Pediatric Surgery NaT

Cystic Fibrosis

Mauricio (Tony) A Escobar, MD, Michael G. Caty, MD, MMM, Elizabeth A Beierle, MD, Adele Williams, MD, Laura Stafman, MD, Woo S. Do, MD, John M McClellan, M.D.
Cystic Fibrosis is a topic covered in the Pediatric Surgery NaT.

To view the entire topic, please or purchase a subscription.

APSA Pediatric Surgery Library combines Pediatric Surgery Not a Textbook (NaT) with APSA ExPERT, a powerful platform for earning MOC CME credits -- all powered by Unbound Medicine. Explore these free sample topics:

Pediatric Surgery Library

-- The first section of this topic is shown below --

Introduction

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.

-- To view the remaining sections of this topic, please or purchase a subscription --

Last updated: April 5, 2018

Citation

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. Y1 - 2018/04/05/ PB - Pediatric Surgery NaT UR - https://www.unboundmedicine.com/medline/view/Pediatric-Surgery-NaT/829674/all/Cystic_Fibrosis ER -