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11/36. Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis.

    Open lung biopsy in a 38-year-old female with Pringle-Bourneville syndrome and recurrent pneumothorax revealed a micronodular pneumocyte II hyperplasia, a new entity probably associated with the tuberous sclerosis syndrome. The lesion caused an obstruction of the alveolar lymphatic vessels and alveolar ducts, resulting in an emphysema-like picture. This cystic dilation of alveoli and draining lymphatics followed by rupture caused the recurrent pneumothorax. The epithelial pneumocytic nature of the lesion was confirmed by immunohistochemistry and electronmicroscopy.
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ranking = 1
keywords = emphysema
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12/36. Definitions in chronic obstructive pulmonary disease.

    States of airflow obstruction are common disorders which span the spectrum from asthmatic-chronic bronchitis to emphysema. Asthmatic and chronic bronchitic states are at least potentially reversible by systematic, pharmacologically oriented therapy focusing on bronchodilators and corticosteroids. Both asthmatic bronchitis, particularly when it is not adequately treated, and emphysema result in the final common pathway of COPD. These are generally progressive states, unless smoking cessation can be achieved in early or mild stages of disease. The future focuses on the great challenge of early identification, classification, and intervention. Thus, all patients with cough, dyspnea, and wheeze should be carefully evaluated by health workers who understand the history, physical examination, and simple pulmonary function tests in the context of chest radiology. These clinical methods together can help define the disease states characterized by airflow obstruction. Often, a final definition of disease cannot be made until aggressive attempts at the treatment of the airflow obstruction and its attendant symptoms complex have been vigorously pursued by experienced clinicians.
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ranking = 2
keywords = emphysema
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13/36. Severe bronchospasm followed by respiratory arrest during thallium-dipyridamole imaging.

    We describe the occurrence of sudden severe bronchospasm and respiratory arrest following dipyridamole infusion in a patient with chronic obstructive pulmonary disease predominantly of the emphysematous type. The severe reaction was unexpected because the patient had tolerated well withdrawal of aminophylline derivatives for 48 hours and was receiving chronic prednisone 20 mg qd. Although the diagnostic and prognostic gains from dipyridamole imaging far outweigh the small risk associated with the test, patients with chronic pulmonary obstructive disease must be closely monitored during thallium-dipyridamole imaging.
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ranking = 1
keywords = emphysema
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14/36. Diffuse hepatocellular dysplasia and carcinoma associated with the Mmalton variant of alpha 1-antitrypsin.

    The cirrhosis and hepatocellular carcinoma associated with alpha 1-antitrypsin deficiency has been exclusively reported with the PI Z allele. We present a 63-yr-old white man with emphysema, cirrhosis, and hepatocellular carcinoma. The latter occurred on a background of diffusely distributed hepatocellular dysplasia. serum protein electrophoresis suggested a deficiency of alpha 1-antitrypsin quantitated at 13% of normal. PI phenotyping showed that he had only the rare PI Mmalton allele, previously associated only with severe lung disease. family studies demonstrated the distribution of this rare allele. The liver at autopsy displayed well-differentiated hepatocellular carcinoma in addition to alpha 1-antitrypsin deposits in normal, dysplastic, and malignant cells.
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ranking = 1
keywords = emphysema
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15/36. Laryngeal dysfunction and pulmonary disorder.

    The normal respiratory function of the larynx has been described in detailed reports in both the otolaryngology and the respiratory physiology literature. The role of the posterior cricoarytenoid muscle in vocal cord abduction has been shown to be paramount in laryngeal respiratory function. However, only in recent reports has attention been directed toward disordered laryngeal function as evidenced in pulmonary disorders, such as asthma, or in association with underlying pulmonary disorders, such as asthma (ROAD) or emphysema (COPD). In this article, cases will be presented to demonstrate the role of disordered laryngeal function appearing as pulmonary disease and associated with various degrees of underlying pulmonary disease. The effect on pulmonary function tests and the role of treatment will be discussed.
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ranking = 1
keywords = emphysema
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16/36. The depressed patient with chronic obstructive pulmonary disease.

    The case of a 65-year-old woman with chronic obstructive pulmonary disease is presented to demonstrate the nature of depression in this disease entity. The patient responded to the therapeutic environment on a psychiatric ward that was comforting to patients with chronic medical illnesses. The use of brief psychotherapy and psychopharmacology in patients with emphysema is also reviewed.
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ranking = 1
keywords = emphysema
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17/36. Intravenous use of glycopyrrolate in acute respiratory distress due to bronchospastic pulmonary disease.

    A patient with acute respiratory distress secondary to emphysema and reactive airway disease had inadequate tidal volumes with and without endotracheal intubation. Because of the patient's failure to respond to maximal standard bronchodilator therapy and the physical inability to ventilate the patient by manual positive pressure, he received IV glycopyrrolate (0.2 mg) approximately 50 minutes after admission to the ED. The patient's condition immediately improved, as evidenced by the ability to manually ventilate the patient; he developed increased tidal volumes; and he began responding to inhalation therapy. This is the first reported case of IV glycopyrrolate administration for chronic obstructive pulmonary disease or asthma in the literature and demonstrates an instance in which inhalation therapy was ineffective due to low tidal volumes.
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ranking = 1
keywords = emphysema
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18/36. Right ventricular thrombus and pulmonary emboli in a patient with emphysema: an echocardiographic and Doppler documentation.

    Pulmonary hypertension is a known complication of chronic obstructive pulmonary disease (COPD). A worsening dyspnea in a patient with COPD is usually a sequela of the pulmonary disease. However, it may be due to pulmonary embolism, a complication which is difficult to diagnose in such patients. We present a patient with COPD in whom two-dimensional and Doppler echocardiography confirmed the diagnosis of pulmonary emboli by documenting right ventricular thrombus and pulmonary hypertension. This case report emphasizes the usefulness of these noninvasive cardiac imaging techniques in the detection and evaluation of pulmonary embolism in patients with COPD.
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ranking = 4
keywords = emphysema
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19/36. Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone.

    A 45-year-old woman with cutaneous urticaria-like lesions subsequently developed polyarthritis, glomerulonephritis, and chronic obstructive pulmonary disease. biopsy of skin showed leukocytoclastic vasculitis of superficial capillaries. biopsy of the kidney revealed mesangioproliferative glomerulonephritis, and lung biopsy revealed severe emphysema and thick-walled blood vessels with immunoglobulin deposition. Therapy with dapsone produced dramatic improvement of the patient's cutaneous vasculitis and arthritis. This case uniquely demonstrates the efficacy of dapsone for both the urticarial vasculitic and the arthritic components of this syndrome and reemphasizes the potentially severe pulmonary and renal complications.
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ranking = 1
keywords = emphysema
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20/36. Management of difficult airway problems with percutaneous transtracheal ventilation.

    Percutaneous transtracheal jet ventilation was used in the management of 13 cases of upper airway obstruction. Cases are subdivided according to the nature of the presenting airway problem. The first group had stable upper airway abnormalities; the second group presented as emergencies with rapidly evolving upper airway obstruction. This technique has two major advantages: 1) it provides rapid access to the airway during acute emergencies; and 2) it provides control of the airway where tissue distortion from head and neck trauma or tumor surgery makes standard anesthetic techniques difficult. Complications including catheter displacement, total expiratory obstruction, pneumothorax, and subcutaneous emphysema have been encountered. The authors conclude that percutaneous transtracheal jet ventilation is effective. However, potential problems exist and an in-depth understanding of the technique is necessary for its appropriate application and successful use.
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ranking = 1
keywords = emphysema
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