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)

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

A 72-year-old woman was admitted because of anorexia and dyspnea. She was given a diagnosis of pulmonary hemorrhage and renal failure. Despite treatment with high-dose steroid and hemodialysis, the patient died of disseminated intravascular coagulation on the 9th hospital day. Autopsy revealed intra-alveolar hemorrhage, crescentic glomerulonephritis, and systemic vasculitis with fibrinoid necrosis. A direct immunofluorescence study demonstrated linear deposition of IgG along the glomerular basement membrane (GBM). Both anti-GBM antibody and anti-neutrophil cytoplasmic antibody with perinuclear pattern (P-ANCA) were detected in the patient's serum by enzyme immunoassay. Goodpasture's syndrome with P-ANCA was diagnosed. There has been some controversy as to whether vasculitis occurs in patients with Goodpasture's syndrome. This was a rare example of well-documented Goodpasture's syndrome with P-ANCA and systemic vasculitis, the exact etiologic relationships among which remain to be clarified.
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PMID:[An autopsy case of Goodpasture's syndrome with P-ANCA and systemic vasculitis]. 1008 78

Pauci-immune glomerulonephritis, i.e., with no evidence of immune deposits in the blood vessel, is the most prevalent form of rapidly progressive glomerulonephritis (RPGN). In the pathogenesis of pauci-immune renal disease inflammation of blood vessels in the presence of circulating anti-neutrophil cytoplasm antibodies (ANCA) takes place. However the lack of ANCA (about 5-30% of patients) does not exclude pauci-immune vasculitis. The patients without circulating ANCA might have fewer extrarenal symptoms than those who are ANCA-positive. We describe a case of a 40-year old women with ANCA-negative renal limited pauci-immune small-vessel vasculitis with rapidly decreasing kidney function. She was ineffectively treated with plasmapheresis combined with a puls of cyclophosphamide (i.v.) and 3 pulses of methyloprednisolone (i.v.). The patient progressed to end-stage renal disease and should be treated with renal replacement therapy. In differential diagnosis we excluded other causes of pauci-immune vasculitis (Churg-Strauss syndrome, Wegener's granulomatosis), vasculitis with immune complexes deposition (systemic lupus erythematosus, Schoenlein-Henoch purpura, post-infection RPGN), Goodpasture disease, haemolytic-uremic syndrome (HUS), disseminated intravascular coagulation (DIC) and Wilson's disease.
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PMID:[Rapidly progressive ANCA-negative glomerulonephritis in the course of pauci immune microscopic vasculitis with hemolytic anemia probable in the course of Wilson's disease]. 2175 23

Renal complications of influenza A virus infections are uncommon but can contribute to a deterioration in the patient's condition, which include acute kidney injury (AKI) in critically ill patients, rhabdomyolysis, hemolytic uremic syndrome (HUS), acute glomerulonephritis (AGN), disseminated intravascular coagulation (DIC), Goodpasture's syndrome, and acute tubulointerstitial nephritis (TIN). The clinical characteristics of AKI in critically ill patients with pandemic influenza A(H1N1) 2009 virus (A(H1N1)pdm09) infection are similar to uninfected patients. Underlying conditions associated with AKI include older age, diabetes mellitus, obesity, pregnancy, history of asthma, and chronic kidney disease. Histologic examination of the kidneys from patients with A(H1N1)pdm09 infection who died include acute tubular necrosis (ATN), myoglobin pigment, and DIC. A(H1N1)pdm09 is present in the kidneys of some patients. The clinical characteristics of patients with rhabdomyolysis associated with influenza A include younger age and the frequent occurrence of muscle symptoms. AKI occurs in approximately one third of patients with rhabdomyolysis due to influenza A. HUS is associated with A(H1N1)pdm09 as follows: Streptococcus pneumoniae-associated HUS following A(H1N1)pdm09 infection, HUS triggered by A(H1N1)pdm09 in patients with genetic complement dysregulation, and HUS associated with A(H1N1)pdm09 without known underlying disorder. AGN, Goodpasture's syndrome, and acute TIN are extremely rare complications of influenza A virus infection. Although the pathogenesis underlying renal injuries due to influenza A virus has not been delineated, some hypotheses have been advanced, including ATN due to renal hypoperfusion or rhabdomyolysis, glomerular microthrombosis due to DIC, direct viral injury to the kidney, and an altered immune system with systemic mononuclear cell activation following influenza A virus infections.
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PMID:Renal complications of seasonal and pandemic influenza A virus infections. 2306 28

We report a 28-year-old female with no history of allergies and recent onset of Goodpasture syndrome who developed life-threatening bleeding immediately after placement of a polytetrafluoroethylene (PTFE) graft as an access for hemodialysis in the left upper limb by an experienced vascular surgeon. In spite of transfusing fresh frozen plasma, packed cells and cryoprecipitate, her prothrombin time (PT), activated partial thromboplastin time and international normalized ratio became progressively worse which were normal at the beginning of the surgery. She had profound hypotension and succumbed within 8 hours. We suspect a rare phenomenon of the interaction of her blood with the PTFE graft causing activation of bleeding and coagulation factors leading to disseminated intravascular coagulation (DIC).
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PMID:A rare fatal complication of bleeding immediately following polytetrafluoroethylene arteriovenous grafting in a lady with Goodpasture syndrome. 3025 34