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Exocrine pancreatic insufficiency has various causes that differ with age, and diagnosis is challenging due to the lack of reliable diagnostic testing approaches. A high index of suspicion based on clinical history should inform testing.
Malnutrition is common in children with cystic fibrosis, and it contributes to both poor pulmonary and overall disease outcomes. But what are the challenges when trying to optimize nutrition?
A 3-year-old with respiratory problems since birth was found to have multiple congenital anomalies affecting the airway including a posterior laryngeal cleft, a tracheal bronchus, and a very narrow distal trachea.
Many people with cystic fibrosis who do not have genetic variants approved for treatment still respond to treatment with elexacaftor–tezacaftor–ivacaftor.
Children with cystic fibrosis who receive therapy requiring general anesthesia before the age of 2 years do not have impaired neurodevelopment in later childhood.
A 3-year-old with respiratory problems since birth was found to have multiple congenital anomalies affecting the airway including a posterior laryngeal cleft, a tracheal bronchus, and a very narrow distal trachea.
Cystic fibrosis-related diabetes (CFRD) is a unique form of diabetes that shares features with both type 1 and type 2 diabetes and is most often characterised by transient postprandial hyperglycaemia as a consequence of delayed first-phase insulin …
This systematic review evaluates the efficacy and safety of COVID-19 vaccines in individuals with cystic fibrosis (CF). A systematic search of major databases conducted between December 2019 and January 2024 identified eight cohort studies …
Panniculitis is associated with a variety of disease processes that can be challenging to distinguish clinically. While diagnosis often relies on histopathology, sometimes this is not sufficiently diagnostic, which requires careful consideration …
Panniculitis, inflammation of the subcutaneous fat, represents a heterogenous group of diseases that can present clinical challenges for inpatient dermatologists [ 1 ]. Despite numerous forms of panniculitis with diverse etiologies, patients …
Cystic fibrosis (CF) is a chronic genetic disorder characterized by pancreatic insufficiency and lung disease. Advancements in highly effective modulator therapies (HEMTs) have improved life expectancy, shifting the focus to endocrine …
Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in individuals with cystic fibrosis (CF), affecting at least 50% of the adult CF population [ 1 ]. Acquiring CFRD is associated with a ~6-fold increase of morbidity and …
This study explores psychosocial wellbeing in adolescents with a physician-diagnosed chronic condition, with emphasis on the disparity between adolescents who self-report their condition (reporters) and those who do not (non-reporters). We also …
Alpha-1-antitrypsin deficiency (AATD) is a rare genetic disorder leading to chronic obstructive pulmonary disease (COPD). Emphysema is the major structural damage visible on Computed tomography (CT) scans and is believed to occur primarily because …