Acute Epiglottises

Definition:
Acute inflammation in the supraglottic region of the oropharynx with inflammation of the epiglottis. Now more common in adults than children due to the vaccination against H. influenzae. It is considered a true emergency.

Etiology/Pathogenesis:
May be caused by H. influenzae. Adult epiglottitis is typically a disease of men. More common in countries that do not vaccinate against H. influenzae.

Pertinent Historical Findings/Clinical Symptoms:

Severe sore throat Odynophagia/Dysphagia Fever Muffled voice Adults may have preceding upper respiratory infection symptoms Drooling

Pertinent Physical Exam Findings:

Fever Drooling/inability to handle secretions Cervical adenopathy Stridor: a late finding indicating advanced airway obstruction. Tripod position Muffled voice aka Hot potato voice Respiratory distress Larynx: erythema, swelling, exudates

Differential Diagnosis:
Croup (barky cough with URI symptoms) Bacterial tracheitis (toxic, fever, URI, cough) Foreign body aspiration Laryngitis Peritonsillar or retropharyngeal abscess

Diagnostic Evaluations:
Lateral neck X-ray: thumb sign (swollen epiglottis) Confirmed in operating room with direct visualization

Medical Management:
IV antibiotics: cefotaxime (2g every 6hrs) of ceftriaxone (1-2g per day) Secure airway (intubate if needed) Corticosteroids: dexamethasone (0.6 mg/kg given once)

Surgical Management
Consider cricothyrotomy in severe cases when endotracheal intubation fails.

Emergency Management
Endotracheal intubation should be performed in the operating room by a skilled anesthesiologist. Avoid using tongue depressor due to risk of spasm and airway obstruction

Patient Education/Maintenance-Prevention
H. Influenzae vaccine

Asthma
Definition:
A chronic inflammatory disorder of the airways in which many cells and cellular elements play a role, in particular, mast cells, eosinophils, T lymphocytes, macrophages, neutrophils, and epithelial cells. It affects 5-12% of adults and up to 15% of children worldwide.

Etiology/Pathogenesis:
It is extremely common, affecting boys more commonly than girls and, after puberty, women more than men. It equalizes between the sexes in adulthood. Most cases begin before the age of 25, although it can develop at any age. The reason for its prevalence is unknown, but may be related to the industrialized lifestyle many countries have adopted. There are both genetic and environmental factors related to asthma. Pathology is characterized by edema and hyperemia of the mucosa and inflammation of the mucosa with mast cells, eosinophils, lymphocytes bearing the Th2 phenotype, and CD1d-restricted natural killer T cells.

Pertinent Historical Findings/Clinical Symptoms:

During an acute attack, patients experience dyspnea with cough, wheezing, tightness of chest and anxiety. Dyspnea can be induced by exercise, aspirin ingestion, or allergen exposure. It can also occur due to an unidentifiable reason, referred to as intrinsic asthma.

Pertinent Physical Exam Findings:

Rapid respiratory rate (often 25-40 breaths per minute) Tachycardia Pulsus paradoxus (an exaggerated inspiratory decrease in systolic pressure) Oxygen saturation is common- 90%. Use of accessory muscles of ventilation can lead to skin over thorax being retracted into the intercostal spaces during inspiration. Chest hyperinflated with expiratory phase prolonged relative to inspiratory phase. Hyperresonance on percussion Diminished tactile fremitus Wheezing on auscultation Adventitious sounds may include rhonchi or rales Loss of intensity or absence of breath sounds indicates severe airflow obstruction. Decreased peak expiratory flow (PEF) and forced expiratory volume in 1 second (FEV1) are frequently seen. Concomitant allergic rhinitis findings are frequently seen. Up to 80% of patients with atopic dermatitis have some form of asthma.

Differential Diagnosis:
Bronchitis or bronchiolitis Exacerbation of COPD Pneumonia Allergic rhinitis or sinusitis Foreign body Upper airway obstruction Pulmonary embolism Congestive heart failure Cystic fibrosis Any condition that produces obstructive airflow can be considered although careful history and physical examination may narrow the list.

Diagnostic Evaluations:
Pulmonary function testing indicates decrease in airflow rates throughout the vital capacity. Peak expiratory flow rate (PEFR), forced expiratory volume (FEV1), and max mid-expiratory flow rate (MMEFR) will all decrease. In patients with normal spirometry, provocation testing using methacoline or histamine will show obstructive airflow and help make the diagnosis. Skin allergen testing may be helpful, as this may identify exacerbating factors. Often, history and physical exam are enough to make presumptive diagnosis.

Medical Management:
Patient logs of peak expiratory flows are important to see trends and management exacerbations early. Treatment depends on severity and frequency of exacerbations. Mild intermittent asthma: inhaled 2 (albuterol) should be used less than twice a week. Mild to moderate persistent asthma: daily anti-inflammatories are crucial for preventing long-term airway disease. Inhaled corticosteroids are safe and most effective. Severe asthma: higher doses of inhaled corticosteroid and inhaled long acting bronchodilators (LABA). Leukotriene receptor antagonists are used as first-line drugs for mild persistent asthma, but their efficacy is not as great as inhaled steroids. Combination products with both inhaled corticosteroid and LABA in the same aerosol device are available and increase the convenience of dosing and administration; however, may not be cost-effective. Nebulized 2 agonists and ipratropium bromide are useful during periods of exacerbation.

Surgical Management
Not applicable.

Emergency Management
In severe exacerbations, emergency measures, such as intubation, may be necessary.

Patient Education/Maintenance-Prevention
Limit allergen or irritant exposure Home peak flow monitoring is essential as well as an action plan to guide care. Proper use of inhalers (spacers) is important. Annual influenza and pneumococcal vaccine.

Chronic Bronchitis
Definition:
A clinical diagnosis, designed as the presence of chronic cough and sputum production for at least 3 months of the year for at least 2 consecutive years in the absence of any other disease.

Etiology/Pathogenesis:
One third of smokers aged 35 to 59 years have chronic bronchitis, and its prevalence increases with age. Anatomic basis is hypertrophy and hyperplasia of the mucus-secreting glands normally found in the epithelium of larger airways. Airway structural changes including atrophy, focal squamous metaplasia, decreased ciliary function, airway smooth muscle hyperplasia, inflammation, bronchial wall thickening, neutrophilia in the airway lumen, neutrophilic infiltrates accumulate in the submucosa.

Pertinent Historical Findings/Clinical Symptoms:

Mild dyspnea Chronic cough productive of sputum Periodic exacerbations Fatigue, fever, malaise Chest discomfort Weight gain

Pertinent Physical Exam Findings:

Increased breathing rate with use of accessory respiratory muscles Patients may have signs of right-sided HF: cyanosis and peripheral edema Decreased level of consciousness Rhonchi and wheezing Frequently overweight Blue Bloaters

Differential Diagnosis:
Asthma Pneumonia Bronchiectasis Congestive heart failure (Note: Because they share many of the same physical signs, COPD may be difficult to distinguish from CHF). One crude bedside test for distinguishing COPD from CHF is peak expiratory flow. If patients blow 150-300 mL or less, they are probably having a COPD exacerbation; higher flows indicate a probably CHF exacerbation.

Pulmonary fibrosis Cystic fibrosis Bronchopulmonary mycosis

Diagnostic Evaluations:
CXR: cardiomegaly; increased interstitial markings, diaphragm not flattened. Sputum stain and culture Total lung capacity normal to slightly elevated. Hemoglobin usually elevated (15 to 18 g/dL). PaO2 reduced (45 to 60 mmHg); PaCO2 slightly to markedly elevated (50 to 60 mmHg).

Medical Management:
Corticosteroids for acute exacerbations Oxygen to relieve hypoxia Bronchodilator: SABA In addition, short-acting anticholinergics, such as ipratropium, can be given.

Surgical Management
Not applicable

Emergency Management
Oxygen therapy as needed. Recognize and treat any signs of respiratory distress or failure. Recognize and treat secondary pneumonia.

Patient Education/Maintenance-Prevention
Cessation of smoking Polyvalent pneumococcal and influenza vaccine

Cystic Fibrosis
Definition:
An autosomal recessive genetic disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator. Relatively common, affecting 1 in 2000 to 3000 whites; 1 in 20 to 25 are carriers. Causes chronic pulmonary infections.

Etiology/Pathogenesis:
Median survival of over 35 years in the US. There is an abnormal transfer of Na and Cl. Patients produce thick mucus, which is difficult to clear. Most men are infertile and women have difficulty conceiving. Persistent bacterial airway infection, especially with Pseudomonas aeruginosa, is the hallmark of lung disease in CF. CF causes a defect in host defense restricted to the lung; patients are not predisposed to infection elsewhere, and normal transplanted lungs are not subject to the same infections. In the respiratory tract, mucociliary function is impaired due to inadequate hydration of the tracheobronchial epithelium causing increased sputum viscosity. Obstruction results in glandular dilation and tissue damage.

Pertinent Historical Findings/Clinical Symptoms

CF causes sinusitis, bronchiectasis, pancreatic insufficiency with occasional portal hypertension, and secondary clubbing. In infants, it causes meconeum ileus (10%) and intussusception. Nasal polyposis, sinusitis, and recurrent pneumonias are common. Tendency to get staphylococcal and pseudomonal infections. Note: If a male patient has fathered a child, he does NOT have CF. Persistent cough Sputum production Wheezing Steatorrhea/Diarrhea Decreased exercise tolerance Abdominal pain Inability to gain weight Sinus pain or pressure and purulent nasal discharge Recurrent hemoptysis

Pertinent Physical Exam Findings

Nose: Rhinitis, nasal polyps, sinus tenderness Pulmonary System: tachypnea, respiratory distress with retractions, wheeze or crackles, cough, increased AP diameter, clubbing cyanosis, hyperresonant chest. GI: abdominal distention, hepatosplenomegaly, rectal prolapse, dry skin, cheilosis (Vitamin B complex deficiency). Other: scoliosis, kyphosis, swelling of submanibular gland or parotid gland, aquagenic wrinkling of the palms.

Differential Diagnosis
Asthma Bronchiectasis/Bronchiolits Sinusitis Recurrent pneumonia

Diagnostic Evaluations
Sweat Chloride test: increase Na and Cl in sweat (assessed using pilocarpine iontophoresis and should always be repeated); Cl concentration>60mEq/L with major clinical manifestations. Genetic testing: when CF suspected with negative sweat test. CXR: hyperinflation, peribronchial cuffing (which creates linear opacities). Bronchietasis changes as disease progresses. Pneumothorax, atelectasis, mucus plugging, small rounded peripheral opacities may be present. PFT: reduced airflow rates; decreased FEV1, FVC, TLC and maximal midexpiratory flow; increased residual volume to total lung capacity; decreased PO2; increased PCO2 late in disease. Mixed obstructive and restrictive pattern; reduction in pulmonary diffusing capacity. Arterial blood gas studies: hypoxemia; chronic, compensated respiratory acidosis in advanced disease. Chest computed tomography (CT) scan: confirms the presence of bronchiectasis.

Medical Management
Inhaled bronchodilators and corticosteroids for airway obstruction. Antibiotics: to treat lung infections Aerosolized tobramycin for suppression of chronic pulmonary infections. Aerosolized recombinant human DNase for persistent airway secretions. Chest physiotherapy (airway clearance techniques, chest percussion, postural drainage, or vibration technique). Pancreatic enzyme replacement if indicated. Supplemental vitamins (A, D, E, K) as needed.

Surgical Management
Bilateral lung transplantation- 50% 3 year survival rate Heart or lung transplantation if indicated

Emergency Management
Recognize and treat any signs of respiratory distress or failure. Provide supplemental oxygen as needed.

Patient Education/Maintenance- Prevention

CF centers that intensely monitor and engage the patient have better outcomes than elsewhere. More frequent monitoring of sputum cultures and administration of antibiotics are associated with slower decrease in lung function. Keep immunization up to date: pneumococcal and annual influenza. Cessation of smoking Regular exercise to loosen mucus in airways Prenatal testing for CF genetics available; most useful in those with family history. Nutritional and psychosocial support: genetic and occupational counseling. Avoid sick contacts during flu season.

Emphysema
Definition:
Enlargement of air spaces distal to the conducting airways (respiratory bronchioles and alveoli) due to destruction of the walls of these air spaces.

Etiology/Pathogenesis:
Two types: centrilobular and panacinar. Centrilobular: involves respiratory bronchioles with normal distal alveoli (may be damaged in severe disease); seen in smokers occurring in upper lobes. Panacinar: involves the entire distal lung unit; may occur anywhere but usually in lower lobes. Alpha 1-Antitrypsin deficiency plays a major role in familial cases. AAT is an acute phase serum protein secreted by the liver and binds to and neutralizes neutrophil elastase. Major contributors: excessive lysis of elastin in the lung, cigarette smoking, and pollutants.

Pertinent Historical Findings/Clinical Symptoms:

Long history of dyspnea with late onset of nonproductive cough Decreased exercise capacity Mild cough (uncommon) Occasional mucopurulent relapses; Sputum usually scant, clear, or mucoid. Usually presents after age 50. Eventual cachexia and respiratory failure.

Pertinent Physical Exam Findings:

Tachypnea Noncyanotic Thin with barrel chest Diminished breath sounds Little or no cough or expectoration Pursed-lip breathing and use of accessory respiratory muscles with tripod sitting position.

Chest hyperresonant with possible wheezing heard. Heart sounds are distant. Pink Puffers Increased expiratory phase No adventitious sounds No peripheral edema

Differential Diagnosis:
Bronchiectasis Bronchitis Asthma Cystic Fibrosis Bronchopulmonary mycosis Central airflow obstruction Carcinoid tumor

Diagnostic Evaluations:
Arterial blood gas analysis: mild to moderate hypoxemia Hematocrit value: chronic hypoxemia may lead to polycythemia. H> than 52% in men and > than 47% in women. Sputum evaluation: to assess for secondary bacterial infection. CXR: hyperinflation and flattening of diaphragms, increased retrosternal airspace, parenchymal bullae, and long narrow heart shadow. CT: more sensitive and specific for emphysema outlining bullae. PFT: necessary for diagnosis and assessment of severity. Spirometry: helpful in assessing responses to treatment and disease progression.

Medical Management:
Bronchodilators: anticholinergics (ipratropium bromide), SABA (albuterol, Xopenex), work by dilating airways and thereby decreasing airflow resistance. This increases airflow and decreases dynamic hyperinflation; provide symptomatic relief but do not alter disease progression or decrease mortality. Corticosteroids for acute exacerbations (fluticasone, budesonide). Oxygen therapy indicated if: PO2 less than 55 mm, desaturation with exercise, polycythemia.

Surgical Management
Bullectomy Lung volume reduction surgery: attempts to decrease hyperinflation by surgically resecting the most diseased parts of the lung. This improves airflow by increasing the elastic recoil of the remaining lung and the mechanical efficiency of the respiratory muscles to generate expiratory pressures. Lung transplantation

Emergency Medicine
Recognize and treat any signs of respiratory distress or failure. Recognize and treat secondary pneumonia. Provide supplemental oxygen as needed.

Patient Education/Maintenance- Prevention

Smoking cessation Remain up to date with vaccinations: Pneumococcal and influenza.