1/56. Bleeding from self-administration of phenindione: a detailed case study.A young woman presented with a 2 year history of a severe bleeding disorder and marked deficiencies in all four vitamin-K-dependent factors. Metabolic studies with tracer doses of tritium-labelled vitamin K1 suggested that the patient might be taking an oral anticoagulant; and subsequently her plasma was found to contain a substance identical to phenindione in its spectrophotometric and chromatographic properties. The half-disappearance times of factors II, IX, X were measured after the administration of a concentrate of these factors and were found to conform with published figures. The concentrate controlled the patient's excessive bruising and prolonged skin and gingival bleeding. It would therefore seem that factor VII may not be essential in reversal of the bleeding disorder induced by anticoagulant overdose.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/56. Spontaneous dural sinus thrombosis in children.The clinical course of spontaneous dural sinus thrombosis in children varies from indolent to fulminant. Although many different etiologies for the development of dural sinus thrombosis have been described, a full recovery can be anticipated in most children following rehydration and the administration of systemic antibiotics. steroids, systemic anticoagulation and intrasinus thrombolysis may be beneficial in selected patients, although the efficacy of these therapies has not been established prospectively in children. We reviewed 12 pediatric patients with spontaneous dural sinus thrombosis (1978-1998) to determine the etiology, clinical course and best treatment options. In the absence of a hypercoagulable state, pediatric patients generally recover well with rehydration and antibiotics and do not require anticoagulation.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
3/56. Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas.Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation. diagnosis was hampered by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of antithrombin III improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date. CONCLUSION: Occult visceral haemangiomatosis without visible cutaneous haemangiomas should be included in the differential diagnosis of thrombocytopenia, anaemia and consumption coagulopathy. Antithrombin III treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit syndrome.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
4/56. Reduced nitric oxide production by L-arginine deficiency in lysinuric protein intolerance exacerbates intravascular coagulation.Lysinuric protein intolerance (LPI) results in low serum L-arginine, hyperammonemia, mental retardation, thrombocytopenia, and an increased frequency of bowel movements. Our objective was to evaluate the effects of low serum L-arginine, the essential substrate for reactions catalyzed by nitric oxide synthetase (NOS), on the serum nitric oxide (NO) level and coagulation activity in a patient with LPI. A 37-year-old Japanese man who presented with abdominal pain and subnormal fasting levels of serum L-arginine and L-lysine was found to have LPI. The result of oral administration of diamino acids was an increased in urine and a decrease in serum, thus confirming the diagnosis. A decrease in the platelet count and an increase in the plasma levels of thrombin-antithrombin III complex (TAT) and fibrin degradation products (FDPs) indicated the presence of subclinical intravascular coagulation. serum levels of NO derivatives and L-arginine were determined after intravenous administration of L-arginine. The effects of intravenous L-arginine or transdermal nitroglycerin on the plasma level of TAT were also investigated. serum levels of NO derivatives were significantly reduced in the LPI patient versus the healthy control group (n = 5). Intravenous administration of L-arginine increased the serum level of NO derivatives and the platelet count and reduced plasma TAT and FDP levels. The plasma level of TAT was also reduced by transdermal nitroglycerin. A decrease in the serum level of L-arginine in patients with LPI appears to result in a decrease in NO production. The improvement in plasma TAT levels produced by administration of intravenous L-arginine or transdermal nitroglycerin suggests that intravascular coagulation is exacerbated by the decrease of NO production in patients with LPI.- - - - - - - - - - ranking = 0.8keywords = administration (Clic here for more details about this article) |
5/56. peritoneal dialysis is the therapy of choice for end-stage renal disease patients with hereditary clotting disorders.Chronic renal failure is an unusual complication of hereditary clotting disorders (HCDs), but this situation could change in the near future. The modality of dialysis for end-stage renal disease (ESRD) in patients with an HCD is a difficult choice. Hemodialysis (HD) may be considered, but intensive treatment with coagulation factors is required for vascular access execution and for each HD procedure. peritoneal dialysis (PD) has been infrequently proposed. However, PD requires coagulation replacement therapy only during peritoneal catheter placement. The aim of this paper is to describe our experience of three patients with ESRD and HCD, successfully treated with chronic PD in the medium term. Case 1 was a 58-year-old man with moderate hemophilia a, type 2 diabetes mellitus, and hepatitis c virus (HCV) infection. His ESRD was secondary to glomerulonephritis. A double-cuff peritoneal catheter was surgically placed with pre-emptive factor viii administration. He began treatment with continuous ambulatory peritoneal dialysis (CAPD). An inguinal hernia was repaired without complications. After eleven months of follow-up, no hemorrhage episodes have been observed and clinical outcome is optimal. Case 2 was a 46-year-old man with severe hemophilia A, type 2 diabetes mellitus, and HCV and human immunodeficiency virus (hiv) infections. He developed a diabetic nephropathy that required renal replacement therapy. A permanent silicone catheter was inserted in the left internal jugular vein, and the patient started HD treatment. Later on, PD therapy was proposed. A peritoneal catheter was implanted with simultaneous factor viii infusion. Minimal bleeding was observed at the subcutaneous tunnel over the following 48 hours. The patient started PD treatment without complications, and two months later, remaining asymptomatic, transferred to another center. Case 3 was a 41-year-old woman diagnosed with von Willebrand disease type 2A, HCV infection, and polycystic kidney disease, who presented with ESRD. An internal arteriovenous fistula was performed under coagulation factor cover. During a fistulography, and despite coagulation factor substitutive treatment, the patient showed an important hematoma. Afterwards, PD was considered. A peritoneal catheter was implanted under coagulation factor cover. The postoperative course was uncomplicated, and the patient started CAPD treatment. During follow up, she suffered two hemoperitoneum episodes that were resolved with cold dialysate. After nine months, she uneventfully continued on PD. In conclusion, PD is the therapy of choice for patients with hereditary clotting disorders and ESRD requiring dialysis. peritoneal dialysis therapy avoids many of the complications related to HD therapy.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
6/56. Successful anticoagulation with hirudin in a patient with mesenteric venous thrombosis and multiple coagulation abnormalities.A case of multiple thrombotic diatheses discovered in the setting of mesenteric venous infarction is discussed. The patient had deficiencies of protein c, protein s, antithrombin III; was heterozygous for factor V Leiden; and had polycythemia vera. Adequate anticoagulation could not be established with heparin administration and hirudin was used. The diagnosis of mesenteric venous infarction, thrombotic tendency of multiple coagulation diatheses, and use of hirudin are discussed.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
7/56. Gingival bleeding, epistaxis and haematoma three days after gastroenteritis: the haemorrhagic lupus anticoagulant syndrome.A 3 year and 9 month-old girl presented with gingival bleeding, epistaxis, and multiple haematomas 3 days after an acute episode of gastroenteritis. prothrombin time and activated partial thromboplastin time were prolonged with reduced clotting activity of factor II (< 10%), VIII (<1%), IX (3%), XII (10%) and evidence of a high titre inhibitor. Prothrombin (factor II) level was below the detection limit, both in a functional and immunological assay. It did not increase after administration of vitamin k or fresh frozen plasma. Further studies revealed presence of a strong lupus anticoagulant and a specific IgG antibody against prothrombin. factor viii antigen levels also were reduced (31%), but to a lesser extent than functionally determined factor viii (<1%). blood coagulation normalised following clinical recovery 6 weeks after admission. The pathophysiology of this acquired inhibitor phenomenon (accelerated clearance of complexes of clotting factors and phospholipids) is discussed. CONCLUSION: The haemorrhagic lupus anticoagulant syndrome (acquired hypoprothrombinaemia lupus anticoagulant syndrome) is a rare presentation of acquired bleeding diathesis in childhood. Since most cases in post-infectious children are asymptomatic, it might be underdiagnosed. In children with newly appearing bleeding symptoms or unclear prolonged prothrombin time or activated partial thromboplastin time, one has to consider this syndrome which could lead to relevant bleeding.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
8/56. A case of forearm amputation after ovarian stimulation for in vitro fertilization-embryo transfer.OBJECTIVE: To report a case of forearm amputation after ovarian stimulation for IVF-ET. DESIGN: Case report. SETTING: A university hospital. PATIENT(S): A 41-year-old woman, who had coagulation disorder as a result of an ovarian hyperstimulation syndrome (OHSS) for IVF-ET. INTERVENTION(S): Retrospective evaluation of angiographic studies and surgical treatment. MAIN OUTCOME MEASURE(S): Medical follow-up after forearm amputation due to OHSS. RESULT(S): The patient underwent many cycles of IVF-ET with administration of purified FSH (75 IU 10 times per day, for 12 days) and chorionic gonadotropin (5,000 IU). The patient had a coagulation disorder as a result of OHSS, with thrombosis of the axillary vein, recurring after thromboarterectomy and leading to the paradoxical result of the amputation of an arm. CONCLUSION(S): An ethical evaluation of this case is mandatory, since the desire for pregnancy, the role of medical science, health, and human life itself are all factors involved.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
9/56. Anticoagulation management in a patient with an acquired antithrombin iii deficiency.We report a case of heparin resistance and its management during cardiopulmonary bypass (CPB). A 63-year-old, 96 Kg female with a posterior myocardial infarction (MI) with previous deep venous thrombosis was treated with intravenous (IV) heparin infusion for 7 days before myocardial revascularization surgery. The patient required 1200 IU/Kg of beef lung heparin to extend the activated clotting time (ACT) in order to initiate CPB. A total of 1562 IU/Kg of heparin was administered throughout the procedure. This acquired heparin resistance was attributed to an antithrombin (AT III) deficiency, and was treated with fresh frozen plasma (FFP) to restore adequate anticoagulation. The patient's heparinized ACTs ranged between 368 seconds and 387 seconds before FFP administration as opposed to 626 seconds to 1329 seconds after treatment with FFP and additional heparin once on CBP. The patient experienced an uneventful postoperative course. Future treatment with AT III concentrate rather than FFP may reduce heparin requirements that will, in turn, reduce protamine reversal dose, postoperative bleeding attributable to heparin rebound, and its associated complications.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
10/56. Investigation of coagulopathy in three cases of tiger snake (Notechis ater occidentalis) envenomation.AIMS: To investigate the severe coagulopathy (fibrinogen < 1.0 g/l) that occurs in some cases of tiger snake envenomation. Specifically, to determine the concentration of clotting factors on presentation and during resolution of the coagulopathy. methods: Clotting factors II, V, VII, VIII, IX, XI were assayed on all coagulation samples received from three successive cases of severe tiger snake envenomations. Assays were performed at dilutions of 1:5 and 1:10 using an MLA 1600c automated coagulation analyser and Dade Behring factor-deficient plasmas, controls and standards. D-dimers were assayed using Agen Dimertest latex kit, fibrinogen was determined using the Clauss method and platelet counts were performed using Abbott Cell-Dyn 4000 analysers. RESULTS: The activity of the coagulation factors of the intrinsic pathway was reduced (factor viii < 44% in all cases, factor ix < 33%, factor xi < 52% in Cases 1 and 2) but the results for the two dilutions were not parallel. In one case, factor viii and factor ix activity increased 2-fold prior to the administration of blood products. Treatment with blood products corrected the coagulation indices in two out of the three cases but factor V remained low in one case (25%). CONCLUSIONS: The non-parallel results and the apparent increase in factor levels prior to treatment may result from the transient presence of an inhibitor to factors VIII, IX and XI in cases of tiger snake envenomation. Insight into the effects of snake venom on individual coagulation factors could be beneficial when considering new treatments for the coagulopathy induced by snake envenomation.- - - - - - - - - - ranking = 0.2keywords = administration (Clic here for more details about this article) |
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