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Cystic Fibrosis is a topic covered in the Pediatric Surgery NaT.

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Cystic fibrosis (CF) is an autosomal recessive genetic syndrome that results in a mucoviscidosis that affects the lungs, pancreas, and other systems in the body and remains the most common lethal genetic disease in the Caucasian population [1]. Modern prenatal screening has allowed for earlier identification, diagnosis and management of patients with CF, and substantial advancements in both the medical and surgical care of individuals with CF has resulted in improved long term survival. Much of this progress can be attributed to a combination of the development of comprehensive CF centers and a more thorough understanding of the pathophysiology of CF. Mutations in the CF transmembrane (conductance) regulator (CFTR) gene result in CF. The alteration in CFTR results in an abnormal electrolyte content in the environment external to the apical surface of epithelial membranes. This leads to desiccation and reduced clearance of secretions from tubular structures lined by affected epithelia. Therapeutic strategies including airway clearance, inhaled medications, modern antibiotic therapy, and pancreatic enzyme replacement therapy are aimed at managing the sequelae of the mucoviscidosis. This understanding of the pathophysiology of CF combined with modern anesthesia techniques has led to improved surgical outcomes in these patients. Pediatric surgeons must remain up to date on the surgical implications of CF, and the current advancements in medical and nutritional care.

content in this topic is referenced in SCORE Cystic Fibrosis overview

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Last updated: August 3, 2018


Escobar, Mauricio (Tony) A, et al. "Cystic Fibrosis." Pediatric Surgery NaT, American Pediatric Surgical Association, 2018. APSA Webapp, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829674/all/Cystic_Fibrosis.
Escobar MA, Caty MG, Beierle EA, Williams, MD A, Stafman L, Do WS, McClellan JM. Cystic Fibrosis. In: Waldhausen J, Powell D, Hirschl R, eds. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829674/all/Cystic_Fibrosis. Accessed July 17, 2019.
Escobar, M. A., Caty, M. G., Beierle, E. A., Williams, MD, A., Stafman, L., Do, W. S., & McClellan, J. M. (2018). Cystic Fibrosis. In Waldhausen, J., Powell, D., & Hirschl, R. (Eds.), Pediatric Surgery NaT. Available from https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829674/all/Cystic_Fibrosis
Escobar MA, Caty MG, Beierle EA, Williams, MD A, Stafman L, Do WS, McClellan JM. Cystic Fibrosis [Internet]. In: Waldhausen J, Powell D, Hirschl R, editors. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. [cited 2019 July 17]. Available from: https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829674/all/Cystic_Fibrosis.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Cystic Fibrosis ID - 829674 A1 - Escobar,Mauricio (Tony),MD AU - Caty,Michael,MD, MMM AU - Beierle,Elizabeth,MD AU - Williams, MD,Adele , AU - Stafman,Laura,MD AU - Do,Woo,MD AU - McClellan,John,M.D. ED - Waldhausen,John, ED - Powell,David, ED - Hirschl,Ron, Y1 - 2018/08/03/ BT - Pediatric Surgery NaT UR - https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829674/all/Cystic_Fibrosis PB - American Pediatric Surgical Association DB - APSA Webapp DP - Unbound Medicine ER -