Cystic Fibrosis Clinical Features, Diagnosis, and Treatments
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This document provides an overview of cystic fibrosis including: - It is a genetic disease caused by mutations in the CFTR gene resulting in defective chloride channels. - Diagnosis is based on clinical features and sweat chloride tests or genetic testing. - Symptoms include chronic lung disease, poor growth, GI issues, and electrolyte imbalances. - Treatments focus on airway clearance, antibiotics for infections, nutrition support, and management of complications.
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- 1. Cystic Fibrosis Esmeralda E. Morales, MD August 28, 2006
- 6. Welsh, MJ and Smith, AE. Cystic Fibrosis. Scientific American . 273 (6): 52, 1995.
- 12. Cell membrane diagram From: http://library.thinkquest.org/C004535/cell_membranes.html
- 13. CFTR Gibson, RL, Burns, JL, and Ramsey, BW. Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis. AJRCCM 168 (918-951); 2003.
- 15. CFTR and Airway Surface Liquid Donaldson,SH and Boucher,RC. Update on the pathogenesis of cystic fibrosis lung disease. Current Opinion in Pulmonary Medicine. 9: 486-491; 2003.
- 21. Infection Gibson, RL, Burns, JL, and Ramsey, BW. Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis. AJRCCM 168 (918-951); 2003.
- 53. “ It is, in fact, nothing short of a miracle that the modern methods of instruction have not entirely strangled the holy curiosity of inquiry.” ----Albert Einstein
Hinweis der Redaktion
- Many functions still under investigation----CFTR
- Chronic sp disease---chronic cough, recurrent sp infection, bronchiolitis/asthma, nasal polyps Nutr---ftt, vit def Gi---mec ileus, DIOS, rectal prolapse, liver disease---biliary cirrhosis
- Delta f508 causes loss of the amino acid phenylalanine located at position 508 in the protein
- Age-specific prevalence of airway infections in patients with CF. Organisms reported to the U.S. Cystic Fibrosis Patient Registry, 2001 Early infections in CF airways are most frequently caused by S. aureus and H. influenzae. natural history study of patients with CF in the first 3 years of life, the mean age of detection of an antibody response to P. aeruginosa was approximately 15 months, whereas the mean ages of first positive upper and lower airway culture were approximately 21 and 23 months
- Biofilms are sessile communities of bacteria that form in aggregates on surfaces using a hydrated polymeric matrix of their own synthesis ( Figures 6D and 6E ). Some common clinical characteristics of biofilm infections have been identified: slow growth of organisms, stimulation of production of antibodies that are ineffective in clearing bacteria, inherent resistance to antibiotics, and an inability to eradicate biofilm infections even in hosts with intact immune systems ( 134 – 137 ).
- Nine recognized genomovars Most common are II, III, V
- Ursodiol increases bile flow {02} . In chronic cholestatic liver disease, ursodiol appears to reduce the detergent properties of the bile salts, thus reducing their cytotoxicity. Also, ursodiol may protect liver cells from the damaging activity of toxic bile acids (e.g., lithocholate, deoxycholate, and chenodeoxycholate), which increase in concentration in patients with chronic liver disease