Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates

Article Abstract:

Alpha-1-antitrypsin (AAT) deficiency correlates strongly with the development of emphysema. AAT inhibits the action of the enzyme neutrophil elastase, which breaks down proteins in the lungs. Most of the studies of AAT deficiency have involved patients who have come to the attention of clinicians because of respiratory problems, thus biasing attempts to determine the direct effect of the deficiency on respiration and the correlation of this effect with other risk factors such as smoking. To study the effects of AAT deficiency on respiration, antitrypsin-deficient people without lung disease were identified from the records of 20,000 blood donors or from the families of patients. Fifty-two AAT deficient people consented to participate in a test which involved answering a questionnaire and undergoing pulmonary function tests. The gene for alpha-1-antitrypsin, called Pi, for protease inhibitor, has over 75 variants; all the patients selected for this study had the particular gene variant called Z type and are called Pi Z individuals. Tabulation of the results revealed that many Pi Z individuals who have normal pulmonary function are likely to lead healthy lives despite their genetic deficit. Although it is not possible to rule out the development of abnormal pulmonary function later in life in some of these individuals, there are some factors which seem to correlate well with the onset of disease in Pi Z individuals. Some factors, like smoking, asthma, and pneumonia are not unexpected. The correlation with asthma or emphysema in a parent was surprising, however, since the parents of the subjects would be expected to possess only one copy of the abnormal Pi gene, and therefore should have normal antitrypsin activities themselves. The results suggest that there may be other hereditary factors which contribute to emphysema, but further study will be necessary to clarify what these may be. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Physiological aspects, Lung diseases, Obstructive, Chronic obstructive lung disease, Tobacco, Alpha 1-antitrypsin

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Acute eosinophilic pneumonia as a reversible cause of noninfectious respiratory failure

Article Abstract:

Eosinophilic pneumonia, a type of respiratory disease characterized by the presence of abnormal concentrations of eosinophils (a type of white blood cell) in the lungs, may be categorized as either chronic or acute and is often caused by hypersensitive reactions to fungi, spores, or drugs, rather than infection. Less is known about the specific nature of acute eosinophilic pneumonia. Four patients ranging in age from 20 to 64 exhibited symptoms that included high fever, low oxygen levels in the arterial blood, congestion and difficulty in breathing accompanied by excess fluid in the lungs. Treatment with erythromycin, a common antibiotic, was unproductive. Bronchoalveolar lavage, extraction of fluid from the lungs, revealed abnormally high concentrations of eosinophils, leading to elimination of infectious pneumonia in favor of acute eosinophilic pneumonia. Once diagnosis was confirmed, treatment with corticosteroids (steroid therapy) was begun and continued from 10 days to 2 weeks, with a favorable response from all four patients. After five months, follow-up tests showed eosinophil levels to be normal and there were no signs of lung disease. The combination of bronchoalveolar lavage for diagnosis and corticosteroids for subsequent treatment in case of acute eosinophilic pneumonia is recommended.

Care and treatment, Evaluation, Diagnosis, Adrenocortical hormones, Pulmonary eosinophilia, Eosinophil disorders

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Increased Susceptibility to Pulmonary Emphysema among HIV-Seropositive Smokers

Article Abstract:

HIV infection appears to accelerate the development of emphysema in patients who smoke. In a study of 114 HIV-positive and 44 HIV-negative smokers, 15% of the HIV patients developed emphysema compared to 2% of the healthy smokers. Among the long-term smokers, 37% of the HIV patients developed emphysema but none of the healthy smokers did. Bronchoalveolar lavage showed that HIV patients with emphysema had high levels of cytotoxic lymphocytes in their lungs.

Health aspects, Complications and side effects, HIV infection, HIV infections, Smokers, Smoking, HIV patients

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