- prognostic factors
- multiple sclerosis
- systematic review
- quality improvement
- oxidative stress
- metabolic syndrome
- skeletal muscle
- heavy metals
- patient reported outcomes
- soft tissue
- bone loss
- wild type