Cases reported "Pulmonary Fibrosis"

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1/75. Combination of membrane oxygenator support and pulmonary lavage for acute respiratory failure.

    A 24-year-old woman with chronic granulocytic leukemia and alveolar proteinosis required extracorporeal membrane oxygenator support for respiratory failure refractory to conventional therapy. During perfusion, each lung was lavaged with 10 L. of normal saline. The lavage led to marked clearing of the lungs and improvement in pulmonary function. Extracorporeal support was terminated successfully after 54 hours. The patient died 2 weeks later with bone marrow insufficiency and overwhelming sepsis. Pulmonary lavage is technically feasible during venovenous oxygenator bypass, and may be of value, since such lavage debrides alveoli as well as the bronchial tree. Because pulmonary lavage provides a possible means of improving pulmonary function, it seems worthy of consideration as an adjunct to membrane oxygenator support.
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2/75. sarcoidosis and common variable immunodeficiency. A case of a malignant course of sarcoidosis in conjunction with severe impairment of the cellular and humoral immune system.

    The occurrence of sarcoidosis in combination with common variable immunodeficiency (CVID) has been described in a small number of patients. In these patients, sarcoidosis consisted of lymphadenopathy, mild to moderate pulmonary involvement and hepatosplenomegaly. However, severe and rapidly progressive pulmonary fibrosis in combination with a severe defect of the cellular and humoral immune system has not been described yet. In our patient, defects of the T and B cell system resulted in severe immunodeficiency. The defect of the humoral immune system was characterized by the impairment of specific antibody production in vivo. In addition, hypogammaglobulinemia with missing IgA and IgE along with a marked defect in IgM and IgG production was noted. There was a progressively reduced lymphocyte proliferation in response to T cell mitogens, while proliferation after specific IL-2 stimulation was normal. A Th1 lymphocyte-subset-like profile might thus play a role in the pathogenesis and might form the connecting link between sarcoidosis and CVID. This is the report of a so far new and unique combination of severe immunodeficiency and sarcoidosis also associated with a congenital dysmorphia consisting of a palatal cleft. The findings of the 40 patients with CVID and sarcoidosis reported so far are discussed in order to point out the typical features of patients with this uncommon syndrome.
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ranking = 0.061122831473281
keywords = deficiency
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3/75. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen.

    We present a case of persistent spontaneous pneumomediastinum precipitated by an upper respiratory infection in a patient with interstitial fibrosis associated with rheumatoid arthritis who was receiving chronic corticosteroid treatment. The persistent nature of the mediastinal emphysema over 2 months eventually required treatment with high concentrations of inhaled oxygen that resulted in rapid resolution of the pneumomediastinum without recurrence over 6 months of follow-up. This case, along with others in the medical literature, emphasizes the need for early use of high-concentration inhaled oxygen in the treatment of pneumomediastinum in high-risk patients, such as those with connective tissue disorders.
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ranking = 0.85714285714286
keywords = oxygen
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4/75. Haemophilia B in a female caused by skewed inactivation of the normal X-chromosome.

    hemophilia b (factor ix deficiency) is an X-linked recessive disorder with a prevalence of 1:30,000 male births, which rarely affects females. A missense mutation T38R (6488C>G) of the factor ix (FIX) gene was characterized in a young female with moderate-to-severe hemophilia b. She is heterozygous for this mutation, which she inherited from her carrier mother. Analysis of the methyl-sensitive HpaII sites in the first exon of the human androgen-receptor locus indicated a de novo skewed X-chromosomal inactivation. This indicates that the paternal X-chromosome carrying her normal FIX gene is the inactive one, which has led to the phenotypic expression of hemophilia b in this patient.
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ranking = 0.0087318330676115
keywords = deficiency
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5/75. Use of nitric oxide for decompensated right ventricular failure and circulatory shock after cardiac arrest.

    We describe a case of peri-operative cardiac arrest, severe right ventricular failure and pulmonary hypertension in a 60-yr-old woman with interstitial pulmonary fibrosis. Inhaled nitric oxide therapy rapidly improved arterial oxygenation and haemodynamic variables, allowing recovery and weaning from mechanical ventilation. Subsequently, the patient was discharged from the cardiac intensive care unit.
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6/75. Pulmonary interstitial fibrosis following near-drowning and exposure to short-term high oxygen concentrations.

    Following near-drowning in fresh water, a 19-year-old man experienced severe adult respiratory distress syndrome, necessitating ventilatory support with positive end-expiratory pressure and high oxygen concentrations. Post-extubation, his course was highlighted by persistent hypoxemia and interrupted by a lung abscess which responded promptly to antibiotics. Pulmonary function tests were consistent with severe restrictive disease and chest radiograph revealed persistent bilateral alveolar and interstitial infiltrates. An open lung biopsy on the 26th hospital day showed interstitial fibrosis. Over the ensuing two months, the chest radiograph and pulmonary function tests returned towards normal. We attribute the pulmonary fibrosis to incomplete resolution of the alveolar interstitial pathology secondary to the near-drowning and exposure to high oxygen mixtures.
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keywords = oxygen
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7/75. Outpatient inhaled nitric oxide in a patient with idiopathic pulmonary fibrosis: a bridge to lung transplantation.

    Inhaled nitric oxide (INO) has been shown to improve oxygenation and decrease intrapulmonary shunt and pulmonary hypertension in various lung diseases. In this study we report a patient with end-stage idiopathic pulmonary fibrosis and pulmonary hypertension who received INO after coronary artery bypass surgery, with significant improvement in arterial oxygenation and pulmonary arterial pressure. Using a pulsing delivery system, the patient continued to receive outpatient INO for 30 months while waiting for lung transplantation. Exercise study and two-dimensional echocardiogram, after 3 months of inhaled NO, demonstrated continued benefits of INO for improvement of arterial oxygenation, pulmonary arterial pressure and exercise tolerance.
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ranking = 0.42857142857143
keywords = oxygen
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8/75. Migratory pulmonary infiltrates in a patient with rheumatoid arthritis.

    The case history is described of an elderly man with rheumatoid arthritis receiving treatment with sulfasalazine and the cyclooxygenase-2 inhibitor celecoxib who presented with severe shortness of breath, cough, and decreased exercise tolerance. The chest radiograph showed unilateral alveolo-interstitial infiltrates and a biopsy specimen of the lung parenchyma showed changes consistent with acute eosinophilic pneumonia. Antibiotic treatment was unsuccessful, but treatment with steroids and discontinuation of sulfasalazine and celecoxib resulted in a marked clinical improvement confirmed by arterial blood gas analysis. The condition may have developed as an adverse reaction either to sulfasalazine or to celecoxib, although hypersensitivity to the latter has not previously been reported.
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ranking = 0.14285714285714
keywords = oxygen
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9/75. Treatment of secondary spontaneous pneumothorax complicating silicosis and progressive massive fibrosis.

    To clarify the management and treatment for the refractory cases of secondary spontaneous pneumothorax (SSP), we analyzed the clinical features in SSP complicating three cases of advanced silicosis, and discussed the available treatment. All three cases were males of age ranging from 60 to 70 years, and had silicosis with massive progressive fibrosis (PMF), classified as type 4 (PR4) according to the ILO guidelines. There was no correlation between the onset of SSP and the smoking habit, or the duration of the occupational exposure to silica. In a total of ten episodes of SSP, a refractory episode occurred in each of the three patients. No surgical treatment was possible because of some complications. Therefore, we administered conservative treatments under mechanical ventilation. The conservative treatments used were tube drainage with suction in each episode and pleurodesis by the combination of minocycline and OK-432 in one case. Approximately one month was the average time required for the air leak cessation. A significant decline in arterial oxygen tension (PaO2) was observed after the treatment of one case, suggesting further respiratory deterioration. These results imply that the more aggressive treatments for refractory SSP should be limited because of the patient status and progression. More information might be required before performing these options safely and effectively.
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ranking = 0.14285714285714
keywords = oxygen
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10/75. Combined methylprednisolone and dexamethasone therapy for paraquat poisoning.

    OBJECTIVE: To report a severe case of paraquat poisoning successfully treated with repeated-pulse therapy of methylprednisolone. DESIGN: Case study. SETTING: University Hospital, Lin-Kou Medical Center, Taipei, taiwan, Republic of china. patients: A 60-yr-old man with paraquat poisoning with severe acute renal failure (serum creatinine level of 11.8 mg/dL and serum paraquat level of 3.66 microg/mL at 10 hrs after ingestion) and severe hypoxemia (Pao2, 66.6 mm Hg). INTERVENTION: Repeated 3-day pulse therapy with methylprednisolone, one course of 2-day cyclophosphamide, and a high dose of dexamethasone for 33 days. MEASUREMENTS AND MAIN OUTCOME: Arterial blood gas analysis was obtained regularly. A chest radiography was obtained every week. The arterial blood oxygen concentrations dramatically elevated from 66 mm Hg to 97 mm Hg, and the chest radiographs markedly improved after repeated-pulse therapy with anti-inflammatory agents and cyclophosphamide. CONCLUSIONS: We successfully treated a severe paraquat poisoned patient with repeated methylprednisolone pulse therapy and prolonged dexamethasone treatment. This case demonstrates that the severe inflammation, not the fibrosis, of the lungs plays a major role in the lethal hypoxemia of patients with paraquat poisoning during the subacute period and confirms our previous hypotheses. Clearly, the use of anti-inflammatory therapy to treat paraquat-poisoned patients needs further evaluation; however, anti-inflammatory therapy may be an effective treatment after failure of standard therapies.
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ranking = 0.14285714285714
keywords = oxygen
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