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Ganong's Review of Medical Physiology, 23rd Edition

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598 SECTION VII Respiratory <strong>Physiology</strong><br />

Type II<br />

cell<br />

TM<br />

RER<br />

FIGURE 35–13 Formation and metabolism <strong>of</strong> surfactant.<br />

Lamellar bodies (LB) are formed in type II alveolar epithelial cells and<br />

secreted by exocytosis into the fluid lining the alveoli. The released<br />

lamellar body material is converted to tubular myelin (TM), and the TM<br />

is the source <strong>of</strong> the phospholipid surface film (SF). Surfactant is taken<br />

up by endocytosis into alveolar macrophages and type II epithelial<br />

cells. N, nucleus; RER, rough endoplasmic reticulum; CB, composite<br />

body. (Reproduced with permission from Wright JR: Metabolism and turnover <strong>of</strong><br />

lung surfactant. Am Rev Respir Dis 1987;136:426.)<br />

epithelial cells that secrete it. SP-B and SP-C are smaller proteins,<br />

which facilitate formation <strong>of</strong> the monomolecular film <strong>of</strong><br />

phospholipid. A mutation <strong>of</strong> the gene for SP-C has been<br />

reported to be associated with familial interstitial lung disease.<br />

Like SP-A, SP-D is a glycoprotein. Its full function is<br />

uncertain. However, SP-A and SP-D are members <strong>of</strong> the collectin<br />

family <strong>of</strong> proteins that are involved in innate immunity<br />

in the conducting airway as well as in the alveoli. For other<br />

roles <strong>of</strong> surfactant, see Clinical Box 35–2.<br />

WORK OF BREATHING<br />

N<br />

Air space<br />

Work is performed by the respiratory muscles in stretching the<br />

elastic tissues <strong>of</strong> the chest wall and lungs (elastic work; approximately<br />

65% <strong>of</strong> the total work), moving inelastic tissues (viscous<br />

resistance; 7% <strong>of</strong> total), and moving air through the respiratory<br />

passages (airway resistance; 28% <strong>of</strong> total). Because pressure<br />

times volume (g/cm 2 × cm 3 = g × cm) has the same dimensions<br />

as work (force × distance), the work <strong>of</strong> breathing can be calculated<br />

from the relaxation pressure curve (Figures 35–10 and<br />

35–14). The total elastic work required for inspiration is represented<br />

by the area ABCA in Figure 35–14. Note that the relaxation<br />

pressure curve <strong>of</strong> the total respiratory system differs from<br />

that <strong>of</strong> the lungs alone. The actual elastic work required to increase<br />

the volume <strong>of</strong> the lungs alone is area ABDEA. The<br />

amount <strong>of</strong> elastic work required to inflate the whole respiratory<br />

system is less than the amount required to inflate the lungs<br />

alone because part <strong>of</strong> the work comes from elastic energy stored<br />

in the thorax. The elastic energy lost from the thorax (area<br />

AFGBA) is equal to that gained by the lungs (area AEDCA).<br />

LB<br />

CB<br />

Golgi<br />

SF<br />

N<br />

Type I cell<br />

N<br />

Alveolar<br />

macrophage<br />

Fatty acids<br />

Choline<br />

Glycerol<br />

Amino acids<br />

Etc<br />

CLINICAL BOX 35–2<br />

Surfactant<br />

Surfactant is important at birth. The fetus makes respiratory<br />

movements in utero, but the lungs remain collapsed until<br />

birth. After birth, the infant makes several strong inspiratory<br />

movements and the lungs expand. Surfactant keeps<br />

them from collapsing again. Surfactant deficiency is an important<br />

cause <strong>of</strong> infant respiratory distress syndrome<br />

(IRDS, also known as hyaline membrane disease), the serious<br />

pulmonary disease that develops in infants born before<br />

their surfactant system is functional. Surface tension in the<br />

lungs <strong>of</strong> these infants is high, and the alveoli are collapsed<br />

in many areas (atelectasis). An additional factor in IRDS is<br />

retention <strong>of</strong> fluid in the lungs. During fetal life, Cl – is secreted<br />

with fluid by the pulmonary epithelial cells. At birth,<br />

there is a shift to Na + absorption by these cells via the epithelial<br />

Na + channels (ENaCs), and fluid is absorbed with the<br />

Na + . Prolonged immaturity <strong>of</strong> the ENaCs contributes to the<br />

pulmonary abnormalities in IRDS.<br />

Patchy atelectasis is also associated with surfactant deficiency<br />

in patients who have undergone cardiac surgery involving<br />

use <strong>of</strong> a pump oxygenator and interruption <strong>of</strong> the<br />

pulmonary circulation. In addition, surfactant deficiency<br />

may play a role in some <strong>of</strong> the abnormalities that develop<br />

following occlusion <strong>of</strong> a main bronchus, occlusion <strong>of</strong> one<br />

pulmonary artery, or long-term inhalation <strong>of</strong> 100% O 2 . Cigarette<br />

smoking also decreases lung surfactant.<br />

Lung volume (L)<br />

6<br />

4<br />

2<br />

0<br />

−20 0<br />

Transmural pressure (cm H2O) +20<br />

FIGURE 35–14 Relaxation pressure curves in the lung. The<br />

relaxation pressure curves <strong>of</strong> the total respiratory system (P TR), the<br />

lungs (P L), and the chest (P W) are plotted together with standard volumes<br />

for functional residual capacity and tidal volume. The transmural<br />

pressure is intrapulmonary pressure minus intrapleural pressure in the<br />

case <strong>of</strong> the lungs, intrapleural pressure minus outside (barometric)<br />

pressure in the case <strong>of</strong> the chest wall, and intrapulmonary pressure minus<br />

barometric pressure in the case <strong>of</strong> the total respiratory system.<br />

From these curves, the total and actual elastic work associated with<br />

breathing can be derived (see text). (Modified from Mines AH: Respiratory<br />

<strong>Physiology</strong>, 3rd ed. Raven Press, 1993.)<br />

F<br />

H<br />

G<br />

B C<br />

A<br />

E<br />

D<br />

P W<br />

P L<br />

P TR

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