Cases reported "Pulmonary Fibrosis"

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1/87. 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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2/87. Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation.

    An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.
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3/87. Coexistent lymphoid interstitial pneumonia, pernicious anemia, and agammaglobulinemia.

    immunologic factors have been incriminated in the pathogenesis of lymphoid interstitial pneumonia. The discovery of a patient with coexistent lymphoid interestitial pneumonia, pernicious anemia, and common variable hypogammaglobulinemia focused attention on the possible autoimmune nature of this pulmonary disease. Extensive immunologic studies demonstrated a noticeably impaired bonemarrow-dependent (B cell) system and intact thymus-dependent (T cell) system. No evidence of humoral or cellular hypersensitivity to homologous lung determinants was found.
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4/87. A patient with exacerbation of idiopathic pulmonary fibrosis which was resolved probably due to the coexisting hyperbilirubinemia?

    This report presents the case of a patient with corticosteroid and cyclophosphamide resistant exacerbation of idiopathic pulmonary fibrosis (IPF), which was definitely resolved in accordance with increased levels of serum conjugated bilirubin due to biliary tract obstruction. Histological examination of the lung showed an accumulation of bile pigments in the alveolar mural tissues, especially in the cytoplasm of the alveolar macrophages, which play crucial roles in the development of IPF. This case suggests that bile pigments have some important roles in tissue protection against inflammatory damage in IPF, and may illustrate an important key for treatment of this fatal disorder.
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5/87. erdheim-chester disease. A case report.

    A 63-year old man had a history of diabetes insipidus, arthralgias and myalgias, weight loss, relapsing fever and malaise. Increased uptake of Tc-99m was found predominantly in distal antebrachia, in distal femurs and in both trochanters and tibias on the bone scintigraphy. The chest radiograph showed reticulonodular pattern and the high resolution computed tomography (HRCT) scans revealed diffuse infiltrative lung disease with small multiple nodules and widening of interlobular septs. Videothoracoscopic lung biopsy and biopsy of tibial lesion were perfomed. The histopathologic examination proved non-Langerhans cell histiocytosis-erdheim-chester disease. Treatment with prednisone reduced the pain and fever and improved the vital capacity of the lungs while the changes in the lungs and bones remained unchanged.
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6/87. Mixed myelodysplastic syndrome and myeloproliferative disorder with bone marrow and pulmonary fibrosis: the role of megakaryocytes.

    The case of an 80-year-old woman displaying myelodysplastic syndrome evolving into a myeloproliferative disorder with myelofibrosis and pulmonary fibrosis, is reported. This case is characterized by an initial presentation of a myelodysplastic syndrome with normal karyotype and moderate fibrosis, its evolution towards a myeloproliferative disorder with myelofibrosis and the worsening of pulmonary fibrosis in parallel to the acceleration of the myeloproliferative disorder and myelofibrosis. These features and the high concentration of plasma platelet factor-4 suggest a role of megakaryocyte/platelet degranulation in the development of fibrosis.
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7/87. Upper lobe pulmonary fibrosis associated with high-dose chemotherapy containing BCNU for bone marrow transplantation.

    Upper lobe fibrotic lung disease is most often associated with sarcoidosis, Langerhans cell histiocytosis, silicosis, and other pneumoconioses but is usually not associated with drug-induced lung disease. carmustine (BCNU) is a chemotherapeutic agent known to cause pulmonary toxicity. The radiographic pattern is usually diffuse bilateral lung disease predominantly in the lung bases. Upper lobe fibrotic disease associated with BCNU has been reported to occur in children treated for central nervous system gliomas. Often the lung disease occurs years after the exposure. Despite the widespread use of BCNU in the treatment of malignancy in adults, to our knowledge, the complication of upper lobe fibrotic disease has not been reported in adults. We describe a patient who presented with pneumothorax and bilateral upper lobe pulmonary fibrosis that we believe was due to BCNU given for bone marrow transplantation as part of therapy for breast cancer. Bilateral upper lobe pulmonary fibrosis can be associated with chemotherapeutic drugs.
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8/87. Fatal lipoid pneumonia due to bronco-aspiration of isoparaffin after ingestion of an organophosphate insecticide.

    A 66-year-old-male patient with a history of depression voluntarily ingested around 400 ml of an insecticide composed of 5% methylparathion, 75% isoparaffin, 8% etoxylated oleic acid, 4% 1,2,4-trimethylbenzene, 6% naphtha, 1% 1,3,5- trimethylbenzene, 0.4% propylbenzene and 0.3% xylene. The patient was conscious and alert at admission. gastric lavage was performed and activated charcoal administered. There were no clinical symptoms of organophospate ingestion despite reduced concentrations of erythrocyte and plasma cholinesterase. Chest X-ray showed pulmonary infiltrate compatible with bronco-aspiration. The patient evolved to respiratory failure refractory to treatment and died from multiorganic failure 23 days after ingesting the insecticide. The pathological findings included a pulmonary fibrosis in the alveolar spaces which caused enlargement of the intra-alveolar septa. Abundant lipin-laden macrophages were observed within the alveolar spaces. We review the most relevant aspects of cases of fatal lipoid pneumonia and point out that on occasion severe or fatal intoxication is due to the substances accompanying the active ingredients.
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9/87. Extensive diffuse pulmonary ossification.

    Diffuse lung injury can either heal normally or progress to fibrosis. Calcification in the setting of fibrosis is common. The appearance of mature woven bone is not. We report a patient with extensive diffuse pulmonary ossification and discuss some of the theories relating to the development of this phenomenon.
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10/87. bone marrow transplantation for chronic myelogenous leukemia in blastic phase using a phenotypically identical unrelated volunteer donor. Nagoya bone marrow transplantation Group (NBMTG), Tokai Marrow Donor Bank (TMDB).

    A 26-year-old male with chronic myelogenous leukemia in lymphoid blast crisis received a bone marrow transplant (BMT) from a phenotypically identical, mixed lymphocyte reaction (MLR)-weakly positive unrelated male volunteer donor. The volunteer was obtained from the Tokai Marrow Donor Bank (TMDB), which was established in japan in 1989. This donor was selected from volunteer donors who were identical with our patient at the HLA-A,B loci, followed by matching at HLA-DQ, DR loci. On MLR testing, the donor's cells showed no response, but the patient's cells showed a low response to the donor's cells (relative response index 0.29). The patient showed rapid hemopoietic engraftment. He developed acute graft-versus-host disease (GVHD) with vesicle formation on palms and soles and mild liver damage, which were successfully treated with intravenous prednisolone 1 mg/kg per day. Although he also suffered from interstitial pneumonitis on day 64 and localized varicella-zoster infection on day 87, and has suffered from moderate stomatitis and dry skin characteristic of chronic GVHD, he is currently 22 months post-transplant with hematological remission and has a normal daily social life.
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