William F. Miller

Pulmonary emphysema and alpha1-antitrypsin deficiency

Abstract A genetically determined deficiency of alpha 1 -antitrypsin has been shown to be associated with pulmonary emphysema. This deficiency is transmitted by a single gene. Heterozygotes for the deficiency gene have intermediate levels of alpha 1 -antitrypsin, homozygotes have low levels. Of twelve patients with evidence of destructive lung disease present prior to age forty, two were found to be homozygous and one heterozygous for the alpha 1 -antitrypsin deficiency gene. Clinical deterioration in our patients was rapid and associated with a respiratory tract infection. A deficiency of alpha 1 -antitrypsin may be an important etiologic factor in the development of destructive lung disease occurring at a relatively young age. The rapid deterioration observed in our patients may be related to this deficiency.

CARDIOPULMONARY PHYSIOLOGICAL RESPONSES TO HEAVY EXERCISE IN PATIENTS WITH ANEMIA

The hemodynamic adaptation of the human body to acute and chronic anemia has been extensively studied. There are few reports, however, of investigations of the response of anemic individuals to exercise. The most exhaustive study to date is that of Bishop, Donald and Wade (1), who evaluated 11 patients with anemia at moderate levels of exercise, performed in a supine position at minute ventilations up to 20 L. Preliminary observations in our laboratory indicated that subjects suffering from moderately severe anemia are capable of exerting themselves on a treadmill at levels of exercise approaching or exceeding those producing maximal oxygen intake in normal subjects. This led us to investigate the response of anemic subjects to very severe exertion.

A STUDY OF CARDIOPULMONARY ALTERATIONS IN PATIENTS WITH SICKLE CELL DISEASE AND ITS VARIANTS

It has recently become apparent that a high proportion of individuals who possess S type hemoglobin exhibit significant desaturation of the arterial blood. In 1942 Klinefelter (1) found arterial oxygen saturations of 89 and 91 per cent in two patients, and in 1943 Reinhard, Moore, Dubach, and Wade (2) found values ranging from 74.2 to 90.8 per cent saturation in one patient with sickle cell disease. Leight, Snider, Clifford, and Hellems (3) studied 13 patients with sickle cell disease in 1954, 10 of whom had arterial 02 desaturation. Within the past year reports from this laboratory (4, 5) and from Jensen, Rucknagel, and Taylor (6) indicate that desaturation of arterial blood exists in most people who possess S type hemoglobin. The exact mechanism responsible for this phenomenon has not been elucidated. The various possibilities may be distinguished with fair accuracy by determining alveolar and arterial oxygen tensions and calculating A-a oxygen tension gradients at three levels of oxygenation. If desaturation is due to hypoventilation, no appreciable gradient exists at any level of oxygenation and CO2 retention is usually evident. Any increase in A-a gradient must, therefore, be due to some other mechanism. Lilienthal, Riley, Proemmel, and Franke (7) suggested that one might distinguish between a diffusion defect and veno-arterial admixture by measurement of the effective alveolar-arterial gradient at two levels of oxygenation, one of which produces significant desaturation of arterial blood. Low levels of inspired 02 (12 to 14 per cent) exaggerate the effects of impaired diffusion and minimize the effects of veno-arterial admixture. Veno-arterial admixture, thus delineated, may be either through actual anatomical communications, or the result of reduction in the ventilation-perfusion ratios.

Physical therapeutic measures in the treatment of chronic bronchopulmonary disorders: Methods for breathing training

Abstract The persistent disability in chronic obstructive bronchopulmonary disease is largely mechanical; therefore, drug therapy alone does not provide an adequate approach to treatment. A complete therapeutic program should include extensive patient education in regard to the nature of the disease. Patients should be taught how to achieve effective muscular relaxation and a pattern of slow breathing with emphasis on expiration both at rest and during exertion. The program should provide training in the proper use of nebulized medication, efficient methods of coughing and postural drainage. In properly selected cases, other mechanical aids such as abdominal belts and pneumoperitoneum are valuable. Such a therapeutic plan would thus be based on principles derived from knowledge of the nature of the physiological disturbances induced by the disease.

Use of ultrasonic aerosols with ventilatory assistors.

(1968). Use of Ultrasonic Aerosols with Ventilatory Assistors. Journal of Asthma Research: Vol. 5, No. 4, pp. 335-354.

The interpretation and diagnostic value of oximetrically-recorded T-1824 dye curves in congenital heart disease☆

Abstract Oximetrically recorded T-1824 dye curves offer a relatively reliable means of identifying the site and type of intracardiac and aortic-pulmonary shunts. The most valid indication of the presence of venoarterial admixture is a shortened appearance time (AT). Curves due to left-to-right shunts can be identified by the presence of a normal appearance time and a prolonged descending slope to which disappearance time (DT) is a good index. The use of ratios between certain time intervals obtained from the curves is of questionable value for the identification of the type of shunt present. Double-peaked dye curves usually, but not always, indicate venoarterial admixture but are not as frequently encountered in patients with such shunts as are shortened appearance times. Comparison of oximetric dye curves and those obtained by serial arterial sampling in normal subjects discloses that the former are quantitatively reliable both as to time intervals and as to the area enclosed, provided 10 mg. or more of dye is injected.

Treatment of Chronic Pulmonary Emphysema

Objectives of treatment for emphysema are prevention and eradication of bronchial inflammation, alleviation of bronchial obstruction, breathing training, physical rehabilitation, and management of major complications. Good bronchial hygiene is stressed. Education of the patient is essential to effective therapy.