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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A life-long bleeding disorder is described, characterized by hemorrhage occurring after surgery, injury, or dental extraction, and finally by spontaneous intracerebral bleeding. No abnormality of platelet function or plasma coagulation was demonstrable, but grossly enhanced overall fibrinolytic activity was present. The patient had, additionally, a hyperlipidemia with gross arterial atheroma and a family history of myocardial infarction but not of any hemorrhagic disorder. Laboratory studies led to the conclusion that the enhanced fibrinolysis was due to consistently greatly raised levels of a plasma plasminogen activator physically and immunologically related to that in human tissues and blood vessel endothelium. No deficiency of any known inhibitor of fibrinolysis was detected. Free plasmin was not detectable in functional assays but continuous intravascular plasmin generation clearly occurred as evidenced by presence of plasmin-alpha 2-antiplasmin complexes and of fibrin/fibrinogen-related antigens. Excessive production of plasminogen activator appeared to have occurred throughout life and to be independent of the hyperlipidemia. The pathologically increased fibrinolytic activity may have accounted for the complete absence of detectable thrombotic vascular occlusion at autopsy despite extensive arterial disease with severe narrowing of coronary and cerebral arteries.
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PMID:A new life-long hemorrhagic disorder due to excess plasminogen activator. 668 88

Abruptio placentae tends to be accompanied by abnormal coagulation and fibrinolysis, and is known as high risk pregnancy allowing both mother and fetal lives to endanger. Blood coagulation and fibrinolytic system in abruptio placentae, in particular, sequential changes around its onset have been remained unclarified despite the progress in hematology. This permits an irregular therapeutical process for its acute clinical manifestation. In this study, observation was made of the changes of coagulation and fibrinolytic system mainly in relation to delivery, and hemorrhagic diathesis in abruptio placentae was studied. The results obtained are as follows. The studied patients were 14 cases classified into 8 multipara, 10 toxemia of pregnancy, 6 cesarean section, 11 intrauterine fetal death, and 8 complicated disseminated intravascular coagulation respectively. A moderate type demonstrating an increased serum FDP with retarded ESR and lowering in fibrinogen level were observed, but those abnormalities recovered to normal by 3 days after delivery. In serious case, observations also were made of marked decrease of fibrinogen, prolonged PT and PTT, high concentrations of serum and urinary FDP, lowering in levels of coagulation factors, attenuated platelet counts, retarded ESR, and inhibition of platelet aggregation along with thrombelastgram that showed thrombocytopenic type. All of those anomalies tended to be improved following delivery.
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PMID:[Alterations in coagulation and fibrinolytic system in abruptio placentae (author's transl)]. 706 57

Post-operative venous thrombosis, blood loss and pre- and post-operative plasma heparin concentrations were studied in a prospective double blind trial with low-dose heparin therapy in 59 patients undergoing transvesical prostatectomy. Thrombosis rate, diagnosed with the 125I-fibrinogen method, was significantly reduced in the first 5 post-operative days, i.e. during but not after the period of heparin therapy. One patient who developed major thrombosis in spite of heparin prophylaxis is presented. Heparin therapy did not increase average blood loss, but was suspected to be the cause of severe bleeding in 1 patient, who may have had a latent hemorrhagic diathesis. Plasma heparin levels were significantly raised during heparin therapy, and were significantly lower in both heparin and placebo treated patients on days when thromboses started.
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PMID:The effects of low-dose heparin treatment on patients undergoing transvesical prostatectomy. 725 29

Peritoneovenous shunting with the LeVeen valve is generally recognized as an effective procedure for the treatment of intractable ascites and renal failure associated with severe liver disease. We recently observed a generalized hemorrhagic diathesis in patients receiving these valves. To investigate the mechanism of this hemorrhagic complication, we prospectively performed kinetic studies with 51Cr-labelled platelets and 125I-labelled fibrinogen in ten patients. When results of studies before and after valve insertion were compared, the following reductions were noted: fibrinogen concentration, 55%; the fibrinogen survival, 49%; the platelet count, 55% and the platelet survival, 35%. No endotoxin was detectable in ascitic fluid preoperatively, and there was no apparent relationship between ascitic fluid cell counts and changes in fibrinogen and platelet survival. Until the component or components of ascitic fluid responsible for accelerated consumption can be identified and steps are taken to modify the rates of platelet and fibrinogen consumption, it would seem prudent to select patients for surgery conservatively.
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PMID:Accelerated fibrinogen and platelet destruction after peritoneovenous shunting. 725 73

Disseminated intravascular coagulation (DIC) is an extremely rare complication during elective brain tumor surgery. We report the case of a life-threatening intraoperative hemorrhagic diathesis due to a fulminating DIC during the removal of a grade III parietooccipital astrocytoma in a patient with a history of three pulmonary embolisms. Intraoperatively, the patient required 13 U of blood, 9 U of fresh-frozen plasma, and 5.45 L of colloids and crystalloids (total volume infused during the procedure: 12.5 L). Bleeding persisted for 24 h and required further blood component therapy. Laboratory data support the diagnosis of DIC: decreased fibrinogen and platelet count, prolonged thrombin and prothrombin times, and the presence of fibrin monomers. With aggressive and swift treatment of the DIC, the patient survived with transient neurological worsening.
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PMID:Life-threatening hemorrhagic diathesis due to disseminated intravascular coagulation during elective brain tumor surgery. 788 Dec 37

Clot formation is the final result of interaction among multiple plasma proteins; after activation, it results in the conversion of fibrinogen to fibrin and cross-linking of fibrin by activated factor XIII, which stabilizes the formed clot. Deficiency or functional abnormality of the factors involved in these reactions causes bleeding disorders. Natural inhibitors of clotting factors include antithrombin III, protein S, and protein C. When activated, these proteins inactivate specific clotting factors, providing a regulatory mechanism that serves to control the coagulation response and limit the extension of the clot. Physiologic or natural inhibitors should not be confused with acquired inhibitors of coagulation factors, which are discussed in this review. Inhibitors to coagulation factors, also known as circulating anticoagulants, are antibodies that neutralize specific clotting proteins, thereby interfering with their normal function. Antibodies may be directed against isolated clotting factors, as is the case with factor VIII or IX inhibitors. On the other hand, the antiphospholipid antibodies are known to develop against multiple coagulation proteins. In contrast to patients with antibodies against isolated clotting factors, who commonly present with spontaneous bleeding, individuals with antiphospholipid antibodies may be asymptomatic or present with venous or arterial thrombosis. In this article I refer to inhibitors developing in patients with hemophilia A or other congenital factor deficiency as alloantibodies, and to spontaneous formation of antibodies in patients without prior history of hemorrhagic diathesis as autoantibodies. The antiphospholipid antibodies are discussed separately.
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PMID:Inhibitors to clotting factors. 932 77

We report a case of spontaneous intracerebral hemorrhage occurring in a young woman and revealing Addison's disease. This autoimmune primary adrenocortical insufficiency was associated with premature ovarian failure. Exhaustive research for nonhypertensive causes of intracerebral hemorrhage was negative. Initial coagulation studies disclosed severe hypofibrinogenemia and prolonged prothrombin time related to vitamin K-dependent coagulation factor deficit. Clotting abnormalities cleared at 4 months under treatment with hydrocortisone. Glucocorticoids are potent regulators of fibrinogen biosynthesis, increasing fibrinogen secretion. We conclude that primary adrenocortical insufficiency induced this hemorrhagic diathesis leading to spontaneous intracerebral hemorrhage. This latter has never been reported in Addison's disease. Primary adrenocortical insufficiency should be considered as a rare potential cause of nonhypertensive intracerebral hemorrhage.
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PMID:Spontaneous intracerebral hemorrhage revealing Addison's disease. 968 65

A 34 year old male bitten by an adult Atheris squamiger snake developed symptoms of nausea, vomiting, diarrhea which were followed by drowsiness and impaired breathing. Local hemorrhage, edema and pain at the bite-site occurred, but no systemic bleeding or hemorrhagic diathesis developed. All clinical and laboratory parameters were in the normal range except for afibrinogenemia, thrombocytopenia and slight proteinuria. Replacement therapy (fibrinogen and platelet concentrates) and treatment of shock stabilized the patient within 2d and coagulation returned to normal. Atheris squamiger venom was subjected to biochemical and biological analysis. The LD50 of the venom was 5 mg/kg (mice, s.c.). It produced local hemorrhage corresponding to about 25% of the activity of puff adder venom (Bitis arietans). In vitro the venom had a fibrinogen-converting activity, it did not activate purified prothrombin but very likely contained a F V and Ca2+-dependent prothrombin activator. The venom exhibited strong platelet-aggregating activity, which was not inhibited by protease inhibitors and by EDTA or EGTA. The venom also aggregated acetylsalicylic acid treated platelets indicating, that the arachidonic acid pathway was not essential for activation. Rat serum rapidly inhibited the platelet-aggregating activity of the venom; human serum, however, had only a partial inhibitory effect. Preliminary experiments showed that platelet-aggregating activity may be separated from fibrinogen-converting activity by anion-exchange chromatography.
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PMID:Severe coagulopathy after a bite of a green bush viper (Atheris squamiger): case report and biochemical analysis of the venom. 972 32

The bleeding syndrome of acute promyelocytic leukemia (APL) is complex and consists of disseminated intravascular coagulation (DIC) and hyperfibrinolysis. Elastase, derived from malignant promyelocytes, is believed to mediate the fibrinogeno- and fibrinolysis by aspecific proteolysis. In this study we measured the role of elastase in fifteen patients with APL by using an assay for elastase degraded fibrin(ogen) and the results were compared with those obtained in patients with sepsis induced DIC. High levels of elastase were observed in sepsis and APL. The levels of fibrinogen and fibrin degradation products were significantly higher in APL patients compared to patients with sepsis induced DIC. Nevertheless, the level of elastase degraded fibrin(ogen) was higher in the sepsis group (635.3 ng/ml, compared to 144.3 ng/ml in APL; p <0.0001). So, the enormous increase in fibrin and fibrinogen degradation products in APL cannot be explained by elastase activity. This study suggests a minor role for elastase mediated proteolysis in the hemorrhagic diathesis in APL patients.
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PMID:Elastase mediated fibrinolysis in acute promyelocytic leukemia. 1089 47

Congenital afibrinogenemia is a rare autosomal recessive disorder characterized by a hemorrhagic diathesis of variable severity. Although more than 100 families with this disorder have been described, genetic defects have been characterized in few cases. An investigation of a young propositus, offspring of a consanguineous marriage, with undetectable levels of functional and quantitative fibrinogen, was conducted. Sequence analysis of the fibrinogen genes showed a homozygous G-to-A mutation at the fifth nucleotide (nt 2395) of the third intervening sequence (IVS) of the gamma-chain gene. Her first-degree relatives, who had approximately half the normal fibrinogen values and showed concordance between functional and immunologic levels, were heterozygtes. The G-to-A change predicts the disappearance of a donor splice site. After transfection with a construct, containing either the wild-type or the mutated sequence, cells with the mutant construct showed an aberrant messenger RNA (mRNA), consistent with skipping of exon 3, but not the expected mRNA. Sequencing of the abnormal mRNA showed the complete absence of exon 3. Skipping of exon 3 predicts the deletion of amino acid sequence from residue 16 to residue 75 and shifting of reading frame at amino acid 76 with a premature stop codon within exon 4 at position 77. Thus, the truncated gamma-chain gene product would not interact with other chains to form the mature fibrinogen molecule. The current findings show that mutations within highly conserved IVS regions of fibrinogen genes could affect the efficiency of normal splicing, giving rise to congenital afibrinogenemia.
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PMID:A G-to-A mutation in IVS-3 of the human gamma fibrinogen gene causing afibrinogenemia due to abnormal RNA splicing. 1100 3


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