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Respiratory Conditions Update: Cystic Fibrosis.
FP Essent 2016; 448:35-43FE

Abstract

Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed.

Authors+Show Affiliations

Medical University of South Carolina Department of Pediatrics, 96 Jonathan Lucas St #312, Charleston, SC 29425.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

27576234

Citation

Pritchard, Lyle L.. "Respiratory Conditions Update: Cystic Fibrosis." FP Essentials, vol. 448, 2016, pp. 35-43.
Pritchard LL. Respiratory Conditions Update: Cystic Fibrosis. FP Essent. 2016;448:35-43.
Pritchard, L. L. (2016). Respiratory Conditions Update: Cystic Fibrosis. FP Essentials, 448, pp. 35-43.
Pritchard LL. Respiratory Conditions Update: Cystic Fibrosis. FP Essent. 2016;448:35-43. PubMed PMID: 27576234.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Respiratory Conditions Update: Cystic Fibrosis. A1 - Pritchard,Lyle L, PY - 2016/8/31/entrez PY - 2016/9/1/pubmed PY - 2017/1/7/medline SP - 35 EP - 43 JF - FP essentials JO - FP Essent VL - 448 N2 - Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed. SN - 2159-3000 UR - https://www.unboundmedicine.com/medline/citation/27576234/Respiratory_Conditions_Update:_Cystic_Fibrosis_ L2 - http://babysfirsttest.org/newborn-screening/conditions/cystic-fibrosis-cf DB - PRIME DP - Unbound Medicine ER -