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)

Since 1984 the peritoneovenous shunt has been installed in 33 patients (10 females - 30.3%; 23 males - 69.7%) of the average age of 54 +/- 8, all in the phase of therapeutically resistant ascites (alcoholic cirrhosis 28 - 84.85%; 4 - 12.12% posthepatitic cirrhosis; and 1 - 3.03% hepatic amyloidosis). The control group consisted of 39 patients (11 females - 28.2% and 28 males - 71.8%) treated in an identical time span with the strict conduction of medicament-diet therapy. The aim of this study was to check the value of this method on our own clinical-patient material, and therefore establish the incidence of complications. By the use of a unique protocol we followed mortality, morbidity, body weight, belly circumference, diuresis, the ultrasonographic finding of the abdominal cavity and the complications which appeared. Out of the group operated on 19 (57%) of the patients died, and so did all the control group patients as well. The average life duration was 275 +/- 810 days in the group operated on, and 44 +/- 29 (p less than 0.005) in the control group. All those alive (14.33-42.42%) lived longer than six months. Six patients lived longer than one year (42.85%), 4 (28.47%) longer than two years, and one (7.14%) longer than three years. There is a statistically significant decrease in body weight, belly circumference, diuresis increase and the consequent ascites withdrawal. DIC occurred in 2 patients, shunt malposition in 2, saccular dilatation in 1, plastic peritonitis in 6, and ileus in 1 patient. Not one of the listed complications resulted by death.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Therapy of resistant ascites using the peritoneojugular (Leveen shunt)]. 228 15

Hemostatic plugs consist of platelet aggregates and fibrin mesh containing blood cells and plasma components. Hemostatic efficiency depends on the rate of formation of hemostatic plugs as well as the structural integrity and stability of the formed hemostatic plugs. Fibrin elements are major constituents contributing to the structural integrity and stability, but they are subject to fibrinolytic activity occurring spontaneously after fibrin formation. Fibrinolysis is usually suppressed by endogenous inhibitors. Increase of a profibrinolytic component or deficiency of an inhibitor would result in an accelerated fibrinolysis, causing a premature lysis of hemostatic plugs before restoration of injured vessels, leading to a hemorrhagic tendency. Such a state can be seen typically in patients with congenital deficiency of alpha 2-plasmin inhibitor or a hereditary increase of plasminogen activator, and it is also seen in acquired situations such as amyloidosis, liver cirrhosis, disseminated intravascular coagulation (particularly in patients with acute promyelocytic leukemia) and thrombolytic therapy. The hemorrhagic tendency can be well controlled by an administration of an antifibrinolytic agent: epsilon-aminocaproic acid or tranexamic acid. In contrast to an accelerated fibrinolysis causing a hemorrhagic tendency, retarded fibrinolysis may predispose an individual to a thrombotic tendency. Retarded fibrinolysis may be due to either an increase in plasminogen activator inhibitors or decrease of plasminogen activators. Quantitative or qualitative deficiency of plasminogen may also lead to a thrombotic tendency.
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PMID:Hemostasis associated with abnormalities of fibrinolysis. 265 Jul 72

The results of many-year studies on the humoral and platelet links of hemostasis in chronic glomerulonephritis (CGN) and amyloidosis were analyzed with relation to a stage of disease and prognosis of its course. Activation of the blood coagulation system (BCS), platelet hyperaggregation and suppression of the fibrinolytic system were revealed in CGN. Hypercoagulation was most noticeable in patients with active and prognostically unfavorable GGN types correlating with the frequency of local (in the kidney) intravascular coagulation, the frequency of peripheral thromboses and DIC-syndrome. In amyloidosis hypercoagulation shifts of BCS were combined with the activation of fibrinolysis and thrombocytopenia. Pathogenetic, adaptive and compensatory significance of changes of system of hemostasis revealed in CGN and amyloidosis was discussed.
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PMID:[Clinical value of the study of hemostasis in nephrology]. 320 76

A few cases of improvement in secondary renal amyloidosis following surgery (in particular, removal of the amylogenic foci) have been published, but cases of aggravation are much more numerous. The authors report on three patients whose renal function deteriorated dramatically after extra-renal surgery (pneumonectomy, aortic valve replacement, mitral valve replacement). None of the usual precipitating factors, such as DIC, cardiovascular collapse, sepsis or renal vein thrombosis, could be detected, but two patients had been under extracorporeal circulation. Such accidents appear to be unpredictable and irreversible. They can be seen in primary or secondary amyloidosis and whether or not surgery involves an amylogenic focus. Indeed, in two of their patients the diagnosis of amyloidosis was unknown before the operation. This suggests that in patients with suspected amyloidosis no major surgical operation should be undertaken without prior renal biopsy.
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PMID:[Dramatic aggravation of renal amyloidosis after surgery. Three cases (author's transl)]. 730 69

Coagulation disorders usually confront the emergency physician as bleeding episodes or as abnormalities of laboratory tests. Bleeding has to be treated aggressively, while pathological coagulation tests should be related to a more differentiated diagnosis at first. The most common causes of acquired coagulation disorders are liver disease, vitamin K deficiency, and disseminated intravascular coagulation (DIC). More rarely, inhibitors, external factors such as drugs or extracorporeal circulation, or other diseases such as amyloidosis are present. Since localized hemorrhage is the most common bleeding source in liver disease, endoscopic and surgical therapeutic measures, respectively, are warranted. Careful and balanced substitution therapy according to laboratory findings should be initiated simultaneously and should consist of fresh frozen plasma (FFP), which contains all components of the coagulation system physiologically balanced. Prothrombin complex concentrates should be used in emergency situations only, keeping their potential hazards in mind. Adequate vitamin K substitution is indicated in liver disease as well as in coagulopathy due to vitamin K deficiency. Management of DIC primarily consists of aggressive treatment of the underlying disease. Substitution therapy is difficult and should be carefully monitored by the adequate laboratory tests. FFP is the adequate source of both procoagulants and inhibitors but may cause certain problems. Heparin therapy can be beneficial but is not recommended generally. Antithrombin III substitution cannot be assumed as established therapy so far. Inhibitors can lead to bleeding, but the most common inhibitor, lupus anticoagulant, rather predisposes to thrombosis. In bleeding patients with inhibitors against single clotting factors, treatment consists of adequate substitution before initiating the diagnostic workup.
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PMID:Management of acquired coagulation disorders in emergency and intensive-care medicine. 871 94

Adult onset Still's disease was first reported by Bywaters in 1971. It is a systemic inflammatory disorder of unknown etiology, characterized by spiking fever, macular rash and polyarthritis. Although the prognosis is generally good, severe cases have been published. They include those with disseminated intravascular coagulation (DIC), hemophagocytosis, amyloidosis and respiratory failure. Among them, DIC is not uncommon. Prednisolone in a dose of 20-60 mg/day is required when patients fail to respond to nonsteroidal anti-inflammatory drugs (NSAIDs) or when they are accompanied by complications including pleuritis, pericarditis, liver dysfunction, severe arthritis and DIC. Recently, disease-modifying antirheumatic drugs (DMARDs) and immunosuppressive agents including cyclophosphamide and methotrexate have been shown to be effective for alleviating refractory cases and chronic arthritis.
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PMID:[Adult Still's disease]. 1007 10

This article describes the first autopsy case of heme oxygenase (HO)-1 deficiency. A 6-year-old boy who presented with growth retardation; anemia; leukocytosis; thrombocytosis; coagulation abnormality; elevated levels of haptoglobin, ferritin, and heme in serum; a low serum bilirubin concentration; and hyperlipidemia was diagnosed as HO-1 deficient by gene analysis several months before death. Autopsy showed amyloid deposits in the liver and adrenal glands and mesangioproliferative glomerular changes in kidneys, in addition to an irregular distribution of foamy macrophages with iron pigments. Fatty streaks and fibrous plaques were noted in the aorta. Compared with HO-1--targeted mice, the present case seems to more severely involve endothelial cells and the reticuloendothelial system, resulting in intravascular hemolysis, disseminated intravascular coagulation, and amyloidosis with a short survival. This contrasts to the predominant iron metabolic disorders of HO-1--targeted mice with a long survival.
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PMID:Heme oxygenase-1 deficiency: the first autopsy case. 1182 83

We document a case of 61-year-old woman with a 24 year history of rheumatoid arthritis (RA), who developed severe polyarthralgia, dry cough, paresthesia in the legs, frequent micturition, and severe macrohematuria. We diagnosed as severe RA with extraarticular manifestations based on interstitial pneumonia, mononeuritis multiplex, subcutaneous nodules, and high titer of rheumatoid factor. Ultrasonography demonstrated an intravesical mass lesion. A histological examination of the urinary bladder by endoscopic biopsy revealed marked deposition of AA amyloid. The diagnosis of secondary amyloidosis and bacterial cystitis were made based on histological findings and urine culture. At first, we administered antibiotics by intravenous infusion, which resulted in cure of cystitis and partial improvement of macrohematuria. Then combination therapy of corticosteroids and cyclophosphamide successfully reduced the disease activity of RA. There have only been a few reports published so far on the vesical amyloidosis in patients with RA. However, 5 of 10 patients (50%) in vesical amyloidosis died because of continuous massive hematuria, which induced disseminated intravascular coagulation and multiple organ failure. In conclusion, secondary amyloidosis of the urinary bladder should be considered as a possible cause of hematuria in patients with long-term RA and as an important prognosis factor of RA.
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PMID:[A case of rheumatoid arthritis with secondary amyloidosis in urinary bladder]. 1216 17

Pathologic informatics is an analysis of mechanism of pathogenesis using various medical information. Biologic reactions by many reactants are studied on molecules, cells, organs, and individuals by using various methods. Themes of our laboratory are as follows: 1) to analyze of the pathogenesis of various diseases by using novel diagnostic methods, 2) to elucidate amyloid formation mechanism in amyloidosis and to develop novel therapies, 3) to develop the methods for tests and diagnosis for DIC, 4) to study on activation mechanism of ribosomal proteins in the tissues by the blood cell function, 5) to study on the expression and suppression of neogenesis of blood vessels by physical invasion in the organ failure, and 6) to develop the diagnostic methods for autonomic dysfunction and to analyze the pathogenesis.
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PMID:[Introduction of Department of Diagnostic Medicine, Graduate School of Medical Sciences, Kumamoto University]. 1713 92

In 1854, the term "amyloid" was first used in the description of a liver specimen at autopsy by Virchow. The kidneys and heart are the most commonly involved organs in amyloidosis; liver and gastrointestinal tract involvement is less common, and the symptoms are usually mild. Here, we report the case of a 57-year-old male patient who presented with oral hemorrhagic bullae, thrombocytopenia and jaundice. Disseminated intravascular coagulation profile was positive. Abdominal sonography showed ascites, and abdominal computed tomography disclosed heterogeneous enhancement of the liver, with focal low attenuation regions and splenomegaly with poor contrast enhancement. Liver decompensation was highly suspected. Diagnostic laparoscopy with liver biopsy and colonoscopic biopsy from the rectum were subsequently performed. Typical apple-green birefringence was demonstrated on polarized light microscopy by Congo red staining. Systemic amyloidosis was diagnosed and colchicine prescribed. However, liver function deteriorated and intermittent gastrointestinal bleeding was found during the patient's hospitalization. The patient died due to uncorrectable coagulopathy and massive gastrointestinal bleeding. The final diagnosis was idiopathic amyloidosis with hepatic failure. Although amyloidosis rarely presents with hepatic failure, it should be considered in patients with signs of liver decompensation. Clinicians should be aware of this rare but potentially lethal presentation and arrange appropriate treatment promptly.
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PMID:Systemic amyloidosis manifesting as a rare cause of hepatic failure. 2023 Oct 2


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