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1/12. Evaluating and monitoring the adolescent with pulmonary function testing.

    This article reviews pulmonary function tests (quantitative measurements of physiologic functioning of lungs, chest wall, and respiratory muscles) as valuable tools for the diagnosis and management of chronic respiratory disease in adolescents. Advances in technology have made pulmonary function testing more available and, for some tests, easier to perform. Pulmonary function evaluation is used for diagnosis of pulmonary disease. Testing will identify abnormal physiologic processes that result in obstructive or restrictive pulmonary disease and, together with a complete history, physical examination, and other tests, help to determine the specific etiology. Pulmonary function measurement may be used to manage a patientis disease over time. Quantitative evaluation of the response to many specific therapies, such as asthma medications and environmental control, can only be achieved by measuring pulmonary function. Monitoring pulmonary toxicity of medications, such as chemotherapeutic agents, is also done by measuring pulmonary function. In many patients with chronic pulmonary disease, such as cystic fibrosis, the pulmonary function laboratory can be used to assess the patient's physiologic limitation.
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2/12. inhalation of decomposed chlorodifluoromethane (freon-22) and myocardial infarction.

    After exposure to decomposed chlorodifluoromethane (freon-22), a 65-year-old man developed respiratory symptoms such as cough, blood-stained sputum, and increasing dyspnea. Three weeks later, his family doctor diagnosed infectious bronchitis. Another week later he died due to myocardial infarction. The discussion focuses on an inflammatory process caused by the inhalation of decomposed freon and its possible association with myocardial infarction.
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3/12. Laryngo-tracheo-bronchial stenosis in a patient with primary pulmonary amyloidosis: a case report and brief review.

    PURPOSE: To report a case of lower respiratory tract obstruction occurring in a patient with primary pulmonary amyloidosis and discuss anesthetic management. CLINICAL FEATURES: A 53-yr-old man was referred to our institution for microlaryngoscopy and laser treatment of the larynx. He presented with a five-year history of primary laryngo-tracheo-bronchial amyloidosis and symptoms consistent with narrowing of the conducting airways. General anesthesia was induced with iv propofol 150 mg and remifentanil 50 microg. Mivacurium 20 mg provided muscle relaxation for endotracheal intubation. Following endotracheal intubation, the airway became obstructed and patient ventilation impossible. The endotracheal tube was removed and a Dedo laryngoscope inserted. Gas exchange was maintained using supraglottic jet ventilation via a distal port of the laryngoscope. Rigid bronchoscopy showed tissue partially obstructing the lumen of the lower trachea. This was removed and the airway appeared patent. At the end of the case, a further episode of lower airway obstruction occurred requiring reinsertion of the laryngoscope and resumption of jet ventilation. Extensive debridement through the bronchoscope was required before adequate ventilation could be restored. Some days later when the patient's condition deteriorated again and he required further debridement of the trachea and insertion of a tracheostomy, guide wires were positioned in the femoral vessels in the event that cardiopulmonary bypass was required for gas exchange. CONCLUSIONS: Primary laryngo-tracheo-bronchial amyloidosis is a recurrent disease, requiring repetitive surgical procedures. Airway compromise can be a persistent problem. awareness of this uncommon disease process and its presentation may serve to caution the anesthesiologist presented with this type of case.
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4/12. Pulmonary manifestations of the eosinophilia-myalgia syndrome associated with tryptophan ingestion.

    Pulmonary manifestations are not infrequent in the L-tryptophan-induced eosinophilia-myalgia syndrome (EMS). However, previous reports have not described the results of longitudinal pulmonary function, exercise testing, high-resolution computerized tomographic (HRCT) scanning of the chest, or detailed bronchoalveolar lavage (BAL) analysis. We report six patients with EMS who had dyspnea. The diffusing capacity for carbon monoxide was decreased in five patients tested. exercise testing with arterial blood gas sampling in three patients was consistent with pulmonary vascular or parenchymal disease. Serial exercise testing in two of these patients demonstrated marked improvement temporally associated with corticosteroid treatment. In four patients, HRCT scanning of the chest was abnormal. One of these patients showed no abnormality on routine chest roentgenogram. Two patients undergoing BAL exhibited increased eosinophils in the lavage fluid; a third had elevated lymphocytes. Serial measurements of fibroblast proliferation-stimulating-activity in samples of BAL fluid obtained from serial examinations in two patients exhibited heightened pretreatment activity that returned to the normal range following corticosteroid therapy. In these two patients, increased proportions of T-suppressor/cytolytic (CD8 ) cells were observed in the BAL fluid. Despite aggressive immunosuppressive therapy, one of the patients died of respiratory failure. Another remains markedly dyspneic with pulmonary hypertension. Of the remaining four patients, two exhibited resolution of pulmonary symptoms after systemic corticosteroid therapy, and two experienced partial improvement.
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5/12. Complex extra-intestinal complications of ulcerative colitis in a patient with alpha1-antitrypsin deficiency.

    Ulcerative colitis (UC) can manifest with a variety of extra-intestinal disorders frequently affecting the skin, joints, and liver. An aetiologic role of alpha1-antitrypsin deficiency in chronic inflammatory bowel disease has recently been suggested. We report a patient with UC and alpha1-antitrypsin deficiency who presented with disseminated cutaneous leucocytoclastic vasculitis clinically appearing with target-like purpuric patches and haemorrhagic oedemas. In addition, he displayed acute haemorrhage of the eyes and the respiratory tract consistent with a systemic vasculitic process. Moreover, he had autoimmune haemolytic anaemia. Systemic vasculitides, such as Wegener's granulomatosis, churg-strauss syndrome, and microscopic polyangiitis, could widely be excluded. Systemically administered glucocorticosteroids and azathioprine led to dramatic improvement of extra-intestinal symptoms. On the basis of alpha1-antitrypsin deficiency and UC, the present patient likely developed severe systemic vasculitis with multi-organ involvement. UC should at times be viewed within the context of a more generalized immune imbalance affecting multiple organs, and not as an isolated pathological entity. Testing for alpha1-antitrypsin deficiency in UC patients may detect individuals at higher risk of severe extra-intestinal involvement.
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6/12. Respiratory abnormalities among photographic developers: a report of three cases.

    Photographic developing processes employ hazardous chemical substances, many with known adverse effects on the respiratory system. Three cases documenting respiratory symptoms and suggesting small airway abnormalities among photographic developers are reported. work-related factors which might contribute to these findings, as well as public health implications, are discussed.
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7/12. Pulmonary manifestations of juvenile laryngotracheal papillomatosis.

    Juvenile laryngotracheal papillomatosis spreads to involve the lungs in less than 1% of cases, and when this occurs, the prognosis is poor. In seven such cases, the lung lesions, which appeared either solid or cystic on radiographs, proved to be benign squamous cell proliferations or papillomas, with central cavities containing debris or air. They seemed to grow centrifugally, using the alveolar walls as scaffolding with central coalescence and lung destruction. Papillomas spread inferiorly from the larynx by direct extension as far as the major bronchi, but rarely beyond. However, the parenchymal lesions were widely scattered, and some were subpleural. This discrepancy suggests that fragments become detached, particularly during endoscopic resection, and are carried down the airways by airflow. Those that lodge proximal to the respiratory bronchioles may be removed by mucociliary action and cough. Those that travel more distally are poorly cleared and may grow. If enough lung parenchyma is destroyed, the patient can develop symptoms of restrictive lung disease in addition to signs of upper airway obstruction.
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8/12. Cardiorespiratory disease associated with Hallermann-Streiff syndrome: analysis of craniofacial morphology by cephalometric roentgenograms.

    This paper analyzes the craniofacial morphology in a patient with typical Hallermann-Streiff syndrome (HSS) who developed symptomatic cardiorespiratory deficiency at the age of 48 years. The patient had obstructive sleep apnea (OSA), hypoxia, hypercarbia, pulmonary hypertension, tricuspid insufficiency, and right ventricular failure. Analysis of cephalometric roentgenograms, done 15 years earlier, revealed severe mandibular hypoplasia with marked underdevelopment of the ramus and body. The gonial angle was abnormally obtuse. The condylar and coronoid processes were reduced in size. The anteroposterior dimension of the upper airway was markedly narrowed. Cephalometric roentgenograms of six other HSS patients from our clinic were compared to those of the reference patient. Considerable variation in the features of the syndrome were noted. None of the other patients showed definitive airway obstruction. Comparison was also made with cephalometric roentgenograms of a patient with Treacher Collins syndrome and of a patient with progeria. The former showed airway obstruction associated with a deformed hypoplastic mandible; the latter had an unobstructed airway despite a small mandible because of associated hypoplasia of the maxilla and tongue. The HSS reference patient improved after oxygen therapy, diuretics, antibiotics, and relief of OSA. patients with HSS, as well as those with Treacher Collins syndrome, appear to be at risk for the development of cardiopulmonary disease if they have obstructed airways. OSA has been shown to have developed in two patients with HSS. The resultant cardiopulmonary insufficiency of such patients may be preventable if airway obstruction can be relieved relatively early in life.
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9/12. Respiratory diseases and food allergy.

    Both upper and lower respiratory tracts can be affected by food allergy. Manifestations in either may be exclusively due to food allergy (common in infants) or may result from the combined effects of food allergy plus another defect such as gastroesophageal reflux, a congenital defect of the heart or tracheo-bronchial tree, an immunodeficiency syndrome such as isolated IgA or IgG4 deficiency, or a concomitant inhalant allergy. Chronic rhinitis is the most common respiratory tract manifestation of food allergy. When it occurs in conjunction with lung disease, it may be a helpful indicator of activity of the allergic lung disease and of the patient's compliance in following a specific diet. Recurrent serous otitis media may be solely or partially due to food allergy. Large tonsillar and adenoid tissues, sometimes with upper airway obstruction, may be caused, or aggravated by, food allergies. Lower respiratory tract disease manifested by chronic coughing, wheezing, pulmonary infiltrates, or alveolar bleeding may also occur. Lower respiratory tract involvement is generally associated with a greater delay in onset of symptoms and with a larger quantity of allergen ingestion than chronic rhinitis. food allergy should be considered when there is a history of prior intolerance to a food in childhood or of symptoms beginning soon after a particular food was introduced into the diet. It is an important consideration in patients who have chronic respiratory tract disease which does not respond adequately to the usual therapeutic measures and is otherwise unexplained.(ABSTRACT TRUNCATED AT 250 WORDS)
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10/12. Physical assessment series: the thorax and the lungs.

    The anatomy of the thorax and lungs and the physiology or respiration are complex. The physical assessment techniques which, on the surface, appear relatively easy to master, require hours of practice of observation, palpation, percussion, and auscultation before the examiner becomes familiar with the more subtle changes of disease process and the wide variations of normal that may be confused with illness. These are skills that may be quickly lost if not regularly applied. But perhaps with the use of such techniques, the pharmacist can play an even greater role in the monitoring of drug therapy.
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