Cystic Fibrosis Overview
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Questions and Answers

Which of the following pathogens is most commonly associated with community-acquired pneumonia?

  • Candida albicans
  • Haemophilus influenza
  • Staphylococcus aureus
  • Streptococcus pneumoniae (correct)
  • What is one consequence of chronic inflammation in brochiectasis?

  • Improved gas exchange
  • Increased lung capacity
  • Reduction in fibrosis
  • Destruction of lung parenchyma (correct)
  • What type of pneumonia involves the alveoli?

  • Lobar pneumonia
  • Alveolar pneumonia (correct)
  • Interstitial pneumonia
  • Bronchopneumonia
  • Which pathogen is more commonly associated with hospital-acquired pneumonia?

    <p>Staphylococcus aureus</p> Signup and view all the answers

    Which risk factor is most associated with Pneumocystis Jirovecii Pneumonia (PCP)?

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

    What is the most common route of infection for pneumonia?

    <p>Inhalation of pathogens in air droplets</p> Signup and view all the answers

    What is the primary treatment for Pneumocystis pneumonia?

    <p>Antifungal medications</p> Signup and view all the answers

    Which of the following symptoms is NOT commonly associated with Legionnaire's Disease?

    <p>Weight loss</p> Signup and view all the answers

    Which of the following classifications of pneumonia involves the segmental bronchi?

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

    What is a significant complication associated with untreated Pneumocystis pneumonia?

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

    Which type of pneumonia is described as widespread or diffuse?

    <p>Lobar pneumonia</p> Signup and view all the answers

    Which group is more likely to develop pneumonia due to immunocompromised conditions?

    <p>Individuals with weakened immune systems</p> Signup and view all the answers

    Which of the following is classified as an extraneous pathogen causing pneumonia?

    <p>Mycobacterium tuberculosis</p> Signup and view all the answers

    What is a characteristic of primary pulmonary TB?

    <p>It usually remains asymptomatic or latent.</p> Signup and view all the answers

    Which factor is NOT considered a risk factor for developing pulmonary TB?

    <p>Participation in regular exercise</p> Signup and view all the answers

    What is the primary etiological agent responsible for pulmonary TB?

    <p>Mycobacterium tuberculosis</p> Signup and view all the answers

    Which step is NOT part of the pathogenesis of pulmonary TB?

    <p>Formation of a bacterial capsule</p> Signup and view all the answers

    What demographic has the highest incidence of pulmonary TB globally?

    <p>People in southeast Asia, Africa, and Eastern Europe</p> Signup and view all the answers

    Which clinical manifestation is most commonly associated with type 4 hypersensitivity reaction leading to granuloma formation?

    <p>Night sweats</p> Signup and view all the answers

    What is a primary method of diagnosing a case of tuberculosis?

    <p>Tuberculin skin test</p> Signup and view all the answers

    Which factor is NOT a common etiology for lung abscess formation?

    <p>Chronic bronchitis</p> Signup and view all the answers

    What type of bacteria is most commonly associated with lung abscesses due to aspiration?

    <p>Anaerobic bacteria</p> Signup and view all the answers

    What is a characteristic symptom of lung abscess that differentiates it from other pulmonary conditions?

    <p>Expectoration of foul-smelling sputum</p> Signup and view all the answers

    Which of the following is NOT a treatment for tuberculosis?

    <p>Immediate vaccination</p> Signup and view all the answers

    What is the first step in the pathogenesis of lung abscess formation after pathogen introduction?

    <p>Inflammation leading to tissue necrosis</p> Signup and view all the answers

    Which of the following patients is most at risk for developing a lung abscess?

    <p>Older patients unable to manage oral secretions</p> Signup and view all the answers

    What is the primary defect in patients with cystic fibrosis?

    <p>Impaired ion transport channels of Na+ and Cl-</p> Signup and view all the answers

    What is a common gastrointestinal manifestation of cystic fibrosis?

    <p>Malabsorption and failure to thrive</p> Signup and view all the answers

    Which of the following is a rare condition associated with the formation of a cyst in the chest?

    <p>Bronchogenic Cyst</p> Signup and view all the answers

    What is the most common viral cause of the common cold?

    <p>Rhino Virus</p> Signup and view all the answers

    What symptom is NOT commonly associated with influenza?

    <p>Persistent runny nose</p> Signup and view all the answers

    Which of the following is a significant risk factor for developing pneumonia?

    <p>Being younger than 5 years old</p> Signup and view all the answers

    In pneumonia, what routes can the infections commonly come from?

    <p>Inhalation and aspiration</p> Signup and view all the answers

    What is the primary treatment for acute bronchitis?

    <p>Symptomatic treatment</p> Signup and view all the answers

    What common complication can arise from influenza?

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

    What is the primary symptom of sinusitis?

    <p>Purulent rhinorrhea</p> Signup and view all the answers

    What is NOT a characteristic clinical manifestation of cystic fibrosis?

    <p>Weight gain</p> Signup and view all the answers

    Which factor can lead to chronic obstructive pulmonary disease (COPD) symptoms, similar to those seen in cystic fibrosis?

    <p>Exposure to second-hand smoke</p> Signup and view all the answers

    What distinguishes intralobar sequestration from extralobar sequestration?

    <p>Presence of a functional bronchial connection</p> Signup and view all the answers

    What is a common symptom of acute bronchitis?

    <p>Chronic productive cough</p> Signup and view all the answers

    Study Notes

    Cystic Fibrosis

    • Inherited genetic disorder, autosomal recessive.
    • Affects exocrine glands, causing thick, viscous mucosal secretions due to defective ion transport channels.
    • Primarily affects respiratory, digestive, and reproductive systems.
    • Predisposes to chronic bacterial airway infections and progressive pulmonary function loss.
    • Most common inherited genetic disease in white Europeans.
    • Over 1000 new cases diagnosed yearly, with 1 in 25 being carriers.
    • Caused by a genetic defect on chromosome 7, usually a spontaneous mutation.
    • Impaired sodium/chloride channels lead to salt accumulation in tissues, resulting in thick mucus.
    • Clinical manifestations include:
      • High sodium and chloride concentrations in sweat.
      • Pancreatic issues: thick secretions block ducts, leading to maldigestion, malabsorption, fibrosis, bulky stools, and potential diabetes.
      • Gastrointestinal issues: meconium ileus, rectal prolapse, intestinal obstruction, poor nutrition, weight loss, and growth retardation.
      • Pulmonary issues: chronic cough, purulent sputum, chronic pulmonary infection, hypoxia, clubbing, cyanosis, barrel chest, pectus carinatum, kyphosis, and respiratory failure.
      • Genitourinary issues: infertility in men and common in women.
      • Musculoskeletal issues: muscle pain and decreased bone density.
      • Endocrine issues: cystic fibrosis-related diabetes, a mix of type 1 and type 2.
    • Diagnosis involves genetic testing, clinical presentation (failure to thrive, respiratory issues), sweat test, pancreatic enzyme test, and pulmonary function test.
    • Treatment depends on the systems involved and may include:
      • Antibiotics for recurrent infections.
      • Medications to decrease mucus production and bronchodilators.
      • Chest physiotherapy.
      • Adequate nutrition and supplementation.
      • Lung transplant in severe cases.
    • Prognosis is improving, with a median survival rate of 37 years.

    Bronchogenic Cyst

    • Rare condition characterized by a cyst formation in the middle of the chest, usually behind the manubrium.
    • Often found incidentally and may be asymptomatic.
    • Symptoms may include chest pain, cough, and shortness of breath.

    Extralobar Sequestration

    • Mass of lung tissue unconnected to the bronchial tree and located outside the visceral pleura.
    • Receives blood supply from an abnormal artery, typically originating from the aorta.
    • Considered congenital.

    Intralobar Sequestration

    • Mass of lung tissue within the visceral pleura, isolated from the tracheobronchial tree and supplied by a systemic artery.
    • Previously thought to be congenital, but now considered acquired.
    • More common than extralobar sequestration.

    Common Cold

    • Acute, usually afebrile viral infection of the upper respiratory tract.
    • Also known as infectious rhinitis.
    • Most commonly caused by rhinovirus (50%), but other viruses like coronavirus, adenovirus, parainfluenza virus, and others can also be responsible.
    • Highly contagious.
    • Clinical manifestations include:
      • Scratchy or sore throat.
      • Sneezing.
      • Rhinorrhea (runny nose).
      • Nasal obstruction.
      • Malaise (general discomfort).
      • Cough.

    Influenza

    • Viral respiratory infection causing fever, coryza (rhinitis), cough, headache, and malaise.
    • Can be fatal during epidemics, particularly among high-risk patients.
    • Etiology: Primarily influenza virus, but other viruses can also cause it.
    • Risk factors include: Children, elderly, people with chronic disease, pregnant women, and those with other disorders.
    • Clinical manifestations include:
      • Common cold symptoms.
      • Conjunctivitis (red, itchy eyes).
      • Chills.
      • Fever.
      • Prostration (extreme fatigue).
      • Cough.
      • Aches and pains.
      • Severe fatigue.
      • Headache.
      • Coryza (runny nose).
      • Scratchy throat.
      • Nausea.
      • Vomiting.
      • Abdominal pain.
    • Diagnosis is made through clinical evaluation.
    • Treatment is symptomatic (fluids, rest) and may include antiviral medications for high-risk individuals.
    • Prognosis is generally good.
    • Complications:
      • Pneumonia: caused by pneumococcus (community-acquired) or Staph aureus (hospital-acquired).
      • Encephalitis (inflammation of the brain).
      • Guillain-Barré syndrome (autoimmune condition affecting nerves).
      • Renal disease (viral infection of the kidney, fluid overload due to fluid loss).
    • Prevention includes handwashing and vaccination.

    Sinusitis

    • Inflammation of the paranasal sinuses.
    • Can be classified as:
      • Acute (bacterial).
      • Subacute.
      • Recurrent (chronic), often due to allergies.
    • Etiology: viral, bacterial, fungal infection or recurrent allergies.
    • Clinical manifestations are variable and may include:
      • Purulent rhinorrhea (pus-like nasal discharge).
      • Pressure and pain.
      • Nasal congestion and irritation.
      • Tenderness, swelling, and redness (erythema).
      • Toothache.
      • Headache.
      • Cough.
      • Tearing.
      • Malaise (general discomfort).
    • Diagnosis is based on history and clinical evaluation.
    • Treatment includes drainage, antibiotics, and sometimes surgery.

    Acute Bronchitis

    • Inflammation of the trachea and bronchi, short-duration and self-limiting with minimal pulmonary signs.
    • Can result from chemical irritation (smoke, fumes, gas) or viral infections (influenza, measles, chickenpox, whooping cough) or bacterial infections.
    • Clinical manifestations may include those of an upper respiratory infection (URI) like:
      • A cold.
      • Fever.
      • Dry, irritating cough.
      • Sore throat.
      • Laryngitis.
      • Chest pain.
      • Productive cough (with phlegm).
      • Wheezing.
    • Treatment is symptomatic, and vaccination may be recommended for certain infections.
    • Prognosis is usually good, with complications like pneumonia possible.

    Pneumonia

    • Inflammation of the lungs, also referred to as pneumonitis.
    • Usually caused by infection (bacterial, viral, fungal) through inhalation or aspiration.
    • Can affect one or both lungs.
    • Very common - over 4 million cases diagnosed yearly in the US.
    • A leading cause of death worldwide.
    • Children are more likely to get viral pneumonia, while adults are more likely to get bacterial pneumonia.
    • Risk factors:
      • Smoking.
      • Acute respiratory infections.
      • Chronic bronchitis.
      • Diabetes.
      • Chronic or critical illness.
      • Immune deficiency.
      • Infants.
      • Elderly.
      • Disabled individuals.
      • Altered consciousness.
      • Periodontal disease.
      • Difficulty swallowing.
    • Classification:
      • By Acquisition:
        • Community-acquired: Streptococcus pneumoniae (most common), Haemophilus influenza.
        • Hospital-acquired: Staphylococcus aureus (often affecting immunocompromised individuals).
      • By Area Involved:
        • Alveolar pneumonia: Involves the alveoli.
        • Interstitial pneumonia: Involves the septa.
        • Bronchopneumonia: Limited to segmental bronchi.
        • Lobar pneumonia: Widespread or diffuse.
    • Pathogens:
      • Upper respiratory flora: Streptococcus, Staphylococcus, Haemophilus.
      • Enteric saprophytes: Candida albicans (rare).
      • Extraneous pathogens: Mycobacterium tuberculosis, viruses.
    • Duration:
      • Acute.
      • Chronic.
      • Recurrent.
    • Routes of Infection:
      • Inhalation of pathogens in air droplets.
      • Aspiration of infected secretion from URT (upper respiratory tract).
      • Aspiration of infected particles from the GI (gastrointestinal) tract.
      • Hematogenous spread (from sepsis).
    • Pathogenesis:
      • Chronic inflammation, especially with unresponsive pneumonia, leading to destruction of lung parenchyma and fibrosis ("honeycomb lungs" on x-ray).

    Forms of Pneumonia

    Pneumocystis Jirovecii Pneumonia (PCP)

    • Often fatal fungal pneumonia if left untreated.
    • Etiology: Idiopathic, with the source of the fungus unknown.
    • Risk factors: Immunosuppression, chemotherapy, transplantation, malnutrition.
    • Clinical manifestations: Fever, impaired gas exchange, progressive dyspnea (difficulty breathing), fatigue, weight loss, cough, pneumothorax (collapsed lung).
    • Diagnosis involves laboratory tests.
    • Treatment consists of antifungal medications.

    Legionnaire's Disease

    • Rare, infectious disease caused by Legionella pneumophila.
    • Can cause widespread consolidation and necrosis of lung parenchyma, potentially fatal.
    • Symptoms include severe fever, nausea, vomiting, headache, chills, disorientation, and others.

    Pulmonary TB

    • Pulmonary TB is an infectious, inflammatory systemic disease of the lungs that may disseminate to lymph nodes and other organs
    • It can be primary or secondary
    • Primary infection is usually asymptomatic and may become chronic (latent)
    • Secondary TB – develops when the primary infection becomes active due to lowered resistance
    • 2 billion cases worldwide
    • Highest incidence in SE Asia, Africa, Eastern Europe
    • Etiology or cause: Mycobacterium tuberculosis
    • Risk factors:
      • Immunocompromised
      • Elderly
      • Drug or alcohol use
      • Malnutrition
      • Poor health
      • Infants and children
      • Chronic diseases (AIDS)
      • Smoking - increases the risk of advanced disease
    • Transmission:
      • Overcrowding
      • Prevalence in other areas of the world
        • Ethnicity
        • Immigrants
        • Low socioeconomic status (poor access to healthcare)
    • Pathogenesis:
      • Inhalation of M. tuberculosis enters the lungs
      • Macrophages and lymphocytes release cytokines
      • Macrophages transform into epithelioid cells
      • Multinucleated giant cells
      • Cluster around to form granulomas with caseous necrosis (type 4 hypersensitivity reaction)
    • Clinical Manifestations
      • Delayed, insidious and non-specific
      • Productive cough
      • Hemoptysis
      • Weight loss
      • Fever
      • Night sweats
      • Fatigue
      • Malaise
      • Anorexia
    • Diagnosis:
      • History
      • Physical Exam
      • Tuberculin skin test
      • Culture sputum
    • Treatment:
      • Medications (antibiotics – 9 months)

    Lung Abscess

    • Caused by aspiration of oral secretions in patients with gingivitis or poor oral hygiene
    • Typically patients have altered consciousness from alcohol intoxication, illicit drugs, anesthesia, sedatives, etc.
    • Older patients and those unable to handle their oral secretions due to neurological issues are at risk.
    • Most common pathogens: anaerobic bacteria, but can include aerobic bacteria
    • Most common aerobic pathogens: streptococci and staphylococci
    • Immunocompromised patients may have infection with mycobacteria or fungi
    • Pathogenesis:
      • Introduction of pathogens into the lungs causes inflammation, which leads to tissue necrosis and then abscess formation
      • Abscess usually ruptures into a bronchus, and its contents are expectorated, leaving an air- and/or fluid-filled cavity
      • Abscesses tend to connect with other airways and erode bronchial walls.

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    Explore the key aspects of cystic fibrosis, an inherited genetic disorder that primarily affects the respiratory, digestive, and reproductive systems. This quiz covers its clinical manifestations, genetic causes, and prevalence, providing insight into the challenges faced by those with this condition.

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