0

The full content of Annals is available to subscribers

Subscribe/Learn More  >
Articles |

Pathophysiology of Hereditary Emphysema

PAUL M. STEVENS, M.D., F.A.C.P.; VIOLETTE S. HNILICA, M.D.; PHILIP C. JOHNSON, M.D., F.A.C.P.; and ROBERT L. BELL, M.D.
[+] Article and Author Information

The authors are grateful to Drs. Arthur Beall, Daniel Jackson, Daniel Jenkins, and Ghent Graves, Jr., for allowing them to study their patients. We thank Drs. Samuel Boushy and S. Donald Greenberg for fixating and photographing the pathologic material.

▸Requests for reprints should be addressed to Paul M. Stevens, M.D., Pulmonary Physiology Laboratory, The Methodist Hospital, Houston, Tex. 77025


Houston, Texas


Ann Intern Med. 1971;74(5):672-680. doi:10.7326/0003-4819-74-5-672
Text Size: A A A

Patients with decreased serum alpha1-antitrypsin were designated as severely deficient (7% to 15% of normal) or intermediately deficient (30% to 65% of normal) by quantitative radial immunodiffusion. All severely deficient patients were symptomatic and had severe emphysema. Among intermediately deficient patients emphysema was clinically evident only after age 50. Younger subjects had significant loss of lung elastic recoil and hyperinflation without expiratory flow obstruction. Older patients with an intermediate deficiency had abnormalities that were radiographically and physiologically identical to those of younger severely deficient patients. Postmortem examination of the lung of two patients with severe deficiency and one patient with intermediate deficiency showed extensive panlobular emphysema of the lower two thirds of the lungs, with relative sparing of the upper third. It is concluded that patients with intermediate alpha1-antitrypsin deficiency may develop emphysema identical to that found in severely deficient patients but at an older age.

Figures

Tables

References

Letters

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Comments

Submit a Comment
Submit a Comment

Summary for Patients

Clinical Slide Sets

Terms of Use

The In the Clinic® slide sets are owned and copyrighted by the American College of Physicians (ACP). All text, graphics, trademarks, and other intellectual property incorporated into the slide sets remain the sole and exclusive property of the ACP. The slide sets may be used only by the person who downloads or purchases them and only for the purpose of presenting them during not-for-profit educational activities. Users may incorporate the entire slide set or selected individual slides into their own teaching presentations but may not alter the content of the slides in any way or remove the ACP copyright notice. Users may make print copies for use as hand-outs for the audience the user is personally addressing but may not otherwise reproduce or distribute the slides by any means or media, including but not limited to sending them as e-mail attachments, posting them on Internet or Intranet sites, publishing them in meeting proceedings, or making them available for sale or distribution in any unauthorized form, without the express written permission of the ACP. Unauthorized use of the In the Clinic slide sets will constitute copyright infringement.

Toolkit

Buy Now

to gain full access to the content and tools.

Want to Subscribe?

Learn more about subscription options

Advertisement
Related Articles
Topic Collections
PubMed Articles
Forgot your password?
Enter your username and email address. We'll send you a reminder to the email address on record.
(Required)
(Required)