OBSTRUCTIVE DISEASE OF THE AIRWAYS IN CYSTIC FIBROSIS

PEDIATRICS ◽  
1968 ◽  
Vol 41 (3) ◽  
pp. 560-573
Author(s):  
Robert B. Mellins ◽  
O. Robert Levine ◽  
Roland H. Ingram ◽  
Alfred P. Fishman

A study of the interrelationships of instantaneous air flow, lung volume, and transpulmonary pressure over the range of the vital capacity has demonstrated striking differences in the determinants of maximum expiratory flow in cystic fibrosis and asthma. At high lung volumes, maximum expiratory flow rates in asthma are limited by the mechanical characteristics of the lungs and airways, whereas in cystic fibrosis and in the normal they are dependent on effort. At lower lung volumes, maximum expiratory flow rates are relatively more reduced in cystic fibrosis than in asthma and pressures in excess of those required to produce maximum flow actually depress flow. Also, forced expiration is associated with a transient reversal in the slope of the single breath nitrogen curve in cystic fibrosis and not in asthma. From these studies it is concluded that: (1) airway obstruction is less uniform and involves larger airways in cystic fibrosis than in asthma, and (2) increased expiratory pressure is associated with collapse of some of the larger airways over most of the range of the vital capacity in cystic fibrosis. A major clinical implication of these studies is that the effectiveness of cough is impaired by large airway collapse in cystic fibrosis.

PEDIATRICS ◽  
1971 ◽  
Vol 48 (1) ◽  
pp. 64-72
Author(s):  
Alois Zapletal ◽  
Etsuro K. Motoyama ◽  
Lewis E. Gibson ◽  
Arend Bouhuys

Maximum expiratory flow rates on flow-volume curves are often decreased below normal limits in children with asthma or cystic fibrosis who are clinically well and whose standard spirometric tests are within normal limits. In particular, maximum flow rates at small lung volumes (25% of vital capacity) are decreased. Maximum expiratory flow-volume (MEFV) curves provide a sensitive and quantitative assessment of small airway obstruction in these and other obstructive lung conditions.


1981 ◽  
Vol 50 (3) ◽  
pp. 650-657 ◽  
Author(s):  
N. J. Douglas ◽  
G. B. Drummond ◽  
M. F. Sudlow

In six normal subjects forced expiratory flow rates increased progressively with increasing degrees of chest strapping. In nine normal subjects forced expiratory flow rates increased with the time spent breathing with expiratory reserve volume 0.5 liters above residual volume, the increase being significant by 30 s (P less than 0.01), and flow rates were still increasing at 2 min, the longest time the subjects could breathe at this lung volume. The increase in flow after low lung volume breathing (LLVB) was similar to that produced by strapping. The effect of LLVB was diminished by the inhalation of the atropinelike drug ipratropium. Quasistatic recoil pressures were higher following strapping and LLVB than on partial or maximal expiration, but the rise in recoil pressure was insufficient to account for all the observed increased in maximum flow. We suggest that the effects of chest strapping are due to LLVB and that both cause bronchodilatation.


1986 ◽  
Vol 60 (2) ◽  
pp. 441-448 ◽  
Author(s):  
H. W. Greville ◽  
M. E. Arnup ◽  
S. N. Mink ◽  
L. Oppenheimer ◽  
N. R. Anthonisen

We examined the mechanism of the reduced maximum expiratory flow rates (Vmax) in a dog model of postpneumonectomy compensatory lung growth. During forced expiration, a Pitot-static tube was used to locate the airway site of flow limitation, or choke point, and to measure dynamic intrabronchial pressures. The factors determining Vmax were calculated and the results analyzed in terms of the wave-speed theory of flow limitation. Measurements were made at multiple lung volumes and during ventilation both with air and with HeO2. Five of the puppies had undergone a left pneumonectomy at 10 wk of age, and 5 littermate controls had undergone a sham operation. All dogs were studied at 26 wk of age, at which time compensatory lung growth had occurred in the postpneumonectomy group. Vmax was markedly decreased in the postpneumonectomy group compared with control, averaging 42% of the control flow rates from 58 to 35% of the vital capacity (VC). At 23% of the VC, Vmax was 15% less than control. Choke points were more peripheral in the postpneumonectomy dogs compared with controls at all volumes. The total airway pressure was the same at the choke-point airway in the postpneumonectomy dogs as that in the same airway in the control dogs, suggesting that the airways of the postpneumonectomy dogs displayed different bronchial area-pressure behavior from the control dogs. Despite the decreased Vmax on both air and HeO2, the density dependence of flow was high in the postpneumonectomy dogs and the same as controls at all lung volumes examined.


1986 ◽  
Vol 60 (6) ◽  
pp. 1834-1838 ◽  
Author(s):  
R. B. Filuk ◽  
N. R. Anthonisen

Nine normal young men inhaled boluses of He at the onset of slow vital capacity (VC) inspirations. During the subsequent VC expirations, we measured expired flow, volume, and He concentrations. Expirations consisted of full or partial maximum expiratory flow-volume (MEFV) maneuvers. Full maneuvers were forced expirations from total lung capacity (TLC). Partial maneuvers were accomplished by expiring slowly from TLC to 70, 60, 50, and 40% VC and then initiating forced expiration. Expired He concentrations from full and partial maneuvers were compared with each other and with those resulting from slow expirations. At comparable volumes less than 50% VC, flow during partial and full MEFV maneuvers did not differ. Expired He concentrations were higher during partial maneuvers than during full ones; at the onset of partial maneuvers upper zone emptying predominated, whereas this was not the case at the same lung volumes during maneuvers initiated at TLC. We observed substantial differences in regional emptying sequence that did not influence maximum expiratory flow.


1989 ◽  
Vol 67 (3) ◽  
pp. 1164-1170 ◽  
Author(s):  
S. N. Mink ◽  
S. G. Holtby ◽  
D. J. Berenzanski ◽  
L. Oppenheimer ◽  
N. R. Anthonisen

Five dogs underwent left pneumonectomy at 10 wk of age, whereas four littermates underwent a sham operation. At 26 wk of age the postpneumonectomy dogs had total lung vital capacity (VC) and lung weight similar to controls, but maximum expiratory flow was reduced. Pressure capsules were glued to right lower (RLL) and right cardiac (RCL) lobes, and alveolar pressures (PA) were measured during forced expiration. In postpneumonectomy dogs RLL and RCL both emptied more slowly than in control dogs, and emptying was especially delayed in RCL, which underwent the most growth. When both lobes deflated together, PA in RCL and RLL were similar in control dogs, but in postpneumonectomy dogs PA in RCL exceeded that in RLL by approximately 3 cmH2O from 80 to 20% VC. Because the higher driving pressure in RCL compensated for the relatively high resistance of RCL, the pattern of lobar emptying was relatively uniform over these lung volumes. This result was compatible with interdependence of lobar maximum expiratory flows. In addition, at PA of 6–10 cmH2O in postpneumonectomy dogs, maximum emptying rates of RCL were less when RCL deflated alone than when RCL and RLL emptied together, again demonstrating interdependence of lobar maximum expiratory flow.


1976 ◽  
Vol 51 (2) ◽  
pp. 133-139
Author(s):  
J. J. Wellman ◽  
E. R. McFadden ◽  
R. H. Ingram

1. Gas-density-dependence of maximal expiratory flow rates (V̇max), defined as the ratio of V̇max while breathing helium/oxygen (80:20) to V̇max. while breathing air at the same lung volume, was examined in relation to other measurements of airways obstruction in patients with obstructive airways disease before and after administration of bronchodilators. 2. Seventeen patients showed a 45% or greater increase in specific conductance(sGaw) after bronchodilator therapy (group A) and thirteen patients demonstrated a lesser response (group B). 3. Before the administration of bronchodilators, the degree of obstruction in the two groups was not different as measured by lung volumes, sGaw, forced expiratory volume in 1 s, and flow rates high in the vital capacity; yet the maximal mid-expiratory flow rate and the degree of density-dependence were significantly lower in group B. 4. After bronchodilators, both groups of patients showed significant improvements in sGaw flow rates and lung volumes. However, group A patients showed a significant increase in density-dependence whereas group B patients did not. 5. Increased density-dependence after bronchodilators in the group A patients was associated with an increase in the computed resistance of the upstream segment with air and a decrease in resistance with helium/oxygen. These changes could be explained by a more mouthward movement of equal pressure points, and therefore a further increase in the relative contribution of the larger density-dependent airways to limitation of flow. 6. The fact that density-dependence was not altered after bronchodilators in the group B patients suggests that the site of limitation of flow did not change appreciably. The shift in the pressure—flow curve for the upstream airways was such that the computed resistance of these airways fell. Thus it appears that the airways comprising the upstream segment were dilated.


1987 ◽  
Vol 62 (2) ◽  
pp. 718-724 ◽  
Author(s):  
J. L. Allen ◽  
R. G. Castile ◽  
J. Mead

The maximal expiratory-flow volume (MEFV) curve in normal subjects is thought to be relatively effort independent over most of the vital capacity (VC). We studied seven normal males and found positive effort dependence of maximal expiratory flow between 50 and 80% VC in five of them, as demonstrated by standard isovolume pressure-flow (IVPF) curves. We then attempted to distinguish the effects of chest wall conformational changes from possible mechanisms intrinsic to the lungs as an explanation for positive effort dependence. IVPF curves were repeated in four of the subjects who had demonstrated positive effort dependence. Transpulmonary pressure was varied by introducing varied resistances at the mouth but effort, as defined by pleural pressure, was maintained constant. By this method, chest wall conformation at a given volume would be expected to remain the same despite changing transpulmonary pressures. When these four subjects were retested in this way, no increases in flow with increasing transpulmonary pressure were found. In further studies, voluntarily altering the chest wall pattern of emptying (as defined by respiratory inductive plethysmography) did however alter maximal expiratory flows, with transpulmonary pressure maintained constant. We conclude that maximal expiratory flow can increase with effort over a larger portion of the vital capacity than is commonly recognized, and this effort dependence may be the result of changes in central airway mechanical properties that occur in relation to changes in chest wall shape during forced expiration.


1982 ◽  
Vol 52 (2) ◽  
pp. 346-351 ◽  
Author(s):  
N. Berend ◽  
W. M. Thurlbeck

Pressure-volume and maximum expiratory flow-volume curves with air and a He-O2 mixture were performed in 25 excised human lungs. Small airway dimensions were measured, and the degree of various small airway lesions and emphysema was graded. Correlations were then made between the maximum flow (Vmax) at a transpulmonary pressure (PL) of 5 cmH2O and these measurements and scores. Small airway dimensions correlated poorly with Vmax. However, significant correlations were obtained between Vmax and the inflammation score (P less than 0.05), fibrosis score (P less than 0.05), and emphysema grade (P less than 0.01) but not smooth muscle hyperplasia or pigmentation. Neither the increase in flow with He-O2 nor the volume of flow correlated significantly with any small airway measurement or score.


PEDIATRICS ◽  
1978 ◽  
Vol 61 (4) ◽  
pp. 636-640
Author(s):  
Saul M. Adler ◽  
Mary Ellen B. Wohl

To describe the maximum expiratory flow-volume relationship in newborn infants, we simulated forced expiration by transiently applying positive pressure in a chamber surrounding the infant's body. Maximum expiratory flows were reached at any given lung volume when increases in chamber pressure failed to produce increases in flow. Maximum expiratory flows were achieved in seven of nine healthy newborn infants at lung volumes equal to functional residual capacity (FRC) and in all infants at lung volumes below FRC. The volume expired below FRC (6.4 ml/kg) was roughly equivalent to previously calculated values of expiratory reserve volume in newborn infants (7 ml/kg). The maximum expiratory flow volume curves showed that the infants were able to increase expiratory flow rates well above those achieved during tidal breathing. The convex shape of the curves at low lung volumes is compatible with flow limitation occurring in peripheral airways.


Sign in / Sign up

Export Citation Format

Share Document