Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 10-year-old boy had a severe lifelong hemorrhagic disorder that had necessitated more than 50 hospitalizations. Laboratory examination showed prolonged bleeding, clotting, partial thromboplastin, prothrombin, and thrombin times. These findings were due to a potent inhibitor of the thrombin-fibrinogen reaction. This inhibitor was similar to heparin in that it acted immediately and did not interfere with the coagulant activities of certain venoms. It differed from heparin in not being adsorbed to barium citrate or neutralized by protamine sulfate. The inhibitory effect was found in the alpha1-globulin fraction. It was identified immunologically and functionally as a double-banded alpha1-antitrypsin of a previously unreported phenotype. The inhibitory effects were depressed by trypsin and heterologous anti-alpha1-antitrypsin.
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PMID:Antithrombin Pittsburgh: an alpha1-antitrypsin variant causing hemorrhagic disease. 41 31

1. Activated factor XIII is the enzyme that covalently cross-links fibrin monomers into fibrin polymers and results in increased clot strength and resistance of the clot to fibrinolysis. 2. Small amounts (greater than 1% of normal) of factor XIII are necessary for normal in vitro and in vivo activity. 3. Factor XIII deficiency is a rare autosomal recessive illness in which a hemorrhagic diathesis is caused by the virtual absence of the active a subunit of factor XIII. Approximately 100 cases have been described. 4. The disease in homozygotes is characterized by umbilical stump bleeding, a high incidence of fetal wastage, delayed soft tissue hemorrhage, and a high incidence of intracranial bleeding. The heterozygote is asymptomatic. 5. This paper calls attention to the apparent high incidence of oligospermia and small testes seen in homozygote males. Otherwise secondary sex characterics are normal. 6. Because there is no abnormality in thrombin generation and conversion of fibrinogen to fibrin, route coagulation tests (prothrombin time, partial thromboplastin time, thrombin time, etc.) are normal. Platelet function tests are normal. 7. Clots made from recalcified plasma severely deficient in factor XIII are soluble in 5 M urea or 1% monochloroacetic acid. These screening tests are simple and nearly pathognomonic of the illness. 8. More sophisticated and quantitative tests (e.g., dansylcadaverine incorporation) are available for definitive diagnosis and heterozygote detection. 9. Replacement treatment of the illness is simple, effective, and relatively inexpensive. Due to the long half-life of infused factor XIII and the small amounts necessary for normal hemostasis, prophylaxis is feasible and encouraged.
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PMID:Factor XIII. 51 66

Hemorrhagic diathesis was observed in patients with renal insufficiency after carbenicillin at serum levels greater than 300 mug/ml. Normal coagulation factors (F. I, II, V, VII, VIII, X), normal PTT, normal platelet counts, negative ethanol gelation test (fibrin monomers) were found as well as a prolongation of thromboplastin time (Quick), thrombin time, reptilase time and thrombin coagulase time. Platelet function was disturbed. In addition, the plasmatic system was involved: inhibition of fibrinogen-fibrin conversion (Belitser assay) and enhanced antithrombin III activity; in vivo the latter was ascribed to a heparin-like activity. In vitro, abnormal III was seen: however an enhanced antithrombin III activity in vitro was not found with carbenicillin and various penicillin derivatives. This study demonstrates that carbenicillin, in addition to its known effect on platelet function, also disturbs the plasmatic coagulation system. This additional effect of carbenicillin is clinically important since protamin chloride effectively blocks bleeding without interfering with antibacterial activity. Both penicillin and penicillin derivatives have been shown to interfere with hemostasis and to cause clinically manifest hemorrhagic diathesis (Fleming and Fish 1947, Lurie et al. 1970a, b, McClure et al. 1970, Yudis et al. 1972, Demos 1971, Waisbren et al. 1971). Carbenicillin interferes with ADP-, collagen- or thrombin-induced platelet aggregation and with the release reaction both in vivo (McClure et al. 1970, Cazenae et al. 1973) and in vitro (McClure et al. 1970, Cazenave et al. 1973). In addition Lurie and colleagues (1970b) concluded that an inhibition of the conversion of fibrinogen to fibrin is involved although no experimental details were given. Later Brown and colleagues (1974) concluded that carbenicillin at usual dose levels "only affects the platelet component of hemostasis and has little effect on fibrin formation or other phases of coagulation in patients with normal renal function".
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PMID:Bleeding in uremic patients after carbenicillin. 103

A new case of facultative hemorrhagic diathesis is described in a 19 year old female and the coagulatory anomaly examined. The experiments show that the coagulatory disturbance should be ascribed to a defective aggregation of fibrin monomers associated with hypofibrinogenemia. Some of its characteristics are the markedly prolonged thrombin-, reptilase-time as well prolonged prothrombin and partial thromboplastin time. The plasma fibrinogen concentration measured by the immunologic method is reduced. The streptokinase induced digestion of fibrinogen Giessen II plasma occurred at a slower rate than normal plasma with persistence of the large fibrinogen degradation products. Fibrinogen Giessen II appears to be a congenital hypodysfibrinogenemia with abnormality of fibrinogen resulting in delayed coagulation and a retarded fibrinogenolysis rate.
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PMID:[Hypodysfibrinogenemia: fibrinogen giessen II (author's transl)]. 116 25

The essentially indefinite storage life of previously frozen erythrocytes (PFE), combined with the virtual freedom from hepatitis, high 2,3-diphosphoglycerate (2,3-DPG) content, and low level of HL-A antigens, should make its use in open-heart surgery attractive. However, since the suspension medium for PFE is usually saline, the potential exists for creating a hemorrhagic diathesis by accentuating the dilution of plasma procoagulants by the pump prime. To test this possibility, we used PFE exclusively in transfusing a group of 13 open-heart surgery patients; they were given no plasma or platelets. A control group of 12 open-heart surgery patients were transfused with only shelf blood. Determination of prothrombin times (PT), partial thromboplastin times (PTT), platelets, and fibrinogen were done at various intervals. No clinically significant differences between the two groups were seen in any of these parameters at any interval, and there was no significant difference between the groups in amount of chest tube drainage or transfusions in the first 24 hours. It is concluded that most open-heart surgery can be safely performed exclusively with frozen blood.
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PMID:The effect on blood coagulation of the exclusive use of transfusions of frozen red cells during and after cardiopulmonary bypass. 116 42

Between October 1988 and May 1989, four cancer patients treated by broad-spectrum antibiotics developed a hemorrhagic diathesis induced by vitamin k (VK) deficiency. Activated partial thromboplastin time (APTT), prothrombin time (PT), factor II (FII) and protein induced by vitamin k absence of antagonist-II (PIVKA-II) were measured after administration of antibiotics and VK in all 4 patients. All these patients had been receiving intravenous hyperalimentation (IVH) and antibiotics for various infections. But all or them developed hemorrhagic diathesis within five days after the initiation of broad-spectrum cephem antibiotics (LMOX or CMNX). The abnormalities were 1) marked decrease of F II (6-18%), 2) prolongation of APTT (58.4-200 seconds), 3) prolongation of PT (7-21%), 4) marked increase of PIVKA-II (17-80 less than AU/ml). After being treated by intravenous administration of VK, hemorrhagic diathesis and abnormalities of coagulation tests except for PIVKA-II were corrected quickly in three evaluated patients. The measurement of PIVKA-II seemed to be useful to diagnose the hemorrhagic diathesis caused by VK deficiency in the patients during administration of antibiotics.
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PMID:[Acquired coagulopathy caused by administration of parenteral broad-spectrum antibiotics]. 190 Nov 16

A 61-years old woman who had been healthy without history of abnormal bleeding, developed widely spread ecchymosis and intramuscular bleeding in March, 1987. She was hospitalized for this hemorrhagic diathesis in May, 1987 and the following laboratory data were revealed: activated partial thromboplastin time (APTT), 76.7 seconds; factor VIII procoagulant activity, 2%; factor VIII inhibitor, 27 Bethesda units/ml. The inhibitor was an immunoglobulin of IgG type. Her clinical data of the blood were normal, and tests for antibodies, such as RA test, LE test and thyroid test were negative. Physical examination revealed ecchymosis over her right arm and swelling and pain in the right arm. She was first treated with a large dose of factor VIII concentrates, but the effect was insufficient. Then prednisolone was given, which resulted in decreasing of the inhibitor and improvement of the coagulation profiles. This treatment appeared to offer effective control on severe hemorrhage in patients with factor VIII inhibitors.
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PMID:[Remission of a non-haemophilic patient with acquired factor VIII inhibitor treated with infusion of factor VIII and corticosteroid]. 250 78

Congenital deficiency of high molecular weight kininogen (HMWK), first reported by Saito et al. in 1975, is a rare disorder with homozygous defect of HMWK and only 14 cases have been reported so far. Prolonged partial thromboplastin time and depressed intrinsic fibrinolytic activity in plasma interacted with negatively charged surface were characterized in these patients without any bleeding problem. On the other hand, patients associated with hemorrhagic disorder, such as haemophiliacs, showed a markedly prolonged partial thromboplastin time but normal intrinsic fibrinolytic activity. These results give us a important suggestion on our understanding of why haemophiliacs bleed and why HMWK deficient patients do not. In immunological studies with specific antisera directed against human plasma HMWK and prekallikrein, HMWK in present in normal plasma in free form and a complexed form with prekallikrein, and whole prekallikrein in present in a complexed form with HMWK. This result is associated with diminished level of prekallikrein in plasma of patients with congenital HMWK deficiency. According to above-mentioned our experimental results and some reports, it is speculated that the deficiency of HMWK with normal level of prekallikrein is acquired.
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PMID:[Coagulation and fibrinolysis associated with contact phase in plasma with high molecular weight kininogen deficiency]. 259 36

A 58-year-old black woman with IgD multiple myeloma developed a hemorrhagic diathesis within 48 hours after receiving mithramycin (20 micrograms/kg/day) for therapy of hypercalcemia. Her coagulation studies were characterized by prolonged prothrombin, partial thromboplastin, thrombin, and reptilase clotting times. Her plasma and partially purified fibrinogen were inhibitory to the clotting of normal plasma and fibrinogen. The patient's isolated fibrinogen showed a normal rate of fibrinopeptide release, but her fibrin monomer aggregation was markedly abnormal. These studies document the development of a dysfibrinogenemia secondary to mithramycin toxicity.
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PMID:Acquired dysfibrinogenemia secondary to mithramycin toxicity. 294 Aug 61

Severe hemorrhagic diathesis caused by hemophilia A (factor VIII:C deficiency) was diagnosed in 2 related Quarter Horse colts. Clinical signs consisted of dyspnea and dysphagia attributable to cranial cervical hematoma in one colt and to intra-abdominal hemorrhage resulting in death of the second colt. Factor VIII:C deficiency, a defect of the intrinsic coagulation pathway, is suggested by results of coagulation studies--prolonged activated partial thromboplastin time, normal prothrombin time, and normal primary bleeding time. The diagnosis was confirmed by results of factor VIII:C assays. Hemophilia A is inherited as an X chromosome-linked trait.
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PMID:Hemophilia A in two related quarter horse colts. 313 24


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