Cases reported "Chronic Disease"

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1/22. Massive tracheal necrosis due to compression by an innominate artery aneurysm associated with a grade IV Chagasic megaesophagus and chronic duodenal ulcer.

    A 49-year-old man suffered necrosis of the cephalad tracheal segment due to compression by an innominate artery aneurysm. A peritracheal abscess, a grade IV chagasic megaesophagus, and a duodenal ulcer were also present. The patient underwent a three-stage surgical treatment, and 7 years later he is doing well, and breathing and eating normally.
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2/22. Intemittent obstruction of the upper airway during sleep causing profound hypoxaemia. A neglected mechanism exacerbating chronic respiratory failure.

    An obese patient with a ten year history of respiratory failure presented with insomnia and marked daytime somnolence. Respriatory failure had been attributed to obesity, respiratory centre insensitivity to carbon dioxide, and to diffuse airways obstruction. To investigate the possible role of episodic apnoea with frequent nocturnal arousals, continous recordings were obtained during sleep of arterial oxygen saturation, oesophageal pressure and the motions of the rib-cage and abdomen/diaphragm. Repeated episodes of hypoventilation and profound hypoxaemia were found which were due to intermittent obstruction of the upper airway rather than to cessation of breathing efforts. During the episodes of hypoxaemia, values of arterial O2 tension fell to as low as 24 mmHg. Episodic hypoxaemia was relieved but not abolished, by the use of a collar, designed to hold the mandible forward. Previous reports indicated that recognition of intermittent obstruction of the upper airway during sleep and treatment by a permanent tracheostomy, resulted in a significant long-term imporvement of pulmonary and cardiac function and relief of insomnia and day-time somnolence. When tracheostomy is inadvisable, as in the present patient, it is hoped that similar long-term benefits will result from a supportive collar.
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3/22. Restructuring the therapeutic environment to promote care and safety for the obese patient.

    Fifty-four percent of American adults are overweight. obesity is a chronic disease associated with a number of conditions, such as diabetes, heart disease, hypertension, certain types of cancers, and breathing problems. The direct and indirect costs related to obesity exceed $70 billion annually. Because of the many cost and quality issues related to obesity, national attention is turning toward the special needs of this population. Strategies to restructure therapeutic intervention with attention to risk management, economic implications, and patient satisfaction are important considerations when managing the obese patient.
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4/22. Venous air embolism during home infusion therapy.

    Venous air embolism (VAE) is a potential complication of surgical procedures as well as central venous access. There are several reports in the literature of VAE during the in-hospital use and placement of central venous access. However, we are unaware of previous cases of VAE in children who received home infusion therapy via central venous access. We report the occurrence of a VAE in a 2-year-old with a Broviac catheter for home intravenous antibiotic therapy. VAE occurred when a bolus of air was unintentionally administered as the mother removed the cassette from the pump when it was alarming air in line. The cassette and tubing had been placed into the pump without a fluid flush. After the tubing and cassette were removed from the pump, the air in the line was allowed to flow by gravity into the patient, resulting in the immediate onset of respiratory and neurologic symptoms. The mother administered 2 rescue breaths, and the child's color and breathing returned to normal over the next 2 minutes. After the child arrived in the emergency department, the child's mental status returned to normal and the remainder of her physical examination was unremarkable. She had an uneventful recovery and was discharged from the hospital the following day. Additional antibiotic administration was accomplished in the emergency department of a local hospital. VAE can occur spontaneously when there is an open venous structure 5 cm or more above the heart or if air is delivered under pressure into the venous system, such as during a laparoscopy or mishaps with infusion bags. The morbidity and mortality of VAE are related to the volume of air, rate of entrainment, the patient's underlying cardiorespiratory status, and the patient's position. morbidity and mortality occur as a consequence of right ventricular outflow obstruction or end-organ dysfunction from left-sided obstruction of coronary or cerebral vasculature as air passes across a patent foramen ovale or through the pulmonary circulation. Of all the literature pertaining to VAE with central lines, there are no previous reports of VAE occurring during home infusion therapy in children. With managed care requiring shorter hospitalizations and more children being discharged from the hospital on home infusion therapy, parents and lay caregivers are being asked to administer medications and perform routine maintenance on central venous devices. In our case, despite the fact that the mother had been educated regarding the appropriate technique for medication administration, she forgot to purge the air from the line before connecting the tubing and administering the antibiotic. Although the infusion pump will alarm when there is air in the line, it detects air only in a small part of the line and this safety feature is not in play if the device is removed from the infusion pump and administered via gravity. If such safety precautions are not adhered to, then the volume of air that fills the intravenous tubing from the drip chamber to the patient (25-30 mL in the pediatric infusion pump tubing used in our patient) can be infused by gravity into the patient's venous system. Because the consequences of VAE are so severe, the focus should be on prevention. Pumps used for home infusion therapy should have appropriate alarms to alert caregivers to the presence of air in the line. Obviously, this will not totally prevent this complication as this type of pump was used in our patient. It is crucial to educate caregivers of patients with central venous access regarding the hazards of VAE and safety measures to prevent it. With the increased use of home infusion therapy, ongoing evaluations of complications related to this form of therapy are mandatory so that there is continued evaluation of practices and appropriate changes made when necessary to increase further the safety of these techniques.
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5/22. Airway complication following functional endoscopic sinus surgery.

    Difficulty breathing after upper airway surgery requires immediate evaluation and treatment. We present a case of airway compromise after sinus surgery due to edema of the uvula. The patient was admitted for observation overnight and discharged the next day. A discussion of potential airway changes after sinonasal surgery is presented.
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6/22. Management of respiratory failure with noninvasive positive pressure ventilation and heliox adjunct.

    Exhausted by persistent coughing and dyspnea, a 63-year-old man with chronic obstructive pulmonary disease was admitted to the emergency department. Initial treatment included oxygen and pharmacologic and noninvasive positive pressure ventilation (NPPV) therapy with minimal clinical improvement. In view of this situation, a gas mixture of helium-oxygen (heliox) 70%:30% was introduced into the nasal mask as an adjunct. Within 20 minutes of therapy, a marked improvement in arterial blood gases and a reduction in respiratory rate and accessory muscle use was noted. The patient expressed in a high-pitched voice that he was "breathing easier." He remained on NPPV-heliox adjunct for 80 minutes. At the end of this period, the patient was placed on a 50% Venturi oxygen mask. He was transferred to the intensive care unit, and 6 days later he was discharged from the hospital without incident.
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7/22. Strong humming for one hour daily to terminate chronic rhinosinusitis in four days: a case report and hypothesis for action by stimulation of endogenous nasal nitric oxide production.

    Rhinosinusitis is an inflammation or infection of the nose and air pockets (sinuses) above, below and between the eyes which connect with the back of the nose through tiny openings (ostia). Rhinosinusitis can be caused by bacteria, viruses, fungi (molds) and possibly by allergies. Chronic rhinosinusitis (CRS) is an immune disorder caused by fungi. The immune response produced by eosinophils causes the fungi to be attacked, which leads to damage of the sinus membranes, resulting in full-blown rhinosinusitis symptoms. Gaseous nitric oxide (NO) is naturally released in the human respiratory tract. The major part of NO found in exhaled air originates in the nasal airways, although significant production of NO also takes place in the paranasal sinuses. Proper ventilation is essential for maintenance of sinus integrity, and blockage of the ostium is a central event in pathogenesis of sinusitis. Concentrations of NO in the healthy sinuses are high. Nasal NO is known to be increased 15- to 20-fold by humming compared with quiet exhalation. NO is known to be broadly antifungal, antiviral and antibacterial. This case report shows that a subject hummed strongly at a low pitch ( approximately 130 Hz) for 1h (18 hums per minute) at bedtime the first night, and hummed 60-120 times 4 times a day for the following 4 days as treatment for severe CRS. The humming technique was described as being one that maximally increased intranasal vibrations, but less than that required to produce dizziness. The morning after the first 1-h humming session, the subject awoke with a clear nose and found himself breathing easily through his nose for the first time in over 1 month. During the following 4 days, CRS symptoms slightly reoccurred, but with much less intensity each day. By humming 60-120 times four times per day (with a session at bedtime), CRS symptoms were essentially eliminated in 4 days. Coincidentally, the subject's cardiac arrhythmias (PACs) were greatly lessened. It is hypothesized that strong, prolonged humming increased endogenous nasal NO production, thus eliminating CRS by antifungal means.
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8/22. Significant improvement from chronic beryllium disease following corticosteroid pulse therapy.

    Chronic beryllium disease (CBD) is a rare disease characterized by diffuse interstitial pulmonary granulomatosis. We report a case of CBD which exhibited marked improvement both subjectively and objectively following pulse therapy. The patient was a 36-year-old man whose chief complaint was dyspnea and a dry cough. Since July 1990, the patient had been working in the development of an automatic or mechanical technique for producing beryllium-copper alloy. It appeared likely that the patient may have been exposed to metal beryllium fumes generated from an opening located just above the furnace. The Be concentration exceeded 25 microg/m3 transiently in the breathing zone in this workplace. A chest x-ray film taken in October 1994 showed fine granular shadows throughout the entire lung fields. Around August 1998, the patient's dyspnea became aggravated. An X-ray taken at that time showed linear and reticular shadows, in addition to the diffuse fine granular shadow. In October 1998, after 3 days of methylprednisolone pulse therapy, oral prednisolone 30 mg was initiated. With this treatment, the patient's pulmonary function tests and blood gases improved. Once the patient's condition had improved sufficiently, the dosage of prednisolone was decreased by 2.5 mg every two weeks. The patient continues to be monitored.
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9/22. Development and clinical application of a portable oxygen concentrator.

    We have produced a compact, lightweight oxygen concentrator, using a newly-developed polymer of poly [1-(trimethylsilyl)-1-propyne] with a performance, i.e. oxygen permeability, of 61 x 10 cm3 (STP) cm/cm2 s cmHg, which is 17 times higher than that of the membrane material of conventional concentrators. The oxygen and nitrogen selectivity was 1.80. The dimensions of the apparatus are 325 x 180 x 150 mm and it weighs about 4.0 kg. It is actuated by a battery (DC 12 V, 5.6 Ah), with a 100 V AC source and a car battery (12 V) source also available. The power consumption is 70 W. The generated oxygen concentration is about 30%, and the maximum flow rate is 41/min. Applying 10 samples of various chronic pulmonary diseases to the subject, the suction of oxygen from the apparatus in the patients' rest state was conducted. An increase in the PaO2, from an average of 59.1 Torr before the suction to an average of 68.5 Torr after the suction, together with easing of breathing, was indicated. The usefulness of this apparatus for supplying portable oxygen-treatment to patients with chronic respiratory disease is shown.
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10/22. Fatal encephalitis in a patient with chronic graft-versus-host disease.

    A 32-year-old male patient with chronic myelocytic leukemia in accelerated phase received a bone marrow allograft from his 42-year-old HLA/MLC-identical sister. He recovered from acute graft-versus-host disease (GVHD) grade III-IV of skin, liver and gut, but chronic GVHD of progressive onset developed. On day 556 post-graft severe thrombocytopenia was resistant to prednisolone, cyclophosphamide and high dose immunoglobulin. splenectomy was followed by a normalization of platelet counts. The subsequent clinical course was characterized by progressive muscular atrophy and weight loss. Dysphagia, dysarthria, cachexia and ultimately recurrent pneumonic episodes ensued. The cachectic patient developed a highly abnormal breathing pattern with hypoventilation and intermittent apnea requiring mechanical ventilation. Auditory evoked potentials revealed a considerable dysfunction of the brainstem. The patient died on day 1120 post-graft from pneumonia, aggravated by thoracic muscular insufficiency. Postmortem examination revealed diffuse predominantly lymphoid perivascular infiltration in meninges and CNS tissue; proliferation of activated microglial cells expressing the HLA-DR antigen was prominent in the brainstem. These histologic changes are similar to those observed in the CNS in experimental GVHD. We suggest that this case represents the first documentation of CNS involvement in chronic GVHD.
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