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)

Two patients with hepatorenal syndrome were treated by insertion of a peritoneovenous shunt. The renal deficit was corrected rapidly in both cases. A 62-year-old woman with a slow onset syndrome with urine output of 100 to 150 ml/day and urinary sodium excretion of 1 mEq/day responded with large volume urinary output and sodium excretion. She is alive with minimal ascites 18 months after shunt. A 53-year-old man with severe nutritional cirrhosis, alcoholic hepatitis, and eventual massive necrosis was treated for bleeding esophageal varices by portacaval shunt. Postoperative massive ascites progressed to acute hepatorenal syndrome. Insertion of a peritoneovenous shunt reversed the renal deficit. HE eventually exsanguinated due to a hemorrhagic diathesis caused by massive hepatic necrosis.
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PMID:Hepatorenal syndrome: reversal by peritoneovenous shunt. 88

alpha 2-Antiplasmin (alpha 2-AP) is a major fibrinolysis inhibitor, whose complete, congenital absence has been found to be associated with a distinct hemorrhagic diathesis. We studied a 15-yr-old male with a hemorrhagic diathesis after trauma from early childhood on. This bleeding tendency was associated with a minimal alpha 2-AP level recorded functionally in the immediate plasmin inhibition test: less than or equal to 4% of normal. However, a normal plasma concentration of alpha 2-AP antigen (83%) was found. His sister (5 yr old) showed similar results (2 and 92%). In their family, eight heterozygotes could be identified by half-normal activity results and normal antigen concentrations. The inheritance pattern is autosomal recessive. On analysis, the alpha 2-AP of the propositus was homogeneous in all respects tested, suggesting a homozygous defect. We designated the abnormal alpha 2-AP as alpha 2-AP Enschede. alpha 2-AP Enschede showed the following characteristics: (a) complete immunological identity with normal alpha 2-AP; (b) normal molecular weight (sodium dodecyl sulfate-polyacrylamide gel electrophoresis); (c) normal alpha-electrophoretic mobility; (d) presence in plasma of both molecular forms excluding an excessive conversion to the less reactive non-plasminogen-binding form; (e) quantitatively normal binding to lys-plasminogen and to immobilized plasminogen kringle 1-3; and (f) normal Factor XIII-mediated binding to fibrin. Functional abnormalities were found in: (i) no inhibition of amidolytic activities of plasmin and trypsin, even on prolonged incubation; (ii) no formation of plasmin-antiplasmin complexes in plasma with plasmin added in excess; and (iii) no inhibition of fibrinolysis by fibrin-bound alpha 2-AP. In the heterozygotes, the presence of abnormal alpha 2-AP did not interfere with several functions of the residual normal alpha 2-AP. One-dimensional peptide mapping showed an abnormal pattern of papain digestion. We conclude that in this family, abnormal antiplasmin molecules, defective in plasmin inhibition but with normal plasminogen-binding properties, have been inherited. The residual plasminogen-binding properties do not protect against a hemorrhagic diathesis.
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PMID:alpha 2-Antiplasmin Enschede: dysfunctional alpha 2-antiplasmin molecule associated with an autosomal recessive hemorrhagic disorder. 244 79

Plasma fibrinogens of sublethally irradiated rabbits (8 Gy X-ray irradiation) were isolated by affinity chromatography on fibrin monomer Sepharose and characterised by polyacrylamide gel electrophoresis with sodium dodecylsulfate before and after reduction with 2-mercaptoethanol. Only a small increase of fibrinogen degradation was mainly observed in early phases after irradiation. Fibrin monomer complexes were precipitated together with fibrinogen by 2,5 mol beta-alanine/l. The content of monomer complexes in the precipitate was estimated using Sepharose 6B gel chromatography. Evidence for an increased formation of monomers in the blood could not be obtained. Therefore, proteolytic changes of fibrinogen do not play a role for inducing a hemorrhagic disorder after sublethal X-ray irradiation in rabbits.
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PMID:[Qualitative changes in plasma fibrinogen in irradiated rabbits]. 615 69

205 cases of clinical poisoning with the mushroom Amanita phalloides (death cap) in the period 1971 to 1980 have been studied retrospectively. The lethality was 22.4%. Age and latency between the ingestion of the mushrooms and the first clinical symptoms were of prognostic significance. The death rate was 51.3% in children below 10 years of age but only 16.5% in patients older than 10 years. The average latency period was 10.3 hours for the fatal cases and 12.6 hours for the surviving patients. Country, year, sex and time of hospitalization did not influence lethality. Prognostic relevance could also be attributed to the thromboplastin time (Quick). 84% of the patients with values below 10% died, while all patients with minimal values of more than 40% survived. The correlation with the outcome was weaker for the serum transaminases and nil for creatinine. The patients underwent on the average 8 therapeutic measures, but up to 20 therapies could be administered to the same patient. Eight of the 30 recorded treatments involved general support, 7 toxin elimination and the remaining 14 could be classified as pharmacotherapy. With the aid of multiple regression analysis taking into account age, latency period and the effects of all the other measures, penicillin and hyperbaric oxygenation were found to contribute independently to a higher survival rate. As compared to penicillin, the combination of penicillin with silybin was associated with still further increased survival. On the other hand, several measures, including exchange transfusion, thiocytic acid, sulfamethoxazole, plasma expanders, haemodialysis, treatment of the hemorrhagic diathesis and THAM/sodium bicarbonate were administered more often to patients who did not survive. For the remaining 20 therapeutic measures our analysis revealed neither a positive nor a negative correlation with the clinical outcome.
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PMID:[Clinical death-cap (Amanita phalloides) poisoning: prognostic factors and therapeutic measures. Analysis of 205 cases]. 629 Nov 47

Heparin-dependent intravascular coagulation is a widely recognized syndrome in which heparin acts as a hapten for an antiplatelet antibody and causes accelerated intravascular thrombosis and thrombocytopenia that may culminate in organ loss, hemorrhagic diathesis, and even death. Diagnosis is made in vitro by observing heparin-stimulated aggregation of normal platelets suspended in the patient's platelet-poor plasma. Treatment consists of cessation of heparin and use of antiaggregating agents with or without warfarin sodium. Management of patients with prior heparin-dependent intravascular coagulation who require cardiopulmonary bypass has not been reported. We now have successfully managed three such patients and herein present guidelines for management. Each patient was undergoing heparin therapy and manifested hallmark heparin tachyphylaxis, thrombocytopenia, and increased thrombotic symptoms (further venous thrombosis after cardiac catheterization in two cases and exacerbation of unstable angina in the other). In vitro aggregation studies were abnormal. Heparin was stopped, and antiaggregative therapy was begun with good response in each instance. In vitro studies were done serially until the antiplatelet antibody reaction had vanished (usually 4 to 8 weeks), and coronary revascularization was then conducted with full heparinization. Further heparin exposure postoperatively was avoided. There was no perioperative evidence of intravascular thrombosis or bleeding diathesis, and in vitro heparin-dependent aggregation did not recur. We conclude that patients with previously documented heparin-dependent intravascular coagulation can safely sustain the massive heparin rechallenge of cardiopulmonary bypass, provided that in vitro aggregation has ceased and rechallenge therapy is not prolonged.
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PMID:Cardiopulmonary bypass for patients with previously documented heparin-induced platelet aggregation. 671 46

A 68-year-old man who had abdominal aortic aneurysm (AAA) associated with idiopathic thrombocytopenic purpura (ITP) and consumption coagulopathy was successfully operated upon. The count of platelet was improved from 1.4 x 10(4)/microL to 8.0 x 10(4)/microL by the preoperative treatment with gamma-globulin (400mg/kg/day for 5 days) and heparin sodium (10000-15000 unit/day). Abdominal aortic aneurysm was replaced with a Y-shaped knitted Dacron graft, and splenectomy was performed for ITP. No severe hemorrhagic diathesis was encountered during and after the operation. This is the first report of surgical treatment of AAA associated with ITP and consumption coagulopathy.
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PMID:[Surgical treatment of abdominal aortic aneurysm associated with idiopathic thrombocytopenic purpura and consumption coagulopathy: a case report]. 830 72

Vitamin K is needed to synthesize coagulation factors II (prothrombin), VII, IX, and X through the carboxylation of glutamic acid in vitamin K-dependent proteins which results in the creation of effective calcium binding sites which, in turn, facilitates the coagulation process. Vitamin K exists as naturally occurring vitamin K-I (phylloquinone) in green leafy vegetables and vegetable oils, vitamin K-II (menaquinone) as produced in the gut by bacteroides fragilis and E. coli, and synthetic vitamin K-III (menadoine sodium bisulfite) which is water-soluble and capable of producing serious jaundice in newborns, especially those with instability of glutathione and deficiency of G6PD. Humans require about 5 mcg of vitamin K daily. Since it is indigenously produced in the gut by bacterial flora, dietary deficiency of vitamin K in healthy subjects is rare. Vitamin K is usually the first vitamin given at birth. Newborn babies, however, absorb only approximately 30% of ingested vitamin K, compared to 50-70% in adults. Hemorrhagic disease is a manifestation of vitamin K deficiency in newborn infants. Hemorrhagic disease of the newborn (HDN), early HDN, classical HDN, and late HDN are discussed. The American Academy of Pediatrics recommended in 1961 that all healthy term newborn babies receive 0.5-1.0 mg of vitamin K-I intramuscularly at birth. However, while the authors have not followed those recommendations in their neonatal unit for 15 years, they have experienced only a 0.1% incidence of classical HDN. High-risk newborns at the unit are routinely given the recommended dose of K-I at birth.
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PMID:Vitamin K during infancy: current status and recommendations. 949 99

Hemorrhagic cystitis (HC) is the syndrome of hematuria combined with symptoms of lower urinary tract irritation in the absence of bacterial infection or generalized hemorrhagic diathesis. HC often occurs as a difficult complication after autologous as well as allogeneic hematopoietic cell transplantation (HCT). It may be secondary to pretransplant preparative regimen (chemotherapy and/or radiation therapy) or viral infection by adenovirus, JC and BK viruses. The most effective treatment for HC has not been established yet. We report a case of a 17-year-old male with common acute lymphoblastic leukemia (cALL) in second CR, who was treated with high-dose chemotherapy (BuCy conditioning regimen) followed by autologous bone marrow transplantation (ABMT), complicated by hemorrhagic cystitis on day 0 (several hours after infusion of transplant material). The immediate use of increased dose of 2-mercaptoethane sulfonate sodium (mesna), bladder irrigation and intensive hydration with forced diuresis resulted in resolution of macroscopic hematuria on day +3 after the transplant and urinary tract recovery with normalization of urine analysis parameters on day +7.
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PMID:[Hemorrhagic cystitis related to the high-dose conditioning therapy in a bone marrow recipient]. 1145 Jan 58

Spontaneous nongastrointestinal bleeding in patients treated with nonsteroidal anti-inflammatory drugs is an extremely rare occurrence. Herein, the case of a 60-year-old woman with severe bilateral hip osteoarthritis treated with diclofenac sodium, who presented with manifestations of a spontaneous hematoma of the right thigh, is described. There was no history of trauma or family history of bleeding disorder. Thorough investigation excluded a hemorrhagic disorder. The patient was treated conservatively with success and was advised to discontinue diclofenac.
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PMID:Spontaneous thigh hematoma associated with diclofenac. 1904 55

Ethylenediaminetetraacetic acid (EDTA)-dependent pseudothrombocytopenia (PTCP) is the phenomenon of a spurious low platelet count due to antiplatelet antibodies that cause platelet clumping in blood anticoagulated with EDTA. The aggregation of platelets in EDTA-dependent PTCP is usually prevented by other anticoagulants, such as sodium citrate or heparin. EDTA-dependent PTCP has never been associated with hemorrhagic diathesis or platelet dysfunction. In this article, a 10-year-old boy with EDTA- and heparin-dependent PTCP is presented because of rare presentation. We report that EDTA and heparin can induce platelet clumping, and thus spuriously low platelet counts. However, aggregation of platelets was not detected in blood samples with sodium citrate, and platelet count was normal.
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PMID:EDTA-dependent pseudothrombocytopenia in a child. 2053 50


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