Pediatric CF is associated with selective alterations in circulating type 3 and regulatory immune responses, characterized by increased IL-22-producing lymphocyte populations, reduced plasma IL-17 A concentrations, and increased numbers of CD3⁺IL-10⁺ cells.
People with aCFLD, renal insufficiency, post-lung transplantation, and pregnancy are prone to vitamin A toxicity, and CF clinicians should be proactive in evaluating these abnormalities and proficient in managing both deficiency and toxicity, as both these conditions can be associated with adverse outcomes.
Senthilkumar Sankararaman, Terri Schindler, Kay Vavrina et al.· Nutrients· 0 citations
A detailed review of elastic fiber biology, the mechanisms by which CF pathophysiology disrupts elastic fiber integrity, the downstream structural and functional consequences, and potential therapeutic avenues targeting elastic fiber preservation or restoration are provided.
This case underscores the importance of recognizing CFTR-RD in patients with multisystem involvement and atypical features of cystic fibrosis, and highlights the value of multidisciplinary evaluation in establishing an accurate diagnosis and developing an individualized treatment strategy.
A.-Y. Li, W.-J. Zhou, Y.-L. Yang et al.· Zhonghua jie he he hu xi za...· 0 citations
Prenatal CFTRm therapy is promising but remains investigational, and care should include multidisciplinary counseling, genetic confirmation of fetal CF, maternal liver function monitoring, fetal ultrasound surveillance, and postnatal follow-up, according to standardized guidelines.
M. E. Norton· Obstetrical & Gynecologi...· 0 citations
CFTR genotype may influence clinical phenotype in CF patients with ABPA, with trends toward poorer growth and lung function in more severe mutation classes, although larger studies are needed to confirm these associations.
M. F. Ali, Hessa AlOtaibi, Talal Alzahrani et al.· Frontiers in Pediatrics· 0 citations
This case highlights an uncommon late-diagnosed CF phenotype in an octogenarian with pancreatic sufficiency, intermediate sweat chloride, chronic sinopulmonary disease, preserved lung function, and new isolation of Pseudomonas aeruginosa.
Brenda M. Hranec, Luke Hudson, Daniel Layish· Cureus· 0 citations
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