Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The diagnostic value of the nasal biopsy in the early diagnosis of Wegener's granulomatosis and its value in prognosis were examined in 11 patients with a clinicopathological diagnosis of the disease. The vascular lesions found included microabscess in the vascular walls in 82%, leukocytoclastic capillaritis in 73%, fibrinoid necrosis of blood vessels in 45%, leukocytoclastic endovasculitis in 27%, and palisading granuloma in vascular wall in 9% of cases. The extravascular lesions included palisading granuloma in all cases, microabscess in 91%, and diffuse granulomatous tissues in 82%. Palisading microgranuloma (82%) was more frequent than palisading macrogranuloma (45%). After therapy, complete remission occurred in 8 patients, but 3 patients died of sepsis, diffuse pulmonary haemorrhage, and cerebral haemorrhage. Comparison of the frequency of each finding in the nasal biopsy specimens between patients who achieved remission and those who died showed that leukocytoclastic vasculitis was found more commonly in fatal cases, and leukocytoclastic endovasculitis was observed only in fatal cases. Palisading granuloma as a vascular or extravascular lesion is the primary and most important finding in a histopathological diagnosis of Wegener's granulomatosis, microabscess in vascular walls is a secondary but the next most important finding, and leukocytoclastic vasculitis heralds dissemination of the disease and poor prognosis. It requires aggressive therapy.
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PMID:Nasal biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis. Significance of palisading granuloma and leukocytoclastic vasculitis. 864 64

Horton giant cell arteritis can present with an atypical clinical picture that often resembles other diseases. In the case described below, the patient initially demonstrated clinical and laboratory evidence of a Candida albicans sepsis, and therefore we started antimycotic treatment with amphotericin B. Because of an adverse reaction to that drug, we added parenteral steroids before every administration of the antimycotic which led to an unexpected improvement of symptoms. This result caused us to reconsider some clinical aspects that could have been interpreted also as vasculitis, in particular for a giant cell arteritis: throbbing temporal headache, diffuse weakness, important rise in ESR, myoarthralgias. We performed a biopsy of the temporal artery that confirmed our diagnosis.
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PMID:[An atypical presentation of a case of Horton's giant-cell arteritis]. 868 82

We retrospectively analyzed the outcome of bone marrow transplantation (BMT) performed in 26 patients with Wiskott-Aldrich syndrome (WAS) in one center. Twenty-eight transplantation procedures were performed. Ten unselected patients received unmanipulated marrow from a donor with genetically identical human leukocyte antigen (HLA). Eight patients were cured and survive 1.5 to 16.5 years after BMT. One patient successfully received a T-cell-depleted marrow from a matched unrelated donor. Sixteen patients were selected to receive a related HLA partially incompatible BMT because of the occurrence of life-threatening complications from the WAS (i.e., refractory thrombocytopenia, autoimmunity including vasculitis and sepsis). All but one received T-cell-depleted marrow after a conditioning regimen of busulfan and cyclophosphamide. One patient had two BMTs. Engraftment occurred in 12 of 17 attempts. The addition of monoclonal antibodies to lymphocyte function-associated antigen-1 and CD2 molecules appeared to improve engraftment. Six patients were long-term survivors, whereas others died of viral infections (n = 7), among which Epstein-Barr virus-induced B-lymphocyte proliferative disorder was predominant. Delay in development of full T- and B-cell functions accounted for severe infectious complications. These results confirm the excellent outcome of HLA genetically identical BMT in WAS, whereas BMT from HLA partially incompatible donors should be strictly restricted to patients with severe complications of WAS.
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PMID:Bone marrow transplantation in 26 patients with Wiskott-Aldrich syndrome from a single center. 876 21

Today, clinicians can choose from a variety of extracorporeal immunomodulatory procedures such as plasma exchange, double filtration, immunoadsorption, chemoadsorption, photopheresis, and cytoapheresis. The mechanisms underlying extracorporeal immunomodulation (ECIM) comprise removal of pathogenic antibodies and circulating immune complexes as well as reticuloendothelial system deblockage; modification of immune complex structure and processing can be induced by changing the antigen/antibody ratio and by modulation of immune complex solubility via complement activation. Finally, cellular components like lymphocyte subsets, can be modified. Clinical examples of ECIM include lupus erythematosus, Goodpasture's syndrome, anti-neutrophil cytoplasmatic antibodies-mediated systemic vasculitis, myasthenia gravis, and, hypothetically, sepsis.
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PMID:Current status in extracorporeal immunomodulation: immune disorders. 885 4

We report here a case of Cogan's syndrome associated with systemic vasculitis as well as myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA)-related glomerulonephritis. A 71-year-old woman with the diagnosis of aortitis syndrome and pulmonary fibrosis for 7 years, complained of vertigo and hearing impairment. A diagnosis of serous otitis media was made. Although steroid therapy was effective, the symptoms relapsed several times. Seven months after the first manifestation of aural symptoms, she developed painful red eyes bilaterally and proteinuria. On admission, perinuclear ANCA without cytoplasmic ANCA was detected by indirect immunofluorescence assay and MOP-ANCA was detected by enzyme linked immunosorbent assay using the 363 ELISA Unit. Renal biopsy showed necrotizing crescentic glomerulonephritis without immune deposits. A diagnosis of atypical Cogan's syndrome with systemic vasculitis and pulmonary fibrosis was made from the clinical and histological findings. As nephrotic syndrome progressed after admission, she was started on high-dose corticosteroid administration. Urinary protein and other symptoms, except for hearing acuity, improved in parallel with a decrease in the MPO-ANCA titer to normal values. While tapering the dose of corticosteroid, the MPO-ANCA titer increased again and dyspnea occurred. Although pulse methylpredonisolone therapy was performed, the patient died of respiratory failure complicated with sepsis. Postmortem lung biopsy showed pulmonary fibrosis and massive alveolar hemorrhage. The findings of this case study suggest that MPO-ANCA may be closely related to the pathogenesis of Cogan's syndrome.
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PMID:[A case of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA)-related glomerulonephritis associated with Cogan's syndrome]. 891 96

We report the case of a 77-year-old man admitted nine days after being commenced on azathioprine with symptoms initially thought to be secondary to sepsis but in fact due to azathioprine hypersensitivity. He developed histologically proven cutaneous leucocytoclastic vasculitis following the re-introduction of azathioprine. We review the literature concerning adverse reactions to azathioprine and the problems of making the diagnosis as well as highlighting azathioprine as a novel cause of leucocytoclastic vasculitis.
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PMID:Leucocytoclastic vasculitis in a patient with azathioprine hypersensitivity. 893 9

The objective of this study was to assess the relationship between amniotic fluid volume (AFV) and fetal movements at < 32 weeks gestation as assessed by routine biophysical profile (BPP). From a database of 465 consecutive nonhypertensive, nondiabetic patients delivering at < 32 weeks gestation, patients with singleton, nonanomalous fetuses with AFV and fetal movements determined as part of a BPP assessment within 24 hours of delivery were studied. Amniotic fluid volume was scored 0 to 2, according to the following criteria: largest pocket in vertical diameter < 1 cm = 0; < 2 but > 1 cm = 1; > or = 2 cm = 2. Fetal movements (FM) were scored over 30 minutes: 0 if absent, 1 if 1 to 2 movements, 2 if > or = 3 gross (limb/trunk) movements. Variables assessed included fetal presentation, gestational age (GA), premature rupture of membranes (PROM) as a principal indication for delivery, clinical chorioamnionitis (diagnosed by previously published criteria), histologic parameters of infection (in amnion and umbilical cord assessed by a single pathologist blinded to clinical data), and neonatal outcome. Statistical analyses included contingency tables and analysis of variance with p < 0.05 considered significant. Three hundred and fifty-two patients met the inclusion criteria. One hundred and sixty-seven patients (47%) had PROM as a primary indication for delivery. Infrequently, decreased fetal well-being manifested by a BPP < 7 of 10 points was an indication for delivery despite prematurity (n = 7). Of the 352 patients, 80 (23%) had AFV = 0, 60 (17%) had AFV = 1, and 212 (60%) had AFV = 2; and 12 (3%) had FM = 0, 30 (9%) FM = 1, and 310 (88%) FM = 2. There was a significant correlation between decreased AFV and decreased fetal movements (p < 0.0001). Fetal presentation and GA were not significantly different between patients based on score of fetal movements. The incidence of clinical chorioamnionitis was significantly greater in patients with FM = 0 (p < 0.005). We conclude that decreased AFV is associated with decreased fetal movements irrespective of fetal presentation or gestational age. Neonatal outcome (umbilical vasculitis, sepsis, intraventricular hemorrhage) is affected only in unusual cases in which otherwise uncompromised (nonhypoxic, nonacidotic) fetuses have low scores on both these antepartum ultrasonographic parameters.
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PMID:Decreased amniotic fluid volume at < 32 weeks of gestation is associated with decreased fetal movements. 898 78

Pseudomona vasculitis is an exceptional disease. Only a few cases have been reported, non with oropharyngeal involvement. The case of a 30-year-old, HIV-positive man who suddenly developed septicemia and necrotizing lesions with tissue destruction of the oropharynx is reported. Histological study confirmed vasculitis. Pseudomona aeruginosa was isolated in peripheral blood and in the biopsy of the palatal lesion. Antibiotic treatment produced satisfactory results.
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PMID:[Vasculitis caused by Pseudomonas: a case report]. 899 11

Extravascular fibrin formation and dissolution is a pivotal event in numerous inflammatory and malignant diseases. In inflammatory cells such as monocytes/macrophages, neutrophil granulocytes appear to interact intimately with hemostasis and regulate the activity of the cascade systems of coagulation and fibrinolysis. Proteases such as neutrophil elastase are thought to influence components of hemostasis, and furthermore provide an alternative pathway of fibrinolysis. Histological, experimental, and clinical data suggest that extravascular fibrinolysis, mediated both by the plasmin system and by proteases like neutrophil elastase, is a prominent finding in various diseases such as lung cancer, chronic inflammatory bowel disease, vasculitis and connective tissue disease, bacterial sepsis, and septic shock.
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PMID:The role of inflammatory cells and their proteases in extravascular fibrinolysis. 912 14

Human natural immunoglobulin (HunIg) preparation for intravenous use has been used in various diseases. The most typical application of this preparation is agammaglobulinemia. Currently, however, this preparation is being used in the therapy of many other disorders. These include thrombocytopenia, Kawasaki disease, systemic vasculitis, several other disorders of autoimmune origin and systemic inflammation such as sepsis. In some diseases, the clinical improvement following use of HunIg has been dramatic, while in others its effect is not striking. Due to rarity of the side effects, the range of application of HunIg has been recently broadened. Such side effects include transmission of several diseases such as hepatitis and retroviral infections. Before it is recommended for use, however, and primarily due to expense, the efficacy of this drug should be carefully evaluated. The mechanism of action of HunIg is not fully understood. However, it has been suggested that its action may involve blockade of Fc-receptor, an anti-cytokine effect, or inhibition of complement activation. In this review, the mechanism of action of HunIgG and its application in human diseases are discussed.
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PMID:Therapeutic application of intravenous human natural immunoglobulin preparation. 915 42


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