Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A factor V inhibitor arose in a 79-year-old man within 1 month of an operation for a fractured leg. Absorption studies with solid-phase antibodies to human immunoglobulins showed the inhibitory activity to be primarily in the IgG class, but also in the IgA class, of immunoglobulins. This is the first report of an IgA immunoglobulin with factor V inhibitory activity. While the inhibitor was present, and at a time when no circulating Factor V activity was detectable, the patient developed septicemia and disseminated intravascular coagulation. The mechanism sustaining disseminated intravascular coagulation despite the absence of circulation factor V activity remains unexplained. The factor V inhibitor disappeared within 5 months of its initial detection. Possible origins of factor V inhibitors are discussed.
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PMID:Factor V antibody and disseminated intravascular coagulation. 67 79

Host versus graft (HVG) syndrome may be induced in parental strain mice by perinatal inoculations of F1 hybrid spleen cells. The principal manifestations of the disease include thrombocytopaenia, intravascular fibrin deposits, intestinal haemorrhage, hepatic infarcts, lymphosplenomegaly and renal disease. Immune complexes have been shown to be the cause of the renal lesions, and have been implicated as the triggers for disseminated intravascular coagulation. In the present studies of RFM mice perinatally inoculated with (T6 x RFM)F1 spleen cells (RFM/(T6 x RFM)F1 mice), quantitative determinations of serum immunoglobulins (Ig) revealed marked elevations of IgG1, IgG2, IgA and IgM. Electrophoretic analyses revealed the polyclonal pattern which typically follows chronic antigenic stimulation. However, IgG1 levels which reached 29 to 72 times control values suggested disruption of homeostatic mechanisms which control circulating Ig levels. Because antibody responses to histocompatibility antigens were present only occasionally, and then in low titre, it seemed unlikely these antigens were the principal causes of hypergammaglobulinaemia and plasmacytosis. Morphological studies indicated that the elevated levels of Ig seen in end-stage HVG syndrome correlated well with marked plasmacytosis, the third morphological finding in a sequence that included the precocious development of germinal centres and subsequent depletion of thymic-dependent (T) lymphocytes. The fact that spleen cells from RFM/(T6 x RFM)F1 mice were severely impaired in their capacity to cause graft versus host disease in related (T6 x RFM)F1 and unrelated C3H mice provided strong evidence that the HVG reaction resulted in T-cell depletion, rather than specific immunoincompetence.
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PMID:Hyperimmunoglobulinaemia, T-cell deficiency and plasmacytosis in RFM mice with host versus graft disease induced by the perinatal inoculations (T6XRFM)F1 spleen cells. 108 3

Three units of group A blood were inadvertently administered to a group O recipient during surgery without evidence of hemoglobinemia, hemoglobinuria, hypotension, disseminated intravascular coagulation, acute renal tubular necrosis, or other signs and symptoms of transfusion reaction. The recipient had normal concentrations of IgG, IgA, and IgM as well as complement (C3) prior to transfusion and anti-A agglutinins titered to 64 (titer of 128 by the antiglobulin technic). Seventeen hours following the transfusion, 28 per cent of the circulating red blood cells were group A (equivalent to 475 ml of packed cells); they were eliminated by day 5 without evidence of hemoglobinuria, hemoglobinemia or hyperbilirubinemia. Anti-A titers (antiglobulin) had risen from a posttransfusion low of 4 to 4,096 by day 10. After treatment of serum with 2-mercaptoethanol, however, hemolytic activity which was first noted on day 5 was lost and the antiglobulin titer dropped to 24 which suggested that most of the anti-A produced in response to the transfusion was IgM rather than IgG. The anti-A titer had dropped to essentialyy pretransfusion levels and the majority of anti-A present was IgM by day 91. The recipient suffered no untoward effects from the transfusion and was in good health three months following the transfusion.
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PMID:Unusual response to ABO incompatible blood transfusion. 119 85

This is a very rare case report of Goodpasture's syndrome with IgA antibasement membrane antibody. A 43-year old male was admitted because of severe dyspnea with slight hemoptysis. Chest X-ray demonstrated extensive bilateral infiltrates with air bronchogram, predominantly in the right lung. Laboratory data on admission showed severe anemia and moderate renal impairment. The pulmonary infiltrates resolved spontaneously within 10 days. Goodpasture's syndrome or collagen vascular disease was suspected and he underwent a percutaneous renal and transbronchial lung biopsy. The renal biopsy showed crescent formation affecting 70-80% of glomeruli. Linear IgA deposits, but not IgG, were demonstrated along the glomerular basement membrane by the direct immunofluorescence procedure. The lung biopsy contained many hemosiderin-laden macrophages in the lumen of the alveoli and showed mild thickening of alveolar walls. However, linear immunoglobulin deposits on the alveolar capillary basement membrane were not demonstrated by direct immunofluorescence. The diagnosis of Goodpasture's syndrome with IgA antibasement membrane antibody was made. His serum was negative for antibasement antibody by indirect immunofluorescence. He was treated with prednisone, 30 mg daily. His pulmonary symptoms and anemia improved markedly, but his renal function did not change. Thirteen months after his first admission, he suffered from severe bacterial pneumonia, which was complicated by disseminated intravascular coagulation. He died of respiratory failure. Autopsy was rejected.
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PMID:[A case of Goodpasture's syndrome with IgA antibasement membrane antibody]. 221 6

Cholera disease can be induced in the rabbit by duodenal inoculation (DI) of Vibrio cholerae organisms after ligation of the cecum (C) (DIC model). When ligation of the cecum is omitted, no disease symptoms develop. In contrast, the animals are primed which becomes apparent as vibriocidal protection upon challenge with V. cholerae in the DIC model. This protection coincides with high anti-O antigen IgA levels in the bile. The O antigen was shown to be the protective antigen and it must be presented by live organisms. A non-enterotoxigenic mutant of V. cholerae induced protective immunity in the rabbit but was reported to cause mild diarrhea in human volunteers. Looking for alternatives, we applied cholera toxin, known as a mucosal adjuvant, together with killed V. cholerae cells to rabbits. Unfortunately, the minimum adjuvant dose was equal to the minimum toxic dose. A Salmonella typhimurium strain expressing also the V. cholerae O antigen induced systemic rather than local immunity which was not protective. Several Escherichia coli strains were able to elicit a local immune response, but the animal to animal differences were considerable. Therefore, V. cholerae itself was thought to be the most appropriate carrier organism. Some non-enterotoxigenic and auxotrophic mutants of V. cholerae were able to prime and did not show any undesired side-effects in the DIC model. Therefore, further attenuation of non-toxigenic V. cholerae strains by means of stable deletions in nutritional genes seems to be the most promising way to obtain acceptable vaccine candidates.
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PMID:Infection and immunity to Vibrio cholerae, Salmonella typhimurium and Escherichia coli in a rabbit model. 246 84

We reported a family with dentato-rubro-pallido-luysian atrophy (DRPLA) and chronic renal failure. The proband was a 66-year-old woman who developed gait disturbance, limb ataxia, pyramidal tract signs, and dementia since age 54. T2-weighted brain MR images revealed symmetric high-signal lesions in the cerebral white matter, in addition to cerebellar, brainstem, and cerebral cortical atrophy. She suffered from renal failure and became dialysis-dependent at the age of 59, four years after the onset of chronic nephritic syndrome. At the age of 66, she was admitted to our hospital because of hyperthermia and disturbance of consciousness, and died of DIC. Her CAG repeats in the DRPLA gene were 58 and 12. An autopsy was performed. The brain weighed 910 g. Histological findings confirmed the diagnosis of DRPLA. Her mother died of chronic renal failure. All three siblings had cerebellar ataxia, and two siblings had chronic nephritic syndrome. Among them, only her younger brother was diagnosed as non-IgA glomerulonephritis based on kidney biopsy findings at the age of 48. Though the nature of the association between DRPLA and renal dysfunction remains obscure, the DRPLA gene abnormality may be correlated with chronic renal failure in this family.
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PMID:[A family with DRPLA and chronic renal failure]. 1096 59

Human patients suffering from leptospirosis present with a diverse array of clinical manifestations, including the more severe and often fatal pulmonary form of the disease. The etiology of pulmonary hemorrhage is unclear. Isolates of Leptospira acquired from patients suffering from pulmonary hemorrhage were used to develop a guinea pig model of pulmonary hemorrhage. Gross findings post-infection confirmed extensive hemorrhage in the lungs and on peritoneal surfaces as the likely cause of death. Immunohistochemistry confirmed the presence of large numbers of leptospires in kidney, liver, intestinal tissues, and spleen, but few inflammatory cells were seen. In marked contrast, few leptospires were detected in infected hemorrhagic lung tissue. Blood chemistries and hematology did not reveal the etiology of the hemorrhage observed. There was no chemical or microscopic evidence for disseminated intravascular coagulation. To ascertain an immunopathologic role during disease, immunofluorescence was performed on infected lung tissues and confirmed the presence of IgM, IgG, IgA, and C3 along the alveolar basement membrane. This suggests that an autoimmune process may be the etiology of fatal pulmonary hemorrhage in leptospirosis.
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PMID:Alveolar septal deposition of immunoglobulin and complement parallels pulmonary hemorrhage in a guinea pig model of severe pulmonary leptospirosis. 1498 64

The treatment of 120 patients with acute purulent pyelonephritis (APP) brought about the following results. 86% patients given standard therapy showed deterioration of their condition, DIC syndrome and inflammatory process were not cured, pyoinflammatory process in the kidney continued. On the contrary, 84.1% patients on combined conservative treatment improved: DIC syndrome and active inflammation relieved, fibronectin content, IgA, IgM and IgG levels rose. Ultrasonic examination and dopplerography indicated that combined conservative therapy including cryoprecipitate arrested development of a purulent focus in the kidney, reestablished intraorganic circulation. Mean stay in hospital was 13.9 days.
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PMID:[Use of cryoprecipitate in combined therapy of acute purulent pyelonephritis]. 1615 47

A 62 year old woman was referred to our hospital because of acute renal and liver dysfunction. Prior to admission, she had already been started on hemodyalysis filtration(HDF). She showed facial edema and lumbar pain caused by an Ll compressive fracture. Laboratory examinations revealed hypercalcemia (13.2 mg/dL), hyperammonemia (297 microg/dL) and her serum creatinine, blood urea nitrogen and total bilirubin levels were 3.9 mg/dL, 37.4 mg/dL and 3.2 mg/dL, respectively. Among the components of immunoglobulin, IgA was increased, while IgG and IgM were decreased. Serum immunoelectrophoresis revealed the presence of the IgA kappa type of M component. Punched out lesions were noted on her head radiography. Severe plasmacytosis (60-70 % of total cells) were observed by a bone marrow aspiration test, indicating the diagnosis of multiple myeloma. Steroid pulse therapy was started with dexamethasone (40 mg/day, 3 days), and plasma exchange was performed 8 times with continuous HDF. These treatments failed to control hemodynamics and she died of disseminated intravascular coagulation (DIC). Autopsy demonstrated amyloid-like depositions in perisinusoidal space in the liver. In the kidney, there were nodular lesions in the glomeruli, and depositions in the basement membrane of the uriniferous tubuli. Congo red staining of these organs for amyloid yielded negative results. Immunohistochemical staining gave positive results for IgA and kappa. Electron microscopy revealed granular electron deposits in the glomeruli and tubular basement membrane as well. Taken altogether, the diagnosis of the patient could be light chain deposition disease (LCDD).
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PMID:[A case of acute renal and liver dysfunction with light chain deposition disease]. 1757 93

An immune-mediated, severe, acquired prothrombotic disorder, heparin-induced thrombocytopenia type II (HIT II) occurs in 0.5-5% of patients exposed to unfractionated heparin longer than 5-7 days. Arterial and venous thromboses are induced by HIT II in about 35-50% of patients. Typical death rate for HIT is about 29%, while 21% of HIT patients result in amputation of a limb. The trend towards the occurrence of HIT due to the administration of low molecular weight heparins (LMWH) taking ever conspicuous place in the standard venous thromboembolism (VTE) prophylaxis has been more frequently observed recently. It is considered that LMWH may cause HIT II in about 0.25-1%. The need for further modification of HIPA assays with LMWH has been imposed in the HIT laboratory diagnostics, heretofore overburdened with complexity. There are several constantly opposing problems arising in HIT laboratory diagnostics, one of which is that in a certain number of patients immunologic assays detect nonpathogenic antibodies (mainly IgM or IgA heparin-PF4 antibodies) while, on the other hand, the occurrence of HIT pathogenetically mediated by minor antigens (neutrophil-activating peptide 2 or interleukin 8) may be neglected in certain cases. The following factors play an important role in the interpretation of each laboratory HIT assays performed: 1. correlation with HIT clinical probability test, the best known of which is 4T'score, 2. the interpretation of the laboratory findings dependent on the time of the thrombocytopenia onset, as well as 3. the sensitivity and specificity of each test respectively. The HIT diagnostics in the presence of other comorbid states which may also induce thrombocytopenia, more precisely known as pseudo HIT (cancer, sepsis, disseminated intravascular coagulation, pulmonary embolism, antiphospholipid syndrome, etc), represents a specific clinical problem.
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PMID:Obstacles in the diagnostics and therapy of heparin-induced thrombocytopenia. 2022 87


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