| Clinical findings and lung pathology in children with cystic fibrosis. | |
| | |
MedLine Citation:
|
PMID: 11956063 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Cystic fibrosis pulmonary disease is assessed by pulmonary function tests, arterial blood gases, and chest X-rays, but the correlation with lung pathology is unknown. We reviewed the clinical findings and lung pathology of 21 cystic fibrosis patients who had lung transplant. Pulmonary function tests, Brasfield scores, arterial blood gases, and age were correlated with lung pathology. All patients had severe Brasfield scores (9.0 +/- 3.2), airways obstruction (FEV1 25.6 +/- 5.6% predicted, FEF(25-75%) 11.0 +/- 4.5% predicted), and hyperinflation (residual volume [RV] 341.8 +/- 75.8% predicted). All patients were hypoxemic (PO2 64.2 +/- 8.2 mm Hg), and 5 of 21 (24%) were hypercapneic (PCO2 > 50 mm Hg). Pulmonary function tests and Brasfield scores were within a narrow range, and did not allow correlation with lung pathology. Small airway density (airways < 2 mm/cm2) decreased with increasing age. There were no differences in small airways inflammation and fibrous narrowing between the hypercapneic and nonhypercapneic patients, but the percent of smallest airways (airways < 0.35 mm) was significantly lower in the hypercapneic group. We conclude that there is significant correlation between airway pathology and increased age and CO2 retention. We speculate that decreased small airway density in older patients and the decreased proportion of smallest airways in hypercapneic patients is caused by increased dilatation of small airways. |
| | |
Authors:
|
Refika Hamutcu; Jon M Rowland; Monica V Horn; Cornelia Kaminsky; Eithne F MacLaughlin; Vaughn A Starnes; Marlyn S Woo |
Related Documents
:
|
264153 - Diphenylhydantoin and pulmonary function. 19219503 - Hla antigens and nontuberculous mycobacterial lung disease in korean patients. 2147603 - Long-term follow-up of children with down syndrome with cardiac lesions. |
Publication Detail:
|
Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
|
Title: American journal of respiratory and critical care medicine Volume: 165 ISSN: 1073-449X ISO Abbreviation: Am. J. Respir. Crit. Care Med. Publication Date: 2002 Apr |
Date Detail:
|
Created Date: 2002-04-16 Completed Date: 2002-05-09 Revised Date: 2006-11-15 |
Medline Journal Info:
|
Nlm Unique ID: 9421642 Medline TA: Am J Respir Crit Care Med Country: United States |
Other Details:
|
Languages: eng Pagination: 1172-5 Citation Subset: AIM; IM |
Affiliation:
|
Division of Pediatric Pulmonology, Childrens Hospital Los Angeles and Keck School of Medicine, University of Southern California, Los Angeles, California 90027, USA. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adolescent Adult Child Cystic Fibrosis / pathology*, physiopathology, surgery Female Forced Expiratory Volume Humans Lung / pathology* Lung Transplantation Male Maximal Midexpiratory Flow Rate Residual Volume Retrospective Studies |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Selective induction of eotaxin release by interleukin-13 or interleukin-4 in human airway smooth mus...
Next Document: Prolonged inflammatory response to acute Pseudomonas challenge in interleukin-10 knockout mice.