4923 C21 Diseases of the Respiratory System Flashcards
cystic fibrosis (CF)
Disease characterized by abnormally thick mucus secretions from the epithelial surfaces of various organ systems, including the respiratory tract, the GI tract, liver, genitourinary system, & the sweat glands.
What is the most common autosomal recessive disease in the US?
cystic fibrosis
autosomal recessive
method of hereditary disease transmission in which the patient receives 2 chromosomes bearing the gene anomaly, one from each parent.
Etiology of CF
Is caused by a mutation of the CFTR (cystic fibrosis transmembrane conductance regulator, a type of ABC transporter, it does not function normally.
Aerosol therapies used to treat CF?
inhaled bronchodilators, inhaled anti-inflammatory agents, inhaled mucolytics
chest physiotherapy
PT that includes a variety of techniques designed to reduce or prevent infection by clearing pooled secretions &/or infected materials from the lungs.
How is CF diagnosed?
Sweat chloride test. CFTR gene mutation blood test. Sputum cultures. Pancreatic function tests.
sweat chloride test
a test to measure the amount of chloride in the sweat by stimulating the skin to produce a large amount of sweat that is then absorbed by a special filter paper & analyzed for chloride content.
plasma prothrombin concentrations
a measure of blood clotting ability. A measure of vitamin K nutriture.
pancreatic enzyme dose for infants
1,000-4,000 lipase units /120mL of formal/breast milk
pancreatic enzyme dose for children
begins at 1,000 units /kg/meal for children < 4. 500 lipase units /kg/meal for children > 4. Require less per kg with age due to decreased fat intake.
Recommended fat intake for CF patients
35-40% of kcal due to decreased absorption.
signs and symptoms of CF
Usually develop in the first year of life. Persistant diarrhea. Bulky, greasy, foul smelling stools. Wheezing & pneumonia. Chronic cough, thick mucous. Salty tasting skin. Poor growth.
Sings and symptoms of CF that may begin to appear after the 1st yr of life?
Clubbing. SOB
How are newborns tested for CF
Immunoreactive trypsinogen. A high amount suggests pancreatic damage, but is not specific for CF.
How are respiratory infections treated in CF?
Inhaled antibiotic Tobramycin for P. areuginosa. Mucolytics. Lung transplant
Pulmozyme
Mucolytic agent
pancreatic enzyme dose for adults
500-2500 lipase units /kg/meal. < 10,000u lipase per kg body wt. /day.
Overall nutrition goal for CF
Increase fat & kcal intake. Maintain LBM
Benefits of Aerobic activity in CF
Loosen chest congestion. Encourages coughing to clear mucous. Improve overall physical condition.
Bronchopulmonary dysplasia (BPD)
A chronic lung disorder that may affect infants who have been exposed to high levels of oxygen therapy and ventilator support. Have received oxygen for at least 28 days, & are less than 32 weeks.
Factors associated with BPD
Extreme prematurity <1500g. Perinatal infection, Patent ductus arteriosus (PDA).
What causes BPD
Genetics play a role. Is linked to ventilator trauma. ROS which the neonate has no antioxidants to neutralize. < 28 weeks. Poor Vit A nutriture.
How is vitamin A nutriture related to BPD
is important in normal alveolar development and surfactant production as well as regeneration of respiratory epithelial cells.