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FIFTH EDITION
PATHOPHYSIOLOGY
Lee-Ellen C. Copstead, PhD, RN
Jacquelyn L. Banasik, PhD, ARNP
Professor
Department of Nursing
College of Nursing and Health Sciences
University of Wisconsin-Eau Claire
Eau Claire, Wisconsin
Associate Professor
College of Nursing
Washington State University
Spokane, Washington
ELSEVIER
512
UNIT VI Respiratory Function
gastric volume and size has proved successful in some patients. These
operations are intended to permanently curtail food intake.
KEY POINTS
• Neuromuscular diseases affect the muscles of respiration, leading to mus-
cular weakness, increased risk of pulmonary infections, and respiratory
failure.
• Kyphoscoliosis is a deformity of the bony structure of the chest wall char-
acterized by hunchback and lateral curvature of the spine. The abnormal
shape of the chest interferes with the normal mechanics of breathing,
resulting in small lung volumes, compression atelectasis, and hypoxemia.
Compensatory tachypnea is usually present.
• Ankylosing spondylitis is a progressive inflammatory disease affecting
vertebrae and ribs. Chronic inflammation leads to chest wall fibrosis and
immobility. Chest wall muscle atrophy and rib cage stiffening result in pul-
monary dysfunction characteristic of restrictive disorders.
Obesity may interfere with the normal mechanics of breathing because of
excessive chest weight and abdominal impingement on the chest cavity.
Pickwickian syndrome is a disorder of obesity associated with hypoventila-
tion and upper airway obstruction during sleep.
INFECTION OR INFLAMMATION OF THE LUNG
Disorders of Obesity
Etiology. Obesity is defined as excessive body fat, with a body mass
index (BMI) greater than 30 kg/m² based on body weight and height.
Overweight is defined as a BMI of 25 to 29.9 kg/m2.43 Obesity results
from excessive caloric intake and/or reduced caloric expenditure. The
National Health and Nutrition Exam survey reported that 59.4% of
men and 49.9% of women are overweight. The findings for obesity were
19.9% of men and 25.1% of women 44,45 A higher prevalence of obesity
was found in blacks than in whites and in persons with lower incomes
than in those with higher incomes.43-45 Obese patients are at risk for
a variety of disorders, the most common of which are diabetes mel-
litus, coronary artery disease, degenerative joint disease, gallstones, cer-
tain cancers (colon, rectum, and prostate in men; uterus, biliary tract,
breast, and ovary in women), and pulmonary impairment. Persons
with a BMI of 30 kg/m² have an all-cause increase in mortality of 50%
to 100% compared to persons with a BMI between 20 and 25 kg/m2.43
Pathogenesis. Endocrine causes of obesity are rare. 10 Hypothy-
roidism, the use of corticosteroids, and hypothalamic lesions all can
lead to weight gain; however, the major cause of obesity is excess
caloric intake in relation to caloric expenditure. Several hormones
act on brain receptors to regulate appetite and metabolism. Leptin
binds to brain receptors, causing the release of neuropeptides that
promote satiety and increase metabolic rate. Ghrelin stimulates appe-
tite. Genetic diseases such as familial partial lipodystrophy, Prader-
Willi syndrome, Laurence-Moon syndrome, Bardet-Biedl syndrome,
and Cohen syndrome are associated with obesity.43-46 Obesity may be
associated with hypoventilation. The mechanisms of obesity hypoven-
tilation are reduced ventilatory drive and increased work of breath-
ing. Some patients are thought to have an abnormality in the central
nervous system. In addition, the increased abdominal size can force
the abdominal contents upward into the chest cavity, thus decreasing
lung expansion and diaphragmatic shortening. Obesity hypoventila-
tion is also called pickwickian syndrome, named after the obese boy
in Pickwick Papers written by Charles Dickens. Pickwickian syndrome
is associated with hypoventilation and airway obstruction. An addi-
tional factor that contributes to the overall clinical picture in many
obese patients is upper airway obstruction during sleep, the obstruc-
tive form of sleep apnea syndrome. Soft-tissue deposits in the neck
and tissues surrounding the upper airway predispose the person to
episodes of complete upper airway obstruction during sleep. In a large
percentage of cases, the daytime somnolence that occurs in patients
who have the obesity hypoventilation syndrome is related to obstruc-
tive sleep apnea.44
Clinical manifestations. Obesity hypoventilation is character-
ized by decreased alveolar ventilation, somnolence, severe hypoxemia,
polycythemia, and cor pulmonale. Patients complain of daytime som-
nolence, impotence, shortness of breath, headache, and enuresis.
Diagnosis. The diagnosis of obesity is self-evident on examination.
Tests for hypothyroidism, Cushing syndrome, insulinoma, diabetes,
and hyperlipidemia may be done to identify comorbid factors.43 For
persons with hypoventilation, arterial blood gas analyses may reveal
hypoxemia and hypercapnia. Chest wall compliance, vital capacity,
total lung capacity, and expiratory reserve volume are all decreased.
Patients may also have an increased red blood cell count and show
signs and symptoms of cor pulmonale and pulmonary hypertension.
Treatment. Primary treatment for obesity consists of a weight loss
program that includes the family members. Caloric intake that pro-
motes an energy deficit of 500 to 1000 kcal/day is recommended.
Aerobic exercise preserves lean body mass and increases energy expen-
diture.43,44 Oxygen delivery through a nasal cannula or mechanical
ventilation may be necessary for patients with morbid obesity. Surgi-
cal intervention with gastric stapling or gastric bypass to decrease the
Pneumonia
Etiology. The term pneumonia (from the Greek pneuma, which
means "breath") refers to an inflammatory reaction in the alveoli
and interstitium of the lung, usually caused by an infectious agent.
Pneumonia can result from three different sources: (1) aspiration of
oropharyngeal secretions composed of normal bacterial flora and/
or gastric contents (25% to 35% of all pneumonias); (2) inhalation
of contaminants (virus, Mycoplasma); or (3) contamination from the
systemic circulation.2,47-49
There are several ways to classify pneumonia. Pneumonias are
typically classified as community acquired or hospital acquired. The
incidence of community-acquired pneumonia is 1 in 100 persons.
Approximately 15% to 20% of persons presenting with pneumonia
require hospitilization.50 Pneumonia is further classified as bacterial,
atypical, and viral. The bacterial pneumonias may be grouped as either
gram-positive or gram-negative, based on the staining characteristics
of the organism. Staphylococcus and Streptococcus (including pneumo-
cocci) are the predominant gram-positive organisms. Gram-negative
bacteria that may cause pneumonia include Haemophilus influenzae,
Klebsiella species, Pseudomonas aeruginosa, Serratia marcescens, Esch-
erichia coli, and Proteus species.
Patients at risk of pneumonia include the elderly; those with a dimin-
ished gag reflex; seriously ill, hospitalized patients; hypoxic patients;
and immunocompromised patients.S0 Anaerobic bacteria may pres-
ent clinically as a lung abscess, necrotizing pneumonia, or empyema.
These diseases are usually caused by aspiration of normal oral bacteria
(such as Bacteroides and Fusobacterium) into the lung. Mycoplasmal
pneumonia is more commonly seen in the summer and fall in young
adults. About half of the cases of pneumonia in persons between 5 and
20 years of age can be classified as mycoplasmal pneumonia. Other
causes of pneumonia occur less frequently in the general population.
Legionnaires disease, for example, is a severe systemic illness character-
ized by fever, diarrhea, abdominal pain, liver and kidney failure, and
pulmonary infiltrates. The causative organism for legionnaires disease
lives in water and is transmitted by means of potable water, condensers,
and cooling towers. The current treatment of choice is administration
of a macrolide antibiotic. Patients whose immune systems have been
CHAPTER 23 Restrictive Pulmonary Disorders
513
compromised by disease or by drug therapy may be susceptible to the
development of opportunistic pneumonia. 50 For example, Pneumocys-
tis (carinii) jiroveci pneumonia, an opportunistic fungal infection, is
commonly found in patients with cancer or with human immunodefi-
ciency virus (HIV). (See Chapter 12 for further discussion of acquired
immunodeficiency syndrome (AIDS).)
Aspergillus, an opportunistic fungus that is widespread in nature,
may cause progressive pneumonia. Aspergillus is released from the
walls of old buildings under reconstruction. Attention should be
given when old hospitals are renovated and when susceptible patients
are located in a reconstruction area.49 To assist the reader in differ-
entiating among the various types of pneumonia, Table 23-7 presents
the etiologic factors, common clinical features with age-related char-
acteristics, radiologic findings, and antibiotic therapies for 11 forms
of the disease. There are many other types of pneumonia that are
not listed.
Pathogenesis. Normally, pulmonary defense mechanisms
(immune responses, cough reflex, sneezing, mucociliary clearance)
protect individuals from pneumonia. Community-acquired pneumo-
nia occurs when defense mechanisms are compromised.2,46 A highly
virulent organism may also overwhelm a person's defense mecha-
nisms. Community-acquired pneumonias are commonly bacterial in
origin." After microbial agents enter the lung, they multiply and trig-
ger pulmonary inflammation. Alveolar air spaces fill with an exudative
fluid, and inflammatory cells invade the alveolar septa. Acute bacte-
rial pneumonia may be associated with significant /Q mismatching
and hypoxemia because inflammatory exudate collects in the alveolar
spaces. Alveolar exudate tends to consolidate and becomes difficult
to expectorate. Viral pneumonia does not produce exudative fluids.
Figure 23-9 shows the histologic progression of acute bacterial pneu-
monia. Patients with chronic illnesses and those who are immobile or
immunosuppressed or have a decreased level of consciousness are at
TABLE 23-7
DIFFERENTIATING FEATURES OF TYPES OF PNEUMONIA
CHEST RADIOGRAPH
ETIOLOGIC ORGANISM
Staphylococcus aureus, gram-
positive cocci in clumps
COMMON CLINICAL FEATURES
Follows upper respiratory tract infection;
fever, chills, pleuritic chest pain, cough,
yellow purulent sputum; seen in patients
in chronic care facilities
Consolidation, may have
cavitation
Patchy infiltrates
Streptococcus pneumoniae
(pneumococcus) gram-positive
diplococci
ANTIBIOTIC TREATMENT
Methicillin-susceptible strains: nafcillin or
oxacillin with or without rifampin; methicillin-
resistant strains: vancomycin with or without
rifampin; alternative choice: cephalosporins,
clindamycin, vancomycin
Procaine penicillin G or aqueous penicillin G,
amoxicillin; alternative choice: macrolides,
cephalosporins, doxycycline, quinolones;
prophylactic vaccine available
Cefotaxime, ceftriaxone, doxycycline,
azithromycin, TMP-SMX; alternative choice:
quinolones or clarithromycin
Consolidation
Haemophilus influenzae;
pleomorphic gram-negative
coccobacilli
More common in alcoholics; also seen
with chronic cardiopulmonary disease;
fever, chills, pleuritic chest pain, cough,
rust-colored sputum
Upper respiratory tract symptoms, fever,
vomiting, irritability, cough, purulent
sputum, dyspnea, affects children and
older adults; affects people with chronic
cardiorespiratory problems
Seen frequently in middle-aged men and
associated with alcoholism and diabetes
mellitus, rust-colored sputum
Chronic obstructive pulmonary disease,
cystic fibrosis, and mechanical ventila-
tion; fever, chills, and copious greenish,
foul-smelling sputum
Complication of gastrointestinal surgery
Consolidation
Klebsiella pneumoniae, gram-
negative encapsulated rods
Pseudomonas aeruginosa, gram-
negative rods
Aminoglycoside plus third-generation cepha-
losporin; alternative: aztreonam, imipenem,
quinolone
Aminoglycoside plus ticarcillin/Clavulanate
or piperacillin/tazobactam or aztreonam or
imipenem
Infiltrates, small pleural
effusion
Escherichia coli gram-negative
rods
Infiltrates, may have pleural
effusion
Aminoglycoside plus third-generation cepha-
losporin; alternative: aztreonam, imipenem,
quinolone
Amantadine, rimantadine
Patchy infiltrates
Consolidation
Macrolides with or without rifampin; alterna-
tive: TMP-SMX, quinolone
Erythromycin, doxycycline; alternative: quino-
lone or other macrolide
Infiltrates
Virus
Fever, malaise, headache, nonproductive
cough
Legionella species; no bacteria Acute onset with fever, diarrhea, myalgia,
and abdominal pain
Mycoplasma pneumoniae (atypi- Ages 5-25 years, most common in young
cal pneumonia); monocytes adults; associated with otitis media and
and neutrophils; no bacteria myringitis, sore throat, headache, myal-
gia, dry cough, fatigue, low-grade fever
Pneumocystis (carinii) jiroveci Immunosuppressed patients infants,
(fungus)
children, and adults); 60% of patients
have AIDS
Anaerobic pneumonia (aspiration Predisposition to aspiration, fever, weight
pneumonia); mixed flora loss, malaise; risk increases with de-
creased level of consciousness, artificial
airway, and sedation; seen in individuals
with poor dental hygiene
Diffuse infiltrates, or chest
X-ray may appear normal
TMP-SMX or pentamidine; isethionate plus
prednisone; alternative: dapsone plus TMP-
SMX, clindamycin plus primaquine
Penicillin G; alternative choices: clindamycin,
metronidazole, cefoxitin
Infiltrates in dependent lung
fields
AIDS, Acquired immunodeficiency syndrome; TMP-SMX, trimethoprim-sulfamethoxazole.
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