Obstructive Lung Disease CIS- Kinder Flashcards
Alpha-1 Antitrypsin Deficiency
Genetic risk factor for COPD
Etiology of 1-2% of COPD cases
Serine protease inhibitor secreted by the liver that protects the lung tissue against the action of neutrophil elastase and serine proteases.
Patients are very susceptible to damage from cigarette smoking.
Should be considered in young patients with COPD
Can lead to LFT abnormalities and cirrhosis
Cystic Fibrosis
Autosomal recessive disorder caused by mutation of the cystic fibrosis transmembrane conductance regulator protein.
Organs affected include lungs, pancreas, intestines, liver, sweat gland, sinuses, and the vas deferens.
Lung disease leads to death in 90% of patients.
Median survival 37 years
Lung Manifestations in CF
Cough, dyspnea, decreased exercise tolerance, fatigue, and increased sputum production
Steep decline in lung function at adolescence
Daily productive cough
Airway infection with Pseudomonas aeruginosa is the primary pathogen. Staphylococcus aureus, and methicillin resistant Staphylococcus aureus are also common.
Other infections include bronchopulmonary mycoses, and nontuberculous mycobacterial infections
Pancreatic Manifestations of CF
Exocrine pancreatic insufficiency can lead to impaired growth
Signs of malabsorption include bulky, foul smelling stools and flatulence.
Malabsorption of fat soluble vitamins occur.
Liver Manifestations of CF
Hepatomegaly
Splenomegaly
Hematemesis secondary to esophageal or gastric varices from portal hypertension
Sweat Gland in CF
Failure of chloride absorption from the lumen into the ductal lining cell.
Marked elevation of chloride and sodium in sweat
Vas deferens in CF
Almost all males are sterile
Other clinical manifestations in CF
Endocrine Pancreas: 1/3 of patients have diabetes by age 30
Electrolyte abnormalities can lead to nausea/vomiting, decreased appetite, circulatory collapse, and seizures
Musculoskeletal: decreased bone density secondary to decreased absorption of vitamin D, glucocorticoid treatment, and decreased exercise.
Kidney: nephrolithiasis
Cystic Fibrosis Diagnosis
Screening with immunoreactive trypsinogen which is a marker of pancreatic injury
Genetic mutation analysis
Diagnosis confirmed with sweat testing
The sweat test measures chloride concentration in sweat that is stimulated by pilocarpine iontophoresis.
5% of diagnosis are made after the age of 18
Pulmonary function testing in CF
Obstruction
Chest x-ray in CF
Hyperinflation
Bronchiectasis
Oral Antibiotics in CF
Azithromycin [25-40 kg] 250 mg PO every Mon, Wed, Fri; [> 40 kg] 500 mg PO every Mon, Wed, Fri
Patients ≥ 6 years of age with Pseudomonas aeruginosa meet criteria for use
Trimethoprim/sulfamethoxazole 20 mg/kg/day PO divided every 6-8 hours
Ciprofloxacin 40 mg/kg/day PO divided every 12 hours
IV antibiotics in CF
Aztreonam 200 mg/kg/day IV divided every 6-8 hours
Cefepime 150 mg/kg/day IV divided every 8 hours
Ceftazidime 200 mg/kg/day IV divided every 8 hours
Ciprofloxacin 30 mg/kg/day IV divided every 8-12 hours
Meropenem 120 mg/kg/day IV divided every 8 hours
Piperacillin/tazobactam 400 mg/kg/day divided every 4-6 hours
Tobramycin 7.5-15 mg/kg/day IV divided every 8-24 hours
[Higher dose, extended interval dosing may be more effective and less nephrotoxic]
Vancomycin 60 mg/kg/day divided IV every 6-8 hours
Inhaled antibiotics in CF
Tobramycin 300 mg inhaled twice daily in 28 day cycle
Aztreonam 75 mg inhaled three times daily in 28 day cycle
Other CF Treatments
Pancreatic enzymes, vitamin supplementation, bronchodilators, hypertonic saline inhalation, dornase alfa(rhDNase), ibuprofen, oxygen, bronchodilators, lung transplant
Chest percussion and postural drainage
Which of the following Pulmonary Function Test results are most likely in a patient with bullous emphysema?
A) Decreased FEV1/FVC
B) Decreased functional residual capacity
C) Decreased total lung capacity
D) Increased FEV1
E) Increased FEV1/FVC
A, decreased FEV1/FVC
Chronic Obstructive Pulmonary Disease
Progressive, mostly irreversible airflow obstruction
Onset: Middle age or elderly 20-30 years after exposure
4th leading cause of mortality in the United States
Cigarette smoking is the leading cause of COPD
Smokers have a 40ml/year reduction in FEV1 after age 30
Lung growth can be impaired by maternal smoking in pregnancy and second hand smoke in childhood
Other exposures: workplace dusts from mining, cotton mills, and grain handling facilities
Lung Mechanics in COPD
Elastic recoil is the lungs innate ability to deflate following inflation.
Elastic fibers in the lung parenchyma, along with surface tension at the alveolar air-liquid interface are responsible for elastic recoil
Elastic recoil maintains the patency of small airways
Elastic recoil is markedly decreased in COPD
Airway resistance is increased in COPD
The sites of airflow obstruction are distal airways less than 2mm diameter
COPD History
Current or past cigarette use Dyspnea with slow progression History of acute bronchitis History of a chronic cough Sputum production Wheezing
Physical Findings COPD
Barrel Chest Prolonged expiratory phase Accessory muscle use Low Diaphragm Distant Heart Sounds Diminished Breath Sounds Rhonchi Wheezing Cyanosis Pedal Edema Distended Jugular Veins Hepatic congestion Cachexia “Blue Bloaters” -cyanotic, respiratory acidosis “Pink Puffers”- usually emphysematous, don't respond to bronchodilators, cachectic
pumonary function testing stages and severity
Mild- FEV1 > 80% predicted
moderate- > 50%
severe: > 30%
Very severe: under 30%
For all categories FEV1/FVC is under 70%
