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)

Glanzmann's thrombasthenia, known also as Glanzmann's disease, is an autosomally inherited hemorrhagic disease with unique abnormalities of platelet functions. Authors present a large family in which Glanzmann's disease was diagnosed in the father and two sons. An analysis of platelet membranes enabled diagnosis of Glanzmann's thrombasthenia type II. A decrease in clot contractibility, fibrinogen binding to blood platelets, and decreased glycoprotein IIb and IIIa levels with marked impairment of GP IIb and IIIa complexes formation were characteristic for affected family members. One daughter died 8 days after birth with the symptoms of hemorrhagic diathesis. Mother and remaining three sons are healthy without the signs of Glanzmann's disease.
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PMID:[Familial occurrence of Glanzmann thrombasthenia]. 143 2

Vitamin K deficient hemorrhagic diathesis is well known as a cause of infantile intracranial hemorrhage. Its occurrence, however, as a post-surgical complication is rare and has never been reported previously. Two cases are presented here which illustrate the existence of such a hazard. Case 1. A 73-year-old woman admitted with subarachnoid hemorrhage (WFNS IV) underwent microsurgical exploration of a left internal carotid aneurysm, and neck clipping of the aneurysm was performed. She had an uneventful postoperative course, but her neurological condition deteriorated suddenly on the fifth postoperative day. CT scan revealed a large epidural hematoma. Case 2. A 6-year-old boy was admitted due to the dysfunction of a ventriculo-peritoneal shunt system that had previously been placed for hydrocephalus. This dysfunction was thought to be caused by meningitis. Twelve days after ventricular drainage and antibiotic therapy, sudden intraventricular hemorrhage occurred. In both cases, PT and APTT were markedly prolonged, FDP slightly increased and fibrinogen slightly decreased. SFMC was positive in case 2. After the administration of vitamin K, PT and APTT were immediately normalized. Recent reports emphasize the adverse effect of antibiotics that leads to vitamin K deficient hemorrhagic diathesis, especially, in patients in a cachectic state. In these two cases, such a cachectic condition was not observed. We presume that the cause of vitamin K deficiency would be, along with the administration of antibiotics, a preliminary condition of disseminated intravascular coagulation which is encountered in some neurological disorders including subarachnoid hemorrhage. We conclude that attention should be paid for these pitfalls in perioperative neurosurgical care.
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PMID:[Postoperative intracranial hemorrhage due to vitamin K deficiency: report of two cases]. 173 30

After a bite by the aglyphous red-necked keelback snake Rhabdophis subminiatus a complete defibrinogenation syndrome with severe hemorrhagic diathesis developed in a 25-year-old man. In vitro studies showed that the venom gland extract of the snake contains a very active prothrombin (Factor II) activator. The thrombin generated is inhibited neither by antithrombin III nor the antithrombin-III-heparin complex. The venom gland extract stimulated also the tissue plasminogen activator; however, it did not cause direct activation of plasminogen, protein C, Factor X or direct degradation of fibrinogen.
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PMID:Hemostatic changes due to the venom gland extract of the red-necked keelback snake (Rhabdophis subminiatus). 180 26

Hemorrhagic diathesis developed 4 hr after a bite by one fang of a two-month-old specimen of Bothrops asper. Severe allergy to horse serum contraindicated the use of horse antivenom, and a substitution therapy was started 20 hr after the bite. During the following 4 days the patient was treated with infusions of 8 g human fibrinogen, 2500 U of cryoprecipitate, 1000 ml of human plasma and vitamin K in several portions. By means of plasmapheresis 1800 ml of plasma was exchanged. Until plasmapheresis on the third day the treatment resulted in short remission and diminution of the spontaneous bleeding, which ceased on the 5th day. Coagulation tests relevant to disseminated intravascular coagulation and consumption coagulopathy were performed for 12 days. Fibrinogen levels started to rise on the 8th day and normalized 12 days after the bite. Analysis of the venoms from juvenile and adult Bothrops asper snakes revealed that the former has a strong prothrombin-converting activity, the latter contained mainly a thrombin-like, fibrinogen-converting enzyme.
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PMID:Non-specific therapy of a hemorrhagic diathesis after a bite by a young Bothrops asper (barba amarilla): a case report. 208 42

Three kinds of anticoagulant therapy for obstetrical DIC were studied. 1. Antithrombin-III (AT) or gabexate mesilate for acute DIC, mainly for abruptio placentae. 2. Heparin or heparin-AT combination therapy for toxemia pregnancy. 3. Low molecular weight heparin (LMWH) for fetus of intrauterine growth retardation (IUGR). The results obtained were as follows, 1. a) Platelet count, and fibrinogen were significantly increased in AT therapy group compared with gabexate mesilate group. b) In clinical manifestation, renal failure and hemorrhagic diathesis were improved especially in AT group. 2. In heparin-AT group, high systolic blood pressure was improved during administration of AT, the high level of thrombin antithrombin complex was also found in these period. 3. a) The improvement of the gain of estimated fetal body weight was found after administration of LMWH. b) Redistribution of blood flow in one case of severe IUGR was observed during administration of LMWH.
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PMID:[Anticoagulant therapy in obstetrical disorders]. 217 Jul 3

Bleeding defects are of great interest in pediatrics since the prevalence of congenital forms and the early appearance of acquired ones. The pathology itself and the therapy indeed can often interfere with the growing-up patients. Bleeding defects have been identified with an heterogeneous group of clinical disease that differs from one another in etiology, pathogenesis, epidemiology and incidence in population. Bleeding diathesis is the common symptom: bleeding tendency may be mild, moderate or severe, localized or generalized, cutaneous or mucosal, superficial or deep. Bleeding disorders may be classified as a) defects in the primary haemostasis, which include quantitative and qualitative abnormalities of platelets and vascular disorders and b) defects in secondary haemostasis, which include intravascular disorders (blood coagulation). Careful history and clinical examination are essential in diagnosis of bleeding disorders. History of patient should be taken a) to differentiate acquired from congenital disease and to know the way of hereditary transmission (family history); b) to know exactly the disease's start and the mutual relation with former or accompanying disease; c) mutual relation with drugs token. Subsequently a careful physical examination should be done. A specific hemorrhagic diathesis has been seen with a deficiency of primary or secondary haemostasis. A deficient or late haemostatic plug in small vessels can cause superficial, interstitial bleeding that may be intracutaneous or intramucosal and is called purpura. In coagulation factor deficiency the haemostatic plug cannot be consolidated by fibrin: spontaneous hematomas, hemarthrosis and ecchymoses often occurs. The initial laboratory work up for screening patients with bleeding disorders should include first step tests to differentiate bleeding disorders for bone-marrow malignancies; from virus infections carrying screening of major viruses and from hepatic diseases. Second step laboratory examination includes a) platelet count or estimation of platelet number on blood smear; b) bleeding time to test small vessel integrity and platelet function; c) aPTT, PT, AP to measure clotting activity; d) fibrinogen determination. With this battery of screening test it is usually possible to determine the general area of the defect (abnormalities of platelets number or function or congenital defect of one or more clotting factors activity). Acute idiopathic thrombocytopenic purpura is the most common bleeding disorders in childhood. Usually no therapy may be required no matter platelet count. Patients with a significant hemorrhagic tendency are treated either with prednisone (2 mg/kg orally in divided daily doses) for a period of 2 weeks or with a 5 days course of special polyvalent intact immunoglobulin (400 mg/kg/die) for intravenous use.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Children with hemorrhagic diathesis: correct diagnostic and therapeutic approach]. 219 46

Partial resection of the liver is the only curative treatment for patients with hepatocellular carcinoma associated with severe cirrhosis of the liver. Surgical hemostasis on the cut surface of the cirrhotic liver appears very difficult because of the resultant deep cavity and the marked hemorrhagic diathesis. However, by using the microcrystalline collagen powder and the fibrinogen tissue adhesive, complete hemostasis and prevention of postoperative bleeding can be obtained, with minimal blood loss and hepatic ischemia.
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PMID:Use of microcrystalline collagen powder and fibrinogen tissue adhesive for hemostasis and prevention of rebleeding in patients with hepatocellular carcinoma associated with cirrhosis of the liver. 246 32

In an abnormal fibrinogen with severely impaired polymerization of fibrin monomers, we identified a methionine-to-threonine substitution at position 310 of the gamma chain. Furthermore, asparagine at position 308 was found to be N-glycosylated due to a newly formed consensus sequence, asparagine(308)-glycine(309)-threonine(310). The two structural defects in the mutant gamma chain may well perturb the conformation required for fibrin monomer polymerization that is specifically assigned to the D domain of fibrinogen. This alteration also seems to affect the intermolecular gamma chain cross-linking of fibrin and fibrinogen, although the amine acceptor gamma glutamine-398 was found to function normally. These functional abnormalities may well be related to posttraumatic hemorrhage as observed in a 33-yr-old man with moderate hemorrhagic diathesis related to injuries since his early adolescence. The structure of the extra carbohydrate moiety attached to asparagine-308 was found to be identical with those derived from the normal B beta and gamma chains as evidenced by HPLC.
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PMID:A gamma methionine-310 to threonine substitution and consequent N-glycosylation at gamma asparagine-308 identified in a congenital dysfibrinogenemia associated with posttraumatic bleeding, fibrinogen Asahi. 249 44

A dysfibrinogenemia (fibrinogen Sevilla) was detected in a 64-yr-old woman with no previous history of hemorrhagic diathesis or thrombosis. Thrombin and reptilase times were prolonged. The aggregation of fibrin monomers showed a prolonged latency time with a defective slope although fibrinopeptide release and clot stabilization were found to be normal. Plasmin proteolysis was abnormal with a much slower plasmic degradation in patient's purified fibrinogen. By chromatofocussing the patient's fibrinogen showed an abnormality in pattern elution with a second peak eluting at a pH slightly more basic than the normal one (pH 5.5). Likewise, the isoelectrofocussing of purified non-reduced patient's fibrinogen in agarose gel showed an abnormal distribution in its focussed bands, especially in a group which focussed in a pI-interval between 5.20-5.85. By two-dimensional electrophoresis we did not find any abnormality in the fibrinogen-reduced chains. These results could indicate that the abnormal monomer aggregation, as well as the defective plasmin lysis, could be due to conformational aspects of fibrinogen rather than to structural defects.
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PMID:Fibrinogen Sevilla, a congenital dysfibrinogenemia characterized by an abnormal monomer aggregation and a defective plasmin lysis. 271 97

A female patient what M3 was seen to incur marked hemorrhagic diathesis during therapy. Heparin infusion was performed to inhibit disseminated intravascular coagulation (DIC). However, oozing from the puncture site of the r-subclavian vein was observed. Her data on coagulation were as follows: AT-3 greater than 100%, fibrinogen 69.0 mg, XIII less than 40%, FPA 2.9 ng/ml and FDP-D dimer 256 ng/ml. The alpha-2-plasmin inhibitor (alpha 2-PI) was 44% and FDP 160 microgram/ml. These results suggested DIC with activated fibrinolysis. Thus, epsilon aminocaproic acid in conjunction with heparin, fibrinogen, and concentrated XIII was administered. The abnormal bleeding improved with an increase of alpha 2-PI and XIII. This clinical response indicated that an activated fibrinolysis and a decreased XIII might have been responsible for provoking the bleeding of the patient.
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PMID:[Antiplasmin drugs and factor XIII concentrates in the treatment of a patient with acute promyelocytic leukemia (M3)]. 281 Jul 83


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