A role for intermediate, heterozygous alpha 1-antitrypsin deficiency in obstructive lung disease.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to C Colp.
Explore the source record for details and available documents.
A high prevalence of asthmalike symptoms was noted among patients of Puerto Rican descent attending Beth Israel and North Central Bronx Medical Centers in New York City, as compared with other ethnic groups. An evaluation of family and medical histories, pulmonary function data, and alpha 1-antitrypsin phenotypes was undertaken in such Puerto Rican patients and control subjects without asthma. The patients showed a higher proportion of MS and MV phenotypes. All the patients in both MM and variant phenotype groups, with the exception of four MM patients, had features indicative of asthma, with labile airway obstruction, and elevated serum immunoglobulin E and eosinophil levels. The latter was significantly higher in the patients with variant phenotypes than in MM patients. Patients with alpha 1-antitrypsin variants also had much shorter smoking histories as compared with the MM group, and all reported histories of asthma in first-degree relatives, as compared with 66% among the MM patients. We conclude that there is an increased incidence of asthma among Puerto Ricans in New York City, and that the antitrypsin variant phenotypes (specifically S and V) play a role in this incidence and its expression.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Pulmonary function tests were performed on a group of 22-ex-heroin addicts with no known pulmonary disease or symptoms. Vital capacity and total lung capacity were normal in all but four patients. Only two patients had evidence of moderately severe airway obstruction. However, the single breath diffusing capacity (Dco) was significantly reduced in many of the patients and averaged 81 percent of predicted for the entire group. Patients with most severe reduction of Dco also had reduction of lung volumes. In addition, six patients had increased lung recoil force (Pmax) and decreased lung compliance. There was no correlation between degree of functional impairment and duration or amount of durg use, incidence of overdose or intravenous injection of crushed tablets. Followup studies in four patients after several months on methadone maintenance showed improvement in lung volumes and/or Dco. Possible etiologic considerations to explain these abnormalities were previous subclinical pneumonias, foreign body granulomas, previous pulmonary edema or other direct toxic effect on alveolocapillary membrane as the result of use of drugs.
Three patients with pleural restriction are presented in detail. One patient had had a right pneumonectomy and died of ventilatory failure due to left-sided restrictive pleurisy. The second patient had neoplastic pleural effusion and inactive tuberculosis. The third patient had systemic lupus erythematosus with bilateral restrictive pleuritis. The physiologic picture was similar in the three cases, with marked decrease of lung volumes, increase of the RV/TLC ratio, absence of airways obstruction, decrease of the Dco and decrease of dynamic lung compliance. In addition, each of these three patients and others with severe pleural restriction had a lower than normal maximum static pulmonary recoil pressure (Pmax). Since in pulmonary restrictive disease, the Pmax was found to be elevated, it was a useful test for distinguishing pulmonary restriction from pleural restriction.