Cystic Fibrosis RSCC RT Pathology I

Classical CF Triad
Pancreatic exocrine insufficiency, pulmonary disease, elevated sweat chloride concentration

Avg life expectancy
30 yrs

Common Bacteria
S. aureus, hemophilia influenza, P. aeruginosa

Chronic dilation and distortion of one or more bronchi

Cause of bronchiectasis
response to extensive inflammation and destruction of bronchial tree cartilage, blood vessels, elastic tissue, and smooth muscle

mucociliary clearing mechanism
impaired due to bronchial wall destruction

3 types of bronchiectasis
varicose, cylindrical, saccular

varicose bronchiectasis
bronchi are dilated and constructed in an irregular fashion similar to varicose veins

cylindrical bronchiectasis
bronchi are dilated and have regular outlines similar to a tube

saccular bronchiectasis
bronchi progressively increase in diameter until ending in large cyst like sac in lung parenchyma

most damaging bronchietasis

CF and bronchiectasis etiology
because of problems associated with increased sputum and bronchial obstruction leading to infection and causing increase in inflammation leading to bronchiectasis

diagnosis of bronchiectasis
diagnosed by bronchogram: injection of opaque contrast materieal into trachobronchial tree

sweat glands
may secrete up to 4 times the normal amount of NaCl

upper airway
recurrent sinusitis

Meconium ileus: obstruction of small intestines of newborn caused by thick, dry, tenacious impaction

Autosome chromosomes
all chromosomes besides sex chromosomes

gene responsible for CF
chromosome #7

appearance of recessive trait
both genes must be identical

Mendelian Pattern
autosomal recessive inheritance pattern

must have recieved a CF gene from both parents

signs of chronic hypoxemia
polycythemia, digital clubbing

lab eval for polycythemia
complete cbc to look for increased RBC count

barrel chest
increased A-P diameter of chest

standard test for CF
sweat chloride test

most significant health threat to CF
recurrent respiratory infections

CF treatment
O2 therapy, mucolytics, bronchial hygiene therapy, CPT, postural drainage, pickle, PEP therapy, vest, antibiotics, bronchodilator therapy

acetylcysteine, breaks down disulfide bounds in mucous

Dornase alfa, breaks up DNA debris in sputum. Lowers viscocity

Antibiotic, given in seperate nebulizer after bronchodilator

short acting beta 2 agonist, bronchodilator, sympathomimetic

Formoterol – Long acting adrenergic bronchodilator

fluticasone, corticosteroid

Tiotropium Bromide
Respiratory Antimuscarinic

Ipratropium Bromide – anticholinergic parasympatholytic bronchodilator

Extended home care
education, hydration, O2 use, aerosol treatments, cough training, brochial hygiene maneuvers, watch sputum, pursedlip breathing and diapragmatic breathing

A 30-year-old female presents with a five day history of a sore throat. She denies cough or nasal congestion. She also denies vomiting or diarrhea. On physical exam, her temperature is 101˚F, the pharynx is red with tonsillar exudates, and …

Abscess Risk factors Smoking (70-80%) Immunodeficiency Bronchiectasis Malignancy (lung cancer in 12%) COPD Steroids Aspiration Decreased level of consciousness Seizures Alcohol abuse Swallowing disorders Mechanical ventilationImaging Thick, irregular wall Thickness > 15 mm suggests neoplasm Spherical Small contact with chest …

Pneumo 1 A 28-year-old man is evaluated for a 6-month history of episodic dyspnea, cough, and wheezing. As a child, he had asthma and allergies, but he has been asymptomatic since his early teenage years. His recent symptoms started after …

1 (Rationale: Peripheral neuropathy is caused by diminished perfusion to neurons and results in loss of both pressure and deep pain sensations. The patient may not notice lower extremity injuries. Neuropathy increases susceptibility to traumatic injury and results in delay …

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