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Oxyhemoglobin dissociation curves measure the most important function of red blood cells - the affinity for oxygen and its delivery to the tissues. This function may be deranged in sickle cell anemia and some other hemoglobinopathies. An automated oxyhemoglobin dissociation curve analyzer constructed dissociation curves in 55 patients with hemoglobinopathies and in 24 control subjects while maintaining constant temperature and pH. Sigmoid curves were converted to rectilinear ones using the Hill equation. Oxygen affinity of red cells was assessed by calculation of P50 (the PO2 at which hemoglobin is half saturated). Results revealed separation of oxyhemoglobin dissociation Hill plots according to phenotype but with wide variability. Mean oxygen affinity of fetal hemoglobin was greatest, whereas that of sickle hemoglobin was least. Other hemoglobins were intermediate. A positive correlation between decreased oxygen affinity and carboxyhemoglobin confirmed the decreased oxygen affinity of sickle hemoglobin and decreased oxygen affinity and increased diphosphoglycerate in red cells. Hill plots are less sensitive discriminators of oxygen affinity than traditional sigmoid dissociation curves and offer no particular advantage. Serial studies in a subset of three sickle cell anemia patients treated conservatively suggest automated oxyhemoglobin dissociation curves may be useful in assessment of effectiveness of newer therapies of sickle cell anemia after refinement of the method and studies of larger populations.

Sarcoidosis is a systemic granulomatous disease of undetermined etiology in which the immune system is overstimulated. Management of the patient with sarcoidosis entails continuity of patient care far beyond disease, even into remission. Care is comprehensive, including all involved organ systems, coordination of specialty consultations and services, and includes diagnostic tests. Therapeutic decisions are the responsibility of the primary care practitioner. Prognosis of sarcoidosis is not uniformly good. Patients can die. Subacute sarcoidosis patients usually do not require pharmacologic therapy. Chronic sarcoidosis may require long-term treatment for years to indefinitely. Corticosteroids are still the drugs of choice and other therapy is now available. Health organizations and private nonprofit support groups are available for patient education and assistance. Close support must still come from the family unit. Sarcoidosis may occur in family members in different cohorts at all ages in life and in any racial or ethnic group. The second in a two-part series on management of the patient with sarcoidosis, this article describes coordination of care and considers community, prevention, and family aspects of the disease.

Sarcoidosis is a systemic granulomatous disease of undetermined etiology in which the immune system is overstimulated. Management of the patient with sarcoidosis entails continuity of patient care far beyond disease, even into remission. Care is comprehensive, including all involved organ systems, coordination of specialty consultations and services, and diagnostic tests. Therapeutic decisions are the responsibility of the primary care practitioner. Prognosis of sarcoidosis is not uniformly good. Patients can die. Subacute sarcoidosis patients usually do not require pharmacologic therapy. Chronic sarcoidosis may require long-term treatment for years to indefinitely. Corticosteroids are still the drugs of choice and other therapy is now available. Health organizations and private nonprofit support groups are available for patient education and assistance. Close support must still come from the family unit. Sarcoidosis may occur in family members in different cohorts at all ages in life and in any racial or ethnic group. This article is the first in a two-part series on management of the patient with sarcoidosis in the 21 st century by the primary are provider. Applications of principles of family medicine are emphasized. Part 1 considers continuity, comprehensiveness, and coordination of care. Part 2 will complete coordination of care, and consider community, prevention, and family aspects of this disease.

A prospective case-control study was undertaken to assess respiratory disability in 133 former coal miners who were claimants for "black lung" benefits. Consecutive assignment was made to either case or control group based on their chest radiograph having shown coal workers' pneumoconiosis or no coal workers' pneumoconiosis. A respiratory occupational survey was completed with physical examination that placed special emphasis on the cardiorespiratory systems. Subjects underwent pulmonary function testing while 92 of these also received arterial blood gases to assess respiratory disability and pulmonary insufficiency. Arterial blood gases were superior to spirometry in assessment of pulmonary insufficiency/disability. Smoking interacts with coal workers' pneumoconiosis to cause pulmonary insufficiency. The most frequent spirometric pattern was obstructive. Disability was caused by occupational injuries and comorbidities, both of which occurred with greater frequency in miners with coal workers' pneumoconiosis than in controls. Pulmonary insufficiency appears to be a better discriminator than respiratory disability in coal miners, suggesting that arterial blood gases replace spirometry in their evaluation. Greater emphasis on smoking intervention among coal miners should be given.

Environmental health concerns in the Persian Gulf are peculiar to the geography of the region. Prevention of heat and solar injury deserves primary consideration, but cold injury also may occur in the desert. Immunizations are recommended against a number of diseases, while malarial chemoprophylaxis is necessary in Iraq and Kuwait. In addition to malaria, other parasitic diseases deserve consideration. Diarrheal diseases, diseases from the desert dust, and products of infected desert animals are of concern. Additional natural hazards are venomous bites from scorpions and desert snakes. Finally, threats of enemy action necessitated protection from nuclear biological and chemical weapons and LASER eye/skin injury. Unexploded ordinance will constitute a continuing hazard into the future.

A link between cigarette smoking and "acute chest syndrome" in sickle cell anemia is suggested. Acute chest syndrome in the patient with sickle cell anemia is characterized by fever, leukocytosis, cough, chest pain, and pulmonary infiltrates in the chest radiograph. This article describes the results of a study of 69 adolescent and young adult sickle cell anemia patients. Twenty-nine of these patients were smokers, three were former smokers, and 37 were nonsmokers. Patients completed respiratory questionnaires that focused on smoking habits and included a history of chest syndrome. Information obtained was confirmed by review of clinical records. The chi-square test demonstrated a strong relationship between cigarette smoking and chest syndrome in sickle cell anemia. All 29 smokers had a history of chest syndrome, but only 24 of 37 nonsmokers had such a history. Although the exact mechanism of the relationship between smoking and the development of acute chest syndrome remains speculative, cigarette smoking joins infection, hypoxia, acidosis, infarction, dehydration, and analgesics as a causative factor in adolescent and adult patients with sickle cell anemia. Behavioral modification of the smoking habit in patients with sickle cell anemia may decrease the frequency of acute chest syndrome and sequelae of sickle cell lung disease.

To aid in diagnostic chest film interpretation of coal workers' pneumoconiosis, a composite profile of common radiologic patterns was developed in 98 Appalachian former coal miners who were diagnosed as having coal miner's pneumoconiosis and who applied for black lung benefits. The mean age was 61 years, with a lifetime coal mine dust exposure of 18.7 years. Results showed that chest radiographs of coal workers' simple pneumoconiosis contained small irregular linear opacities more frequently (47%) than small rounded opacities. Sparse profusion of all small opacities was the rule. Small opacities involved two out of six lung zones simultaneously 39% of the time while other combinations occurred less frequently. Lower zones were involved more frequently than upper ones. Thickened pleura occurred in 18% of radiographs. Other frequent radiographic abnormalities were parenchymal calcifications (19%), marked emphysema (12%), and inactive tuberculosis (12%). Calcification of the aortic knob, a degenerative process reflecting age, occurred in 9%. Only one instance of complicated coal workers' pneumoconiosis (progressive massive fibrosis) was encountered (0.7%). Many of the descriptive features of coal workers' pneumoconiosis noted in the literature were not observed in this study. Only one instance of complicated pneumoconiosis was encountered.

Graduate and postgraduate health-care professional training and postdoctoral fellowship programs that deny trainees opportunities to practice both oral and written communication skills produce an incompletely trained health-care provider unable to compete for faculty positions at university hospitals and affiliated staffs. Therefore, it is imperative that program directors make medical writing a prerequisite to successful completion of postgraduate training programs. To make trainees as well as administrators and faculty aware of the importance of oral and written communication skills, a variety of oral abilities needed for presenting medical findings prior to publication are detailed. The use of 2 x 2 slides to support a presentation as well as transparencies, movies, and videotapes are considered. The poster session/scientific exhibit, now becoming more visible because of increasing attendance at professional meetings, is also explained. Written communication abilities are discussed. Consideration is given to the writing of professional manuscripts for publication in a refereed journal. Other types of written communication include case reports, clinicopathological conferences, letters to the editor, book reviews, books, and book contributions. The opportunity to learn needed skills must be offered in the postgraduate health-care curriculum. Mandatory medical writing will maximize the marketability of black health-care professionals for faculty staff placement. Moreover, the establishment of a "track record" early in a professional career will increase the likelihood that black health providers are awarded grants for research.

Sarcoidosis, an affliction of mankind named only as recently as this century, was first described by Hutchinson in 1878, and noted in a second patient, one Mrs. Mortimer, in 1898. In 1889, Besnier described lupus pernio.Boeck obtained skin biopsies in 1899. Kreibich described punched-out bone lesions in 1904. Darier and Roussy described subcutaneous nodules in 1906, and Heerfordt described uveoparotid fever in 1909. Schaumann synthesized the many diverse syndromes of sarcoidosis into a single disease in a prizewinning essay in 1914. Kveim biopsied the sarcoid skin test-site and established a diagnostic test in 1941.Lofgren described the bilateral hilar lymphadenopathy-erythema nodosum syndrome in 1946. In 1954, Israel and Sones helped establish sarcoidosis as an immunologic disorder. In 1963 Cummings compiled a bibliography on sarcoidosis, and in 1966 Siltzbach conducted his renowned international Kveim test trial.At Howard University, Harden ignited an interest in sarcoidosis. In 1944 Johnson and Jason contributed to a description of cardiac involvement, while Cowan studied ocular involvement in sarcoidosis.

Emphysema, chronic bronchitis, asthma, and cystic fibrosis are often cited as examples of chronic airways obstruction, while sarcoidosis is typically restrictive. Approximately 15 percent of sarcoidosis patients, however, have airways obstruction clinically characterized by wheezing with granulomatous involvement of airways. Since the majority have Stage IV disease by chest radiograph, their lungs usually have honey-combining with pulmonary fibrosis, adhesions, cavities, and mediastinal distortion.Patients had a mixed ventilatory defect, but obstruction of large airways was present as shown by decreased specific airways conductances. Small airways obstruction was also present as shown by low instantaneous flows at the terminal portion of the maximum expiratory flow-volume curve and diminished helium response of this curve. The closing volume, however, was not very sensitive. Radioactive xenon washout from ventilation lung scans and N2 washout from the lungs were prolonged in patients with worse disease.The authors conclude that the obstructive type of physiologic pattern is more frequent than recognized in sarcoidosis, which like that of cystic fibrosis has some restrictive element and is characterized by poor reversibility to bronchodilators. A trial period of beclomethasone dipropionate aerosol was not helpful in two patients. Relief of this distressing airways obstruction continues to pose a challenging problem in management.

Previous studies of lung function tests performed on patients with sickle cell disease have shown a restrictive ventilatory defect, usually a diffusion defect, and mild hypoxia at rest. The present study was undertaken to explain the pathophysiology of these changes and to extend these studies to include functional measurements not reported previously.Lung function studies were performed at rest and during treadmill walking on 66 patients with sickle cell anemia and on 16 healthy control subjects. Patients had restrictive ventilatory defects, decreased lung compliance, and uneven ventilation-perfusion ratios. These abnormalities caused an increased alveolar-arterial oxygen tension difference that caused hypoxemia. The diffusion defects were because of the sickle cell disease.Carboxyhemoglobin levels were increased in patients with sickle cell disease. This increase may be caused by a combination of factors, including increased cigarette smoking, hemolysis, and preferential survival of red blood cells that contain carbon monoxide and which do not sickle. During treadmill walking, the patients with sickle cell disease showed a decreased work tolerance caused by impaired oxygen delivery. The anaerobic threshold is reached sooner in patients with sickle cell disease and may also account for the limitations in work capacity of these patients.

The rare association of protease inhibitor deficiency (Pi(zz) genotype) in a black American with chronic, obstructive pulmonary disease due to asthmatic bronchitis, rather than basal pan lobular emphysema, is presented. The late onset of symptoms, despite environmental exposures, is also unusual in this homozygote, as is his ethnic background.

Twenty-three hospital employees who suffered ill effects of smoke inhalation were pulmonary-function tested within 72 hours, after ten weeks, and again after 45 weeks of exposure. Four nonexposed control subjects were selected for comparison of data.Exposed persons commonly had restrictive lung disease which may have resulted from stimulation of irritant receptors in bronchi and/or interstitial edema of chemical injury at the level of the ventilation-perfusion unit. Airways obstruction was infrequent, despite the possibility of inhalation of di-isocyanates, an intermediary product of burning polyurethane foam that may cause chemical asthma. Eye and constitutional symptoms were also frequent.Clinical recovery was as a rule rapid and complete (within a week), but physiologic recovery was protracted (over 45 weeks in some cases). Most surprisingly, cigarette smoking and/or presence of cardiopulmonary disease did not contribute to morbidity.

Sarcoidosis is a recently identified cause of functional asplenia that can be diagnosed by radionuclide imaging. A 31-year-old woman with a five-year history of histologically compatible sarcoidosis was found to have nonvisualization of the spleen on technetium 99m sulfur colloid (radiopharmaceutical) liver-spleen scan. This scintigraphic finding was accompanied by poikilocytosis and Howell-Jolly bodies in the peripheral blood smear. A subsequent gallium 67 citrate scan reflected an abnormal increase in concentration of activity in the spleen, suggesting an active inflammatory process.Based upon this constellation of findings, it was concluded that acquired functional asplenia is the result of reticuloendothelial cell replacement via infiltration of the spleen by epithelioid cell granulomas of active sarcoidosis. This case also illustrates the reversibility of functional asplenia of sarcoidosis following adrenocorticosteroid therapy. Functional asplenia in sarcoidosis is now found to have a recognizable radionuclide imaging pattern.

About 80 percent of sarcoidosis cases are benign and do not require treatment, but 20 percent will have chronic unremitting disease for which therapy is essential. It is important that the physician identify this group and begin therapy promptly. If the disease is active, treat. If it is inactive, do not treat. Activity depends upon three major tests: serum angiotensin converting enzyme, gallium 67 scan, and bronchoalveolar lavage. The other consideration is involvement of vital organ systems; ie, active ocular disease, progressive pulmonary involvement as evidenced by increasing symptoms, impaired and deteriorating pulmonary function, or radiographic changes; hypercalcemia or hypercalciuria; central nervous system involvement; disfiguring cutaneous lesions; and myocardial sarcoidosis. Following a therapeutic decision to treat, adrenocorticoids are the drugs of choice. Methylprednisolone, prednisone, and cortisol are listed in order of benefit. Alternate day and/or low-dose steroids are increasing in popularity. Chloroquine phosphate is beneficial for skin lesions, while oxyphenbutazone has been found to be at least as effective as prednisone. Immunosuppressives may be used also. Chlorambucil and azathioprine have shown variable results. Cyclosporine (Cyclosporin A) shows promise and is now undergoing therapeutic trials.

The diagnosis of sarcoidosis, as with other intrathoracic lesions, remains a challenge to the clinician. An adequate history, a thorough physical examination, and the employment of various laboratory tests often establish the diagnosis. When a definitive diagnosis is not made, biopsy of suspected lesions is indicated. Various diagnostic procedures are presented and compared.

An epidemiologic study of protease inhibitor (alpha(1)-antitrypsin) was undertaken among 599 ambulatory and hospitalized black American patients with chronic cardiopulmonary disease referred for pulmonary function testing, and 115 ethnically matched, healthy control subjects. Clinical evaluation consisted of respiratory questionnaire completion, physical examination, chest radiograph, and spirography. Protease inhibitor evaluation consisted of measurement of serum trypsin inhibitory capacity in all subjects corrected by comparison with control sera, while 200 of these subjects were phenotyped for alpha(1)-antitrypsin electrophoretic variants.Results showed mean serum trypsin inhibitory capacity for all subjects was 1.56, SD ± 0.47 mg/ml, while corrected values were 111.2, SD ± 30.5 percent of control. Acute phase reactivity was present for patients with heart disease, pulmonary malignancy, p<0.01 for both, and pulmonary fibrosis, p<0.05, when compared with controls. Prevalence of protease inhibitor variants in 29 controls was two heterozygotes for the Z variant (seven percent), and one homozygote for the S variant. Among 94 patients with chronic obstructive pulmonary disease, prevalence was 1.1 percent each for ZZ and SZ phenotypes, and 2.1 percent for MZ. Suprprisingly, the sole ZZ patient had asthmatic bronchitis rather than emphysema.Computed allele frequencies for Pi M and Z were comparable to those for a random sample of black Americans in St. Louis, but differed from a sample of black infants in Brooklyn, NY.These results indicate that protease inhibitor deficiency variants are not as uncommon among black Americans as the literature suggests. Furthermore, the heterozygous state is not necessarily a risk factor in development of chronic obstructive pulmonary disease. Protease inhibitor deficiency states therefore appear to play less important a role in etiology of chronic cardiopulmonary disease in black Americans than among their Caucasian counterparts.Preliminary work was published in abstract form.(1)

Acute pulmonary complications of sickle cell anemia are sickle cell lung disease and bacterial pneumonias. Chronic abnormalities in lung function include a restrictive ventilatory defect and perhaps increased venous admixture to the pulmonary circulation. Coexisting sarcoidosis may complicate sickle cell anemia and interact to potentiate sickling. Sickle cell lung disease, or acute "chest syndrome," occurs with greatest frequency in adults, is due primarily to pulmonary infarction, and may lead to cor pulmonale. On the other hand, bacterial pneumonia due to Streptococcus pneumoniae occurs with greater frequency in infancy and childhood. Mycoplasma and other organisms may also cause pneumonia with protracted illness and slow resolution. Bacteremia and meningitis may be further complications, particularly in children. Precise diagnosis of the acute febrile pulmonary episode is often difficult. In adults the illness is commonly self-limited. However, a vigorous diagnostic approach is warranted in all severely ill patients.

Eight of ten cigarette smokers successful in a smoking cessation group therapy program were examined, initially, and at six and 12 months postcessation. Examination included completion of a respiratory questionnaire and selective pulmonary function testing that included maximum expiratory flow volume curves, slope of phase 3 alveolar plateau, and closing volume. Two symptomatic subjects became symptom-free within six months postcessation. Results indicate a mean weight gain of 4.1 kg within 12 months (P less than 0.01), deterioration in mean forced expiratory volume in one second as percent of forced vital capacity (P less than 0.05), and deterioration in mean forced expiratory volume in three seconds as percent of forced vital capacity postcessation (P less than 0.05). Other tests derived from the maximum expiratory flow-volume curve, slope of phase 3, or closing volume showed no change. These results may be explained by a number of factors and may have implications for future studies.

Tuberculosis in the elderly takes on new significance. The case of an 87-year-old female nursing home resident is presented. She was admitted to the hospital with atypical pulmonary manifestations and right pleural effusion. Tuberculin skin tests were negative. The patient deteriorated and died. Disease due to Mycobacteria tuberculosis was discovered by culture after death.The prevalence of tuberculosis in the aged is increasing as the population ages. In the long-term-care setting, residents infected early in life outlive their tubercle bacilli. They are at risk of being infected again.Continuous clinical awareness in the geriatric segment of the population is essential if tuberculosis control is to be achieved. Tuberculin skin testing is important, with chemoprophylaxis for converters, and treatment for active disease.