COPD/Cystic fibrosis Flashcards

Question

# COPD Long-acting bronchodilators

Answer 1

Shown to be superior to short-acting bronchodilators Long-acting beta agonist (LABA) salmeterol (Serevent), formoterol (Perforomist), arformoterol (Brovana) Long-acting muscarinic antagonist (LAMA) tiotropium (Spiriva), aclidinium (Tudorza), umeclidinium (Incruse) Group B patients – more symptomatic, low risk of exacerbation Regular use of a long-acting bronchodilators (beta agonist + antimuscarinic agent) LABA – resting tachycardia, tremor LAMA – dry mouth, constipation, urinary retention

Answer 2

Group E – more or less symptomatic, high risk of exacerbation Regular use of a long-acting bronchodilators (beta agonist + antimuscarinic agent) Patients with a blood eosinophil count ≥300 cells/microL or features of **asthma-COPD overlap, an inhaled glucocorticoid (ICS) should be added** budesonide or fluticasone **ICS should NOT be used as sole therapy for COPD** Adverse effects: dysphonia, skin bruising, oral candidiasis, lung infection, osteopenia/osteoporosis, HPA axis suppression

Answer 3

Outpatient or inpatient therapy depending upon severity Short-acting bronchodilators: Scheduled every 4–6 hours Continuous nebulization may be needed for severe bronchospasm Long-acting bronchodilators Systemic corticosteroids Indications for antibiotics (Augmentin as first-line option): Purulent sputum Increased dyspnea Moderate or severe exacerbation requiring hospitalization Controlled O2 therapy for acute respiratory failure: Nasal cannula Noninvasive ventilation: Hypercapnia and hypoxemia Significant effort to breathe Invasive ventilation: Severe respiratory failure May be difficult to wean patients with severe COPD

Answer 4

Inherited **(autosomal recessive)** disease of the exocrine glands affecting primarily the gastrointestinal and respiratory systems Caused by mutations in the **CFTR** (cystic fibrosis transmembrane conductance regulator) gene >1,500 causative mutations have been identified Type of mutation is crucial in determining the severity of the disease Most common mutation: **delta F508 mutation (deletion) on chromosome 7 → loss of phenylalanine in position 508** Most common life-threatening genetic disease in the white population 3-4% of the white population carries the autosomal recessive trait Incidence in the United States: 1 in 3,400 live births

Answer 5

CFTR (cystic fibrosis transmembrane conductance regulator) gene Codes for the CFTR protein → Cyclic adenosine monophosphate (cAMP)–regulated chloride channel, regulating chloride, sodium, and bicarbonate transport across epithelial membranes and into various secretions Water is attracted to the secretions leading to thinning Pathophysiology Mutated CFTR → **absent or dysfunctional chloride channel** → dysfunctional transport of chloride → abnormal secondary transport of sodium and water **Disease manifests only in homozygotes**

Answer 6

CFTR protein with the ∆F508 mutation gets misfolded and can’t migrate from the endoplasmic reticulum to the cell membrane, meaning there is a lack of CFTR protein on the epithelial surface ## Footnote Less chloride ions out → less water drawn into secretions = thickened secretions

Answer 7

Absent or dysfunctional chloride channel → **dysfunctional transport of chloride** → **abnormal secondary transport of sodium and water** In the sweat glands: Inability to reabsorb chloride from the lumen → reduced reabsorption of sodium and water → **sweat with elevated levels of sodium chloride** In the rest of exocrine glands: Inability to secrete chloride into the lumen → accumulation of intracellular chloride → increased sodium and water reabsorption → formation of **hyperviscous mucus → accumulation of secretions → blockage of small passages** → chronic inflammation → multiple organ damage

Answer 8

Ineffective mucociliary clearance Obstruction of the alveoli/bronchioles with an increased risk of infection Chronic/recurrent sinus infections **Nasal polyps** Recurrent pulmonary infections **Early in the course….Staphylococcus aureus is the most common pathogen Later in the course….Pseudomonas aeruginosa is the most common** pathogen (multidrug-resistant strains) Persistent or recurrent cough, increased sputum production, gagging, intercostal retractions, barrel-chest deformity

Answer 9

Gastrointestinal system Small intestine Thick secretions **impair absorption**, increasing the risk of obstruction Large intestine Incompletely digested macronutrients lead to thick stool, predisposing patient to impaction and obstruction **Meconium ileus**: Surgical emergency Failure to pass meconium within 48 hours of life in newborn (10-20% pf patients) Abdominal distention, vomiting, poor appetite, inadequate weight gain | At risk for rectal prolapse due to straining

Answer 10

Thick **secretions block pancreatic duct and impair fat absorption** (deficiency of fat-soluble vitamins) Decreased bicarbonate damages digestive lining and impairs absorption **Pancreatic enzymes cannot be released and slowly destroy pancreas**, leading to fibrosis and impaired endocrine function (diabetes) Abdominal pain; abdominal cramping; bloating; frequent bulky, oily stools (steatorrhea); weight loss

Answer 11

Hepatobiliary Bile is dehydrated, leading to **gallstones** (asymptomatic) Chronic cholestasis leads to inflammation and fibrosis, which results in cirrhosis and portal hypertension Right upper quadrant pain after large, fatty meals; nausea; vomiting; jaundice

Answer 12

Reproductive system Males Infertility and delayed secondary sexual development due to: Obstructive azoospermia due to bilateral aplasia/atresia of the vas deferens Undescended testicle; 15x more likely than in the general population Females Reduced fertility due to: Viscous cervical mucus Amenorrhea Excessive sweating (hot weather or fever) leads to hyponatremic/hypochloremic dehydration

Answer 13

C: Chronic cough F: Failure to thrive P: Pancreatic insufficiency (exocrine) A: Alkalosis and hypotonic dehydration N: Nasal polyps, neonatal dehydration C: Clubbing of fingers – chronic pulmonary disease R: Rectal prolapse E: Electrolyte elevation (sweat) A: Atresia, absence of vas deferens S: Sputum with Staphylococcus aureus or Pseudomonas; Stones in the gallbladder

Answer 14

Measurement of immunoreactive trypsinogen (IRT) A pancreatic enzyme released when there is pancreatic damage If elevated → mutation analysis of CFTR If results indicate CF → sweat chloride test Infant - at least 2 weeks of age with a weight over 2 kg 10% are not diagnosed until adolescence or early adulthood

Answer 15

Gold-standard of diagnosis **Pilocarpine iontophoresis** used to determine sweat chloride concentration **> 60 mmol/L in 2 tests = diagnosis of CF** Infants who have a positive newborn screening result and evidence of possible CFTR dysfunction but do not meet the diagnostic criteria for CF are classified as having CFTR-related metabolic syndrome (CRMS) **<= 29 rules out CF 30-59 is intermediate**

Answer 16

Fecal elastase Pancreatic insufficiency can be detected when screening the stool for pancreatic elastase-1, which is absent in 80% of people with CF Airway culture Persistent detection of the following bacteria in respiratory secretion and sputum 80% grow S. aureus or Pseudomonas aeruginosa Haemophilus influenzae, Burkholderia cepacia, Stenotrophomonas maltophilia All (except S. aureus) would be unusual in people without CF

Answer 17

Multidisciplinary approach Therapies: Antibiotics, inhaled drugs to thin airway secretions, and physical maneuvers to clear airway secretions (airway clearance techniques (ACTs)) Inhaled bronchodilators and sometimes corticosteroids Pancreatic enzyme replacement and vitamin supplementation High-calorie diet (sometimes requiring supplemental enteral tube feedings)

Answer 18

Prevention of infection: All routine childhood vaccines, annual influenza vaccine, high-risk protocol for pneumococcal vaccine, covid-19 vaccine Palivizumab to prevent respiratory syncytial virus infection

Answer 19

Largely determined by the degree of pulmonary involvement (deterioration is inevitable) CF patients have a median life expectancy of 45 years 50% of patients die before the age of 18 from respiratory failure and cor pulmonale Steady improvement over the past 5 years due to early diagnosis and aggressive treatment The prognosis depends on several factors: Age Gene variant Colonization by multi-drug resistant pathogens Lung function Complications Access to medical care

COPD/Cystic fibrosis Flashcards

(47 cards)