Cystic Fibrosis Overview
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Questions and Answers

What is the main cause of respiratory issues in cystic fibrosis patients?

  • Obstruction caused by parasites
  • Deterioration of lung tissue due to aging
  • Increased blood pressure in the lungs
  • Genetic defect affecting sodium chloride movement (correct)

    • Which of the following medications is specifically used to improve lung function in cystic fibrosis?

  • Ursodeoxycholate
  • Corticosteroids
  • Tobramycin (correct)
  • Leukotriene receptor antagonist

    • What is the significance of chronic infections in cystic fibrosis patients?

  • They reduce the need for antibiotic therapy.
  • They indicate the presence of lung cancer.
  • They serve as reservoirs for transmission to other patients. (correct)
  • They enhance immune system function.

    • Which type of vitamin supplementation is particularly needed for cystic fibrosis patients due to malabsorption issues?

    <p>Both water-soluble and fat-soluble vitamins</p> Signup and view all the answers

    What type of precautions should be maintained for cystic fibrosis patients to minimize infection spread?

    <p>Universal and droplet precautions</p> Signup and view all the answers

    Which of the following mechanisms contributes to airway obstruction during an acute asthma attack?

    <p>Mucous membrane edema and bronchospasm</p> Signup and view all the answers

    Which symptom is often the sole manifestation in cough-variant asthma?

    <p>Cough</p> Signup and view all the answers

    What physiological effect does pulsus paradoxus indicate in an asthma patient?

    <p>Increasing severity of asthma attack</p> Signup and view all the answers

    What is the primary purpose of chest physiotherapy in managing cystic fibrosis?

    <p>To promote expectoration and dislodge secretions</p> Signup and view all the answers

    What are the absolute indications for the use of intubation and ventilation in status asthmaticus?

    <p>Cardiac arrest and severe hypoxia</p> Signup and view all the answers

    Which of the following assessments is NOT part of the asthma diagnosis according to NIH and NHBLI guidelines?

    <p>Genetic testing for asthma predictors</p> Signup and view all the answers

    In managing cystic fibrosis, what is included in the dietary recommendations?

    <p>120 to 150% of RDA of calories and 200% of RDA of protein</p> Signup and view all the answers

    Which of the following is NOT a common symptom of asthma?

    <p>Metabolic alkalosis</p> Signup and view all the answers

    What is a significant danger of using mechanical ventilation in status asthmaticus?

    <p>Increased bronchospasm and barotrauma</p> Signup and view all the answers

    What is the recommended action when assessing responsiveness to therapy in asthma patients?

    <p>Inhale a short-acting bronchodilator and measure FEV1 change</p> Signup and view all the answers

    Which cytokines are primarily involved in the inflammatory response during an asthma attack?

    <p>Cytokines produced by mast cells and T lymphocytes</p> Signup and view all the answers

    Which statement about education in asthma management is correct?

    <p>Education should include a written asthma action plan for daily management</p> Signup and view all the answers

    What is the clinical significance of decreased pulse on inspiration and increased on expiration in a patient with asthma?

    <p>Suggests worsening respiratory distress</p> Signup and view all the answers

    What is the role of allergens in asthma management according to the guidelines?

    <p>Patients should be evaluated through history and skin testing.</p> Signup and view all the answers

    Which of the following should patients avoid to manage their asthma effectively?

    <p>Sulfite-containing foods</p> Signup and view all the answers

    What is one of the main purposes of corticosteroids in asthma treatment?

    <p>To reduce symptoms and gain control when starting long-term therapy.</p> Signup and view all the answers

    How do leukotriene modifiers function in asthma treatment?

    <p>They help with reducing inflammation and mucus production.</p> Signup and view all the answers

    In what cases is Omalizumab prescribed as part of asthma management?

    <p>As adjunctive therapy for those with allergies and severe asthma.</p> Signup and view all the answers

    What characterizes status asthmaticus?

    <p>Severe attacks unresponsive to conventional therapy leading to respiratory failure.</p> Signup and view all the answers

    What does a pulse paradoxus measurement greater than 20-40 mm Hg indicate?

    <p>Potential respiratory distress</p> Signup and view all the answers

    What is indicated if pulse paradoxus is absent?

    <p>Imminent respiratory arrest</p> Signup and view all the answers

    What is the significance of a PaO2 reading of 95%?

    <p>Suggests optimal oxygenation</p> Signup and view all the answers

    If PaCO2 is greater than 55 mm Hg, what can be inferred about the patient's respiratory status?

    <p>Possible respiratory acidosis</p> Signup and view all the answers

    Which of the following could indicate a respiratory status issue?

    <p>Pulse paradoxus present</p> Signup and view all the answers

    What does a pulse paradoxus measurement signify in the context of respiratory assessment?

    <p>Presence of respiratory distress</p> Signup and view all the answers

    When assessing a patient with breathing difficulties, what parameter is critical for evaluating respiratory status?

    <p>Ratio of PaCO2 to PaO2</p> Signup and view all the answers

    A patient presents with a pulse paradoxus >40 mm Hg. What is the most immediate concern?

    <p>Possible respiratory failure</p> Signup and view all the answers

    Which condition is most likely responsible for a pulse paradoxus reading?

    <p>Cardiac tamponade</p> Signup and view all the answers

    Which of the following outcomes is least likely in a patient with a PaCO2 of 60 mm Hg?

    <p>Respiratory alkalosis</p> Signup and view all the answers

    What condition results from the failure of the ductus arteriosus to close after birth?

    <p>Patent Ductus Arteriosus</p> Signup and view all the answers

    Which symptom is associated with a significant left to right shunt due to a large ventricular septal defect?

    <p>Heart murmur with difficulty feeding</p> Signup and view all the answers

    What is the primary treatment for closing a patent ductus arteriosus in infants within 10 days of birth?

    <p>Indomethacin (Indocin®)</p> Signup and view all the answers

    Which complication is a risk associated with both patent ductus arteriosus and ventricular septal defect?

    <p>Bacterial endocarditis</p> Signup and view all the answers

    In patients with patent ductus arteriosus, which of the following findings may indicate increased pulmonary hypertension?

    <p>Widened pulse pressure</p> Signup and view all the answers

    What is a potential complication of ventilating a patient with submersion asphyxia using positive-end expiratory pressure (PEEP)?

    <p>Barotrauma risk</p> Signup and view all the answers

    In the context of atrial septal defect (ASD), what is the most likely long-term consequence if the defect is not treated?

    <p>Pulmonary hypertension</p> Signup and view all the answers

    What type of defect involves openings between the atria and ventricles and is often associated with Down syndrome?

    <p>Atrioventricular canal defect</p> Signup and view all the answers

    What immediate action is critical for patients suffering from submersion asphyxia?

    <p>Establish airway, breathing, and circulation</p> Signup and view all the answers

    What symptom might suggest a partial atrioventricular canal defect that has remained undiagnosed for an extended period?

    <p>Weakness and fatigue</p> Signup and view all the answers

    What is a significant risk associated with high flow oxygen therapy in patients with respiratory distress from submersion asphyxia?

    <p>Oxygen toxicity</p> Signup and view all the answers

    With an atrial septal defect, which clinical finding is most indicative of increasing pulmonary vascular obstructive disease?

    <p>Heart murmur</p> Signup and view all the answers

    What is the goal of fluid management in patients with submersion asphyxia?

    <p>Control cerebral and pulmonary edema</p> Signup and view all the answers

    What can occur as a result of the extra blood flow to the lungs in patients with atrioventricular canal defect?

    <p>Congestive heart failure</p> Signup and view all the answers

    Which of the following treatments may not be necessary for a small atrial septal defect?

    <p>Open-heart surgical repair</p> Signup and view all the answers

    Study Notes

    Cystic Fibrosis

    • Cystic fibrosis (CF) is a progressive congenital disorder primarily affecting lungs and pancreas, leading to digestive and respiratory complications.
    • Caused by a genetic defect impacting sodium chloride movement in cells, resulting in thick mucus production that clogs airways and fosters bacterial growth.
    • Historically, most patients died in childhood, but current life expectancy has improved to about 30 years.
    • Common infections in CF patients involve Pseudomonas aeruginosa and Burkholderia cepacia, with infection spread occurring through close contact.
    • Universal and droplet precautions are crucial for infection control in CF patients during hospitalization.

    Management of Cystic Fibrosis

    • Medications: Include antibiotics (e.g., Tobramycin), bronchodilators, corticosteroids, Dornase alfa, lactulose, Montelukast, pancreatic enzyme supplements, ursodeoxycholic acid, and vitamin supplements.
    • Chest physiotherapy: Involves techniques like percussion and vibration to clear mucus from lungs.
    • Exercise: Promotes muscle maintenance and lung function.
    • Surgery: May involve chest tube insertion or thoracostomy in severe cases; lung transplant is an option for respiratory failure.
    • Diet: Recommended caloric intake is 120-150% RDA, with increased protein and moderate fat intake to support nutritional needs.

    Asthma Guidelines (NIH/NHLBI)

    Component 1 - Assessment and Monitoring

    • Severity of asthma is assessed through patient history, physical examination, and spirometry for those age 5 and older.
    • Key diagnostic indicators include cough, wheezing, varying symptoms, and airflow obstruction which is reversible.

    Component 2 - Education

    • Education for asthma management begins at diagnosis and should be integrated across multiple care settings.
    • Includes a written asthma action plan and fostering patient involvement in decision-making.

    Component 3 - Environmental Factors

    • Assessment of allergens, potential irritants, and occupational exposures is crucial for asthma management.
    • Recommendations include avoiding allergens and respiratory irritants and utilizing air conditioning to reduce exposure levels.

    Component 4 - Pharmacologic Treatment

    • Long-term asthma control medications include corticosteroids, cromolyn, omalizumab, leukotriene modifiers, and long-acting beta agonists (LABAs).
    • Medications for acute exacerbations feature short-acting beta agonists (SABAs), anticholinergics, and systemic corticosteroids.

    Status Asthmaticus

    • A severe asthma attack unresponsive to standard treatments, leading to potential respiratory failure.
    • Common symptoms include cough, wheezing, dyspnea, and increased work of breathing, with potential for respiratory distress.

    Submersion Asphyxia

    • Can result in significant CNS damage, pulmonary complications, and multi-organ failure.
    • Treatment involves establishing ABCs, oxygen administration, fluid management, and continuous monitoring for complications.

    Congenital Heart Defects

    Atrial Septal Defect (ASD)

    • An opening in the atrial septum can lead to left-to-right shunting of blood, potentially asymptomatic or causing heart murmur and congestive heart failure.
    • Treatment options include surgical repair or catheter-based closure.

    Atrioventricular Canal Defect

    • Commonly associated with Down syndrome, involves multiple defects in heart structure.
    • Symptoms may include failure to thrive and heart failure manifestations.

    Patent Ductus Arteriosus (PDA)

    • PDA leads to increased pulmonary blood flow due to failure of ductus arteriosus closure.
    • Symptoms can include a machinery-like murmur and frequent respiratory infections. Treatment includes medications or surgical ligation.

    Ventricular Septal Defect

    • An opening between ventricles causing left-to-right shunting; smaller openings may be asymptomatic.
    • Signs and treatment similar to other congenital defects, focusing on symptom management and possible surgical interventions.

    Aortic Stenosis and Coarctation of the Aorta

    • Aortic Stenosis: Narrowing of the aortic valve leading to increased left ventricular pressure, potentially resulting in chest pain and fainting.
    • Coarctation of the Aorta: Stricture of the aorta, causing differential blood pressures in body regions and symptoms ranging from headaches to CHF signs.

    Types of Congenital Heart Disease

    • Acyanotic Defects: Includes ASD, VSD, PDA and results in increased blood flow.
    • Cyanotic Defects: Involves conditions like Tetralogy of Fallot with decreased pulmonary blood flow.

    Pulmonic Stenosis

    • A stricture of the pulmonic valve may present with symptoms ranging from asymptomatic to right ventricular hypertrophy as the demand for oxygen increases.

    These study notes provide a comprehensive overview of cystic fibrosis, asthma management, congenital heart defects, and related conditions, highlighting essential treatments, diagnostic criteria, and pathophysiology involved.### Pulmonic Stenosis

    • Associated with various heart defects.
    • Symptoms include:
      • Loud heart murmur and congestive heart murmur.
      • Mild cyanosis and cardiomegaly.
      • Angina and dyspnea.
      • Fainting episodes.
      • Increased risk of bacterial endocarditis.
    • Treatment options:
      • Balloon valvuloplasty for children to separate valve cusps.
      • Surgical repair via closed transventricular valvotomy (Brock procedure) for infants.
      • Cardiopulmonary bypass pulmonary valvotomy for older children.

    Tetralogy of Fallot (TOF)

    • Comprises four defects:
      • Ventricular septal defect with a large opening.
      • Pulmonic stenosis causes reduced pulmonary blood flow.
      • Overriding aorta resulting in blood mixing from both ventricles.
      • Right ventricular hypertrophy is present.
    • Symptoms include severe cyanosis in infants, particularly right after birth.
    • Some infants may experience increasing cyanosis within the first year.
    • Additional indicators:
      • Intolerance to feeding or crying leads to "tet spells."
      • Failure to thrive with inadequate growth.
      • Clubbing of fingers may develop over time.
      • Activity intolerance as the child grows.
      • Increased risk for emboli, strokes, seizures, and sudden death.
    • Preferred treatment: total surgical repair by age one, replacing previous palliative approaches.

    Tricuspid Atresia

    • Characterized by absence of the tricuspid valve between the right atrium and ventricle.
    • Blood flow through foramen ovale or atrial defect to the left atrium, then through ventricular wall defect to the right ventricle and to lungs.
    • Commonly presents with pulmonic obstruction.
    • Symptoms include:
      • Post-natal cyanosis.
      • Tachycardia and dyspnea.
      • Rising hypoxemia and clubbing in older children.
      • Increased risk for bacterial endocarditis, brain abscess, and stroke.
    • Treatment options:
      • Prostaglandin (alprostadil) to maintain ductus arteriosus and foramen ovale if no septal defects are present.
      • Several surgical interventions: pulmonary artery banding, aortic-pulmonary shunting, Glenn procedure for deoxygenated blood flow to lungs, atrial septostomy, and Fontan corrective procedure (typically performed at 2-4 years following initial stabilizing procedures).

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    Description

    This quiz explores cystic fibrosis, a progressive congenital disease that primarily affects the pancreas and lungs. Learn about its genetic causes, symptoms, and the challenges faced by patients. Test your knowledge on the impact of this condition on respiratory and digestive functions.

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