5 курс / Пульмонология и фтизиатрия / Clinical_Manifestations_and_Assessment_of_Respiratory

4. Dornase alpha

a.1 only

b.2 only

c.3 and 4 only

d.1, 2, and 4 only

9.With regard to the secretion of sodium and chloride, the sweat glands of patients with cystic fibrosis secrete up to:

a.2 times the normal amount

b.4 times the normal amount

c.7 times the normal amount

d.10 times the normal amount

10.Which of the following clinical manifestations are associated with severe cystic fibrosis?

1.Decreased hemoglobin concentration

2.Increased central venous pressure

3.Decreased breath sounds

4.Increased pulmonary vascular resistance

a.1 and 3 only

b.2 and 3 only

c.3 and 4 only

d.2, 3, and 4 only

1Cystic fibrosis does not affect the lungs exclusively. It also affects the function of exocrine glands in other parts of the body. In addition to being characterized by abnormally viscid secretions in the lungs, the disease is clinically manifested by male impotence, pancreatic insufficiency and high chloride concentrations in the sweat.

2CF Foundation (http://www.cff.org).

3CF Foundation (http://www.cff.org).

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C H A P T E R 1 6

Bronchiectasis

CHAPTER OUTLINE

Anatomic Alterations of the Lungs

Varicose Bronchiectasis (Fusiform Bronchiectasis)

Cylindrical Bronchiectasis (Tubular Bronchiectasis) Cystic Bronchiectasis (Saccular Bronchiectasis)

Etiology and Epidemiology Diagnosis

Overview of the Cardiopulmonary Clinical Manifestations Associated With Bronchiectasis

General Management of Bronchiectasis

Respiratory Care Treatment Protocols

Medications Commonly Prescribed by the Physician

Expectorants Administration of Antibiotics

Case Study Bronchiectasis

Self-Assessment Questions

CHAPTER OBJECTIVES

After reading this chapter, you will be able to:

•Describe the anatomic alterations of the lungs associated with bronchiectasis.

•Discuss the etiology and epidemiology of bronchiectasis.

•Identify the common classifications used to group the causes of bronchiectasis and include specific examples under each classification.

•Describe the various diagnostic tests used to identify the presence of bronchiectasis.

•Describe the cardiopulmonary clinical manifestations associated with bronchiectasis.

•Describe the general medical and surgical management of bronchiectasis.

•Describe the respiratory care modalities used in the treatment of bronchiectasis.

•Describe and evaluate the clinical strategies and rationales of the SOAPs presented in the case study.

•Define key terms and complete self-assessment questions at the end of the chapter and on Evolve.

KEY TERMS

Acquired Bronchial Obstruction

Bronchography

Congenital Anatomic Defects

Cylindrical (Tubular) Bronchiectasis

Cystic (Saccular) Bronchiectasis

High-Frequency Chest Compression Devices

High-Resolution Computed Tomogram (HR-CT)

Kartagener Syndrome

Lung Mapping

Noncystic Fibrosis Bronchiectasis (NCFB)

Pneumovest

Primary Ciliary Dyskinesia

Reid Classification

Varicose (Fusiform) Bronchiectasis

Anatomic Alterations of the Lungs

Bronchiectasis is an acquired disorder of the major bronchi and bronchioles characterized by chronic dilation and

distortion of one or more bronchi, usually as a result of extensive inflammation and destruction of the bronchial wall cartilage, blood vessels, elastic tissue, and smooth muscle components. One or both lungs may be involved. Bronchiectasis is commonly limited to a lobe or segment and is frequently found in the lower lobes. The smaller bronchi, with less supporting cartilage, are predominantly affected.

Because of bronchial wall destruction, normal mucociliary clearance is impaired. This results in the accumulation of copious amounts of bronchial secretions and blood that often become foul-smelling because of secondary colonization with anaerobic organisms. Infection and irritation may lead to secondary bronchial smooth muscle constriction and fibrosis. The small bronchi and bronchioles distal to the affected areas become partially or totally obstructed with secretions. This condition leads to one or both of the following anatomic alterations: (1) hyperinflation of the distal alveoli as a result of expiratory check-valve obstruction or (2) atelectasis, consolidation, and fibrosis as a result of complete bronchial obstruction.

Based on gross anatomic appearance, the long-accepted Reid classification subdivides bronchiectasis into the following three patterns:

•Varicose (fusiform)

•Cylindrical (tubular)

•Cystic (saccular)

Varicose Bronchiectasis (Fusiform Bronchiectasis)

In varicose (fusiform) bronchiectasis, the bronchi are dilated and constricted in an irregular fashion similar to varicose veins, ultimately resulting in a distorted, bulbous shape (Fig. 16.1A).

FIGURE 16.1 Bronchiectasis. (A) Varicose bronchiectasis. (B) Cylindrical bronchiectasis. (C) Cystic (saccular) bronchiectasis. Also illustrated are excessive bronchial secretions (D) and atelectasis (E), which are both common anatomic alterations of the lungs in this disease.

Cylindrical Bronchiectasis (Tubular Bronchiectasis)

In cylindrical (tubular) bronchiectasis, the bronchi are dilated and rigid and have regular outlines similar to a tube. X- ray examination shows that the dilated bronchi fail to taper for 6 to 10 generations and then appear to end abruptly because of mucous obstruction (see Fig. 16.1B).

Cystic Bronchiectasis (Saccular Bronchiectasis)

In cystic (saccular) bronchiectasis, the bronchi progressively increase in diameter until they end in large, cystlike sacs in the lung parenchyma. This form of bronchiectasis causes the greatest damage to the tracheobronchial tree. The bronchial walls become composed of fibrous tissue alone—cartilage, elastic tissue, and smooth muscle are all absent (see Fig. 16.1C).

The following are the major pathologic or structural changes associated with bronchiectasis:

•Chronic dilation and distortion of bronchial airways

•Excessive production of often foul-smelling sputum (see Fig. 16.1D)

•Bronchospasm

•Hyperinflation of alveoli (air trapping)

•Atelectasis (see Fig. 16.1E)

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deficiency, CD40 ligand deficiency, and others)

Abnormal Secretion Clearance

AFB, Acid-fast bacilli; COPD, chronic obstructive pulmonary disease; CT, computed tomography; Ig, immunoglobulin; MRI, magnetic resonance imaging; PFT, pulmonary function test; PPD, percussion and postural drainage.

Modified from Wolters Kluwer Health/UpToDate.com: Clinical Manifestations and diagnosis of bronchiectasis in adults. Accessed March 25, 2017.

Diagnosis

A routine chest radiograph may reveal such findings as overinflated lungs or marked volume loss, increased opacities, dilated fluid-filled airways, crowding of the bronchi, and atelectasis. Although bronchoscopy is rarely performed today, bronchograms can confirm cylindrical, cystic, or varicose bronchiectasis, as well as crowding of the bronchi, loss of bronchovascular markings, and, in more severe cases, honeycombing, air-fluid levels, and fluid-filled nodules. Bronchoscopy once was the absolute gold standard for the diagnosis of NCFB.

Today, the high-resolution computed tomogram (HR-CT) scan has virtually replaced bronchography (see Computed Tomography Scan later in this chapter) as the best tool for diagnosing NCFB. The diagnosis is made on the basis of the internal diameter of a bronchus that is wider than its adjacent pulmonary artery, a failure of the bronchi to taper, and the visualization of bronchi in the outer 1 to 2 cm of the lung fields. The HR-CT scan is used to better clarify the findings from the chest radiograph and standard CT scans, and allows lung mapping of airway abnormalities that cannot be identified on routine films of the chest.

Spirometry testing can be used to determine if the bronchiectasis demonstrates primarily an obstructive or restrictive lung pathophysiology, and arterial blood gas measurements can confirm if the patient has mild, moderate, or severe gas exchange compromise.

Overview of the Cardiopulmonary Clinical Manifestations Associated With Bronchiectasis

The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by excessive bronchial secretions (see Fig. 10.11), bronchospasm (see Fig. 10.10), atelectasis (see Fig. 10.7), consolidation (see Fig. 10.8), and increased alveolar-capillary membrane thickness (see Fig. 10.9), which are the major anatomic alterations of the lungs associated with bronchiectasis (see Fig. 16.1).

Clinical Data Obtained at the Patient's Bedside

Depending on the amount of bronchial secretions and the degree of bronchial destruction and fibrosis/atelectasis associated with bronchiectasis, the disease may create an obstructive or a restrictive lung disorder or a combination of both. If the majority of the bronchial airways are only partially obstructed, the bronchiectasis manifests primarily as an obstructive lung disorder. If, by contrast, the majority of the bronchial airways are completely obstructed, the distal alveoli collapse, atelectasis results, and the bronchiectasis manifests primarily as a restrictive disorder. Finally, if the disease is limited to a relatively small portion of the lung—as it often is—the patient may not have any of the following typical clinical manifestations of bronchiectasis.

The Physical Examination

Vital Signs

Increased Respiratory Rate (Tachypnea)

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Several pathophysiologic mechanisms operating simultaneously may lead to an increased frequency of breathing (respiratory rate [RR]):

•Stimulation of peripheral chemoreceptors (hypoxemia)

•Decreased lung compliance and increased ventilatory rate relationship

•Anxiety

Increased Heart Rate (Pulse) and Blood Pressure Use of Accessory Muscles During Inspiration Use of Accessory Muscles During Expiration

Pursed-Lip Breathing (When Pathology Is Primarily Obstructive in Nature)

Increased Anteroposterior Chest Diameter (Barrel Chest) (When Pathology Is Primarily Obstructive in Nature)

Cyanosis Digital Clubbing

Peripheral Edema and Venous Distention

Because polycythemia and cor pulmonale are associated with severe bronchiectasis, the following may be seen:

•Distended neck veins

•Pitting edema

•Enlarged and tender liver

Cough, Sputum Production, and Hemoptysis

Chronic cough with production of large quantities of foul-smelling sputum is a hallmark of bronchiectasis. A 24-hour collection of sputum is usually voluminous and tends to settle into several different layers. Streaks of blood are seen frequently in the sputum, presumably originating from necrosis of the bronchial walls and erosion of bronchial blood vessels. Frank hemoptysis also may occur occasionally, but it is rarely life-threatening. Because of the excessive bronchial secretions, secondary bacterial infections are frequent. Haemophilus influenzae, Streptococcus, Pseudomonas aeruginosa, and various anaerobic organisms are commonly cultured from the sputum of patients with bronchiectasis.

The productive cough seen in patients with bronchiectasis is triggered by the large amount of secretions that fill the tracheobronchial tree. The stagnant secretions stimulate the subepithelial mechanoreceptors, which in turn produce a vagal reflex that triggers the cough. The subepithelial mechanoreceptors are found in the trachea, bronchi, and bronchioles, but they are predominantly located in the upper airways.

Chest Assessment Findings

When the bronchiectasis pathologic factors are primarily obstructive:

•Decreased tactile and vocal fremitus

•Hyperresonant percussion note

•Diminished breath sounds

•Wheezing

•Crackles

When the bronchiectasis pathologic factors are primarily restrictive (over areas of atelectasis and consolidation):

•Increased tactile and vocal fremitus

•Bronchial breath sounds

•Crackles

•Whispered pectoriloquy

•Dull percussion note

Clinical Data Obtained From Laboratory Tests and Special Procedures

Pulmonary Function Test Findings

Moderate to Severe Bronchiectasis (When Primarily Obstructive Lung Pathophysiology)

Forced Expiratory Volume and Flow Rate Findings

Lung Volume and Capacity Findings

Pulmonary Function Test Findings

Moderate to Severe Bronchiectasis (When Primarily Restrictive Lung Pathophysiology)

Forced Expiratory Flow Rate Findings

Lung Volume and Capacity Findings

Arterial Blood Gases

Bronchiectasis

Mild to Moderate Stages

Acute Alveolar Hyperventilation With Hypoxemia1 (Acute Respiratory Alkalosis)

1See Fig. 5.2 and Table 5.4 and related discussion for the acute pH, PaCO2, and changes associated with acute alveolar hyperventilation.

Severe Stage

Chronic Ventilatory Failure With Hypoxemia2 (Compensated Respiratory Acidosis)

2See Table 5.6 and related discussion for the pH, PaCO2, and changes associated with chronic ventilatory failure.

Acute Ventilatory Changes Superimposed on Chronic Ventilatory Failure3

Because acute ventilatory changes are frequently seen in patients with chronic ventilatory failure, the respiratory therapist must be familiar with and alert for the following two dangerous arterial blood gas (ABG) findings:

•Acute alveolar hyperventilation superimposed on chronic ventilatory failure, which should further alert the respiratory therapist to document the following important ABG assessment: possible impending acute ventilatory failure

•Acute ventilatory failure (acute hypoventilation) superimposed on chronic ventilatory failure

3See Table 5.7, Table 5.8, and Table 5.9 and related discussion for the pH, PaCO2, and changes associated with acute ventilatory changes superimposed on chronic ventilatory failure.

Oxygenation Indices4

Bronchiectasis Moderate to Severe Stages

5The DO2 may be normal in patients who have compensated to the decreased oxygenation status with (1) an increased cardiac output, (2) an increased hemoglobin level, or (3) a combination of both. When the DO2 is normal, the O2ER is usually normal.

4, Arterial-venous oxygen difference; DO2, total oxygen delivery; O2ER, oxygen extraction ratio; QS/QT, pulmonary shunt fraction; , mixed venous oxygen saturation; VO2, oxygen consumption.

Hemodynamic Indices6

Bronchiectasis Moderate to Severe Stages

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FIGURE 16.2 Gross cystic bronchiectasis. Posteroanterior chest radiograph showing overinflated lungs. There are multiple ring opacities, most obvious at the lung bases, ranging from 3 to 15 mm in diameter. (From Hansell, D. M., Lynch, D. A., McAdams, H. P., et al.

[2010]. Imaging of diseases of the chest [5th ed.]. Philadelphia, PA: Elsevier.)

When the bronchiectasis is primarily restrictive:

•Atelectasis and consolidation

•Infiltrates (suggesting pneumonia)

•Increased opacity

In generalized bronchiectasis, such as commonly seen in cystic fibrosis, there is usually overinflation of the lungs. However, when the bronchiectasis is localized, the chest radiograph often reveals restrictive pathologic conditions such as atelectasis, consolidation, or infiltrates. When atelectasis and consolidation develop as a result of bronchiectasis, an increased opacity and reduced lung volume are seen in these areas on the radiograph. For example, Fig. 16.3 illustrates a marked volume loss in a patient with left lower lobe bronchiectasis.

Left lower lobe bronchiectasis. The marked volume loss of the left lower lobe is indicated by a depressed hilum, vertical left mainstem bronchus, mediastinal shift, and left-sided transradiancy.

[2010]. Imaging of diseases of the chest [5th ed.]. Philadelphia, PA: Elsevier.)

Bronchogram

In the past, bronchography (the injection of an opaque contrast material into the tracheobronchial tree) was routinely

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