Treatment for primary pulmonary hypertension. Back to the future.
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Biomedical subjects
Publications and source records attributed to M F Tenholder.
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Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. It is sufficiently uncommon that randomization or any other systematic evaluation of regimens of treatment has been difficult. Review of scattered case reports implies that a number of hormonal manipulations may be equally effective. A comprehensive review of the literature revealed 30 cases of LAM treated with eight regimens of treatment. Evaluation with predetermined criteria (meta-analysis) shows that administration of progesterone or oophorectomy or both are the most effective treatments, resulting in improvement or stabilization of the disease in the majority of cases.
A normal host can be colonized by mycobacteria other than tuberculosis (MOTT), resulting in bronchoscopic isolates of no clinical significance. In the acquired immunodeficiency syndrome (AIDS), Mycobacterium avium-intracellulare, Mycobacterium kansasii, and Mycobacterium xenopi have caused widely disseminated infection. To determine the usefulness of fiberoptic bronchoscopy (FB) in evaluating MOTT infection in AIDS, we reviewed MOTT cultures from 36 FBs, correlated these to clinical course, and identified MOTT isolates from cultures of all other sources in these patients. Of ten bronchoscopic MOTT isolates, seven were not related to lung disease or to dissemination within one month of FB. Of the four Mycobacterium fortuitum and seven M avium-intracellulare that did disseminate within one month, only two were reflected in bronchoscopic cultures. In patients with AIDS, bronchopulmonary MOTT colonization does occur. We recommend that standard criteria for pulmonary mycobacterial disease be applied. Negative bronchopulmonary cultures do not seem to exclude dissemination.
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This study examined the questions of whether pregnancy decreases physical fitness, as measured by maximal oxygen consumption, between the second and third trimesters, and whether maintaining a regular exercise program during the second half of pregnancy affects fitness. At the beginning of their second trimester, pregnant women were randomly assigned to either a nonexercising control group or an exercising group. They completed a maximal progressive exercise test on a cycle ergometer at 20 and 30 weeks, during which pulmonary parameters of aerobic capacity were measured. The exercising group demonstrated greater improvement in aerobic capacity than did the control group, manifested by increases in tidal volume and oxygen consumption and a stable ventilatory equivalent for oxygen. Pregnancy did not reduce maximal oxygen consumption between the second and third trimesters of pregnancy.
Orthodeoxia--arterial desaturation accentuated by the upright position and improved by recumbency--has been described earlier with true pulmonary vascular shunts and intracardiac shunts. This phenomenon has been described in some parenchymal lung diseases, but has not been reported with interstitial fibrosis. We describe 2 patients with predominantly basal interstitial fibrosis, disabling dyspnea, and severe hypoxemia who demonstrated this positional oxygenation change. Large or surgically correctable arteriovenous malformations (AVM), or intracardiac shunts, were not demonstrated in either patient. This finding has important implications for oxygen prescription and the explanation of positional dyspnea.
Since bronchography is helpful in certain clinical settings, we describe a simplified method for performing selective bronchography in conjunction with fiberoptic bronchoscopy. Selected cases are presented which illustrate that this technique is easily performed, has minimal risk, and produces bronchograms of high quality.
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From January 1974 to December 1983, positive mycobacterial isolates from all sources were reviewed to determine the impact of fiberoptic bronchoscopy (FB) on retrieval and identification of these organisms. There were 112 patients with positive cultures obtained during FB, 25 with Mycobacterium tuberculosis and 87 with mycobacteria other than tuberculosis (MOTT). We reviewed the results of prebronchoscopy and postbronchoscopy sputum specimens, bronchial washings, brushings, and transbronchial biopsy to determine the yield from each specimen in patients with M. tuberculosis. The bronchial washings provided positive cultures in 24 of 25 and were exclusively positive in 10 of 25 (40%). We also reviewed the clinical presentation, chest roentgenogram, bronchoscopy findings, and culture data for the 87 patients with MOTT isolated. The isolation of MOTT from bronchoscopy specimens increased throughout the study, most notably with the introduction of a rapid radiometric method (the Bactec system) for the recovery of mycobacteria to our laboratory in June 1983. Active disease could be established in only 13 of 87 cases (15%). Our findings confirm the sensitivity of Bactec in the isolation of MOTT from bronchoscopic specimens. The Bactec system, on the other hand, does not differentiate saprophytic colonization from clinical disease. To avoid expensive, time-consuming biochemical identification necessary to evaluate these MOTT isolates, careful selection of patients prior to obtaining mycobacterial cultures during FB is a critical factor.
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Eleven adult men with sleep apnea underwent nocturnal polysomnography on two successive nights. The first study, done without NCPAP, served as the control. The second (treatment) was done with the application of 7.5 to 15 cm H2O nasal continuous positive airway pressure (NCPAP). A subjective sleepiness index (SSI) was noted upon awakening from each night of polygraphic recording. During the control night, the mean frequency of apnea episodes/sleep hr was 35.95 +/- 4.5 SE, and the mean duration was 28.68 +/- 2.7 sec. Mean frequency of disorder of breathing (DOB) episodes/sleep hr was 19.25 +/- 6.2 and mean duration of DOB episodes was 23.1 +/- 2.8 sec. During the treatment night, all obstructive apnea episodes were abolished. During the control night, the mean decrease in arterial oxygen saturation during obstructive apnea episodes was 11.2 +/- 1.9 percent and the mean lowest saturation was 67.6 +/- 4.0 percent. NCPAP eliminated arterial oxygen desaturation. While 44.5 +/- 5.7 percent of total sleep time was spent in either apnea or disordered breathing during the control night, NCPAP decreased this to 0.73 +/- 0.3 percent. In addition to the improvement in respiration during sleep, SSI decreased from a mean of 3.73 +/- 0.49 after the control night to 1.64 +/- 0.24 after treatment, reflecting an improvement in daytime hypersomnolence. We conclude that nasal CPAP is effective in eliminating obstructive apnea episodes, and results in a marked decrease in daytime hypersomnolence after one treatment night.
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Forty-four patients with newly discovered biopsy-proven pulmonary sarcoidosis in roentgenographic stage II (adenopathy and interstitial abnormalities) who also had abnormal pulmonary function (TLC and/or DCO less than 80 percent predicted) were assigned in alternate sequence to either a daily or alternate day prednisone treatment protocol. Both groups showed statistically significant improvement in pulmonary function (TLC, FVC, FEV1, DCO) at three or six months, but there were no significant differences between the two groups. Radiographic adenopathy and interstitial scores (interpreted by blinded readers in random sequence according to a quantitative scale) also showed significant improvement in both groups at three or six months, and again no significant difference was noted between the two groups. Thus, both daily and alternate day prednisone regimens were effective therapy for stage II sarcoidosis. Alternate day therapy may be the treatment of choice for those patients at high risk for significant adverse steroid effects.
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The recognition of aspergillus in all its clinical disguises remains a challenge to clinicians in all fields of medicine. Fortunately, aspergillus has low pathogenicity for humans and requires very heavy inoculum of spores (aspergillary pneumonitis) in order to infect those whose pulmonary defense mechanisms, inherent structure, and physiology are intact. Patients with problems from aspergillus may be seen in either inpatient or outpatient clinical practice. The patient with fibrotic or cavitary lung disease finds himself at risk to be colonized and develop an aspergilloma (fungus ball). Conservative therapy (that is, antibiotics, pulmonary hygiene) or simple observation is often all that is required. With significant hemoptysis, surgical removal could be definitive treatment; but these patients often have such compromised pulmonary function that alternative therapies like infusion of antifungal agents locally are tempting. Part of the problem of the patient with asthma or COPD may actually be secondary to hypersensitivity to aspergillus (ABPA), which exacerbates bronchospasm and adds "pulmonary infiltrates" to the underlying disease. The recognition of this entity and then the judicious use of corticosteroids to control the symptoms will stabilize the clinical course of the disease. The immunocompromised patient may be relatively free of pulmonary disease.; but aspergillus, waiting until cytotoxic agents, corticosteroids, granulocytopenia, broad-spectrum antibiotics, and previous pneumonias destroy the local lung defense mechanisms, will then attack with a vengeance. The resultant invasive pulmonary aspergillosis requires treatment with amphotericin B, along with its own inherent toxicity.