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1/112. 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.
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keywords = thrombosis
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2/112. Cerebral venous sinus thrombosis associated with hepatic cirrhosis.

    Cerebral venous sinus thrombosis is not a recognized complication of end-stage liver disease. A case of sagittal sinus thrombosis in a 44-year-old male with end-stage hepatic cirrhosis is described. Recurrent seizures were the only manifestation. work-up revealed severe deficiency of protein c, protein s, and antithrombin iii. He was treated with low molecular weight heparin and underwent an orthotopic liver transplant after three months. Follow-up helical CT venogram showed resolution of the sinus thrombosis.
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keywords = thrombosis
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3/112. Carbohydrate-deficient glycoprotein syndrome type 1a: a variant phenotype with borderline cognitive dysfunction, cerebellar hypoplasia, and coagulation disturbances.

    An 8-year-old boy is described with borderline cognitive impairment, cerebellar hypoplasia, a stroke-like episode, and venous thrombosis of the left leg after a period of immobilization. The pattern of multiple abnormalities in blood coagulation suggested carbohydrate-deficient glycoprotein syndrome type 1a. isoelectric focusing of serum transferrin was abnormal. The activity of phosphomannomutase in leukocytes and fibroblasts was decreased. mutation analysis of the PMM2 gene revealed the R141H/E151G genotype. These results confirm the presence of carbohydrate-deficient glycoprotein syndrome type 1a without severe psychomotor retardation.
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ranking = 0.18469086677785
keywords = thrombosis, venous thrombosis
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4/112. 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.
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ranking = 0.0001824719851221
keywords = vein
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5/112. 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.
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ranking = 0.73876346711141
keywords = thrombosis, venous thrombosis
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6/112. Internal jugular vein thrombosis after ovarian stimulation.

    Thromboembolic events are serious, but fortunately rare, complications following ovarian stimulation for IVF. Here, we report a case of internal jugular vein thrombosis after ovarian stimulation with gonadotrophins. Most of the cases of thrombosis are late complications of ovarian hyperstimulation syndrome (OHSS) or hereditary hypercoagulability. Screening for these risk factors in our patient was negative. The patient was successfully treated with low molecular weight heparin and a twin pregnancy is ongoing.
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ranking = 0.8781529328452
keywords = thrombosis, vein thrombosis, vein
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7/112. Thrombotic formations within the aortic arch as source of embolization in patients with coagulopathia.

    Thrombotic formations on atherosclerotic lesions of the thoracic aorta are potential sources of cerebral and systemic embolization. Especially younger patients without calcifications of atherosclerotic plaques or coagulation disorders have a higher risk for embolization. magnetic resonance imaging and transesophageal echocardiography are the diagnostic methods of choice. As an alternative to anticoagulation surgical therapy is indicated to prevent severe brain damage or multiorgan failure in patients with mobile thrombotic formations. Herein we describe two patients in whom successful surgical treatment was performed in deep hypothermic circulatory arrest by excision of the aortic arch atheroma.
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ranking = 0.00017844143509582
keywords = deep
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8/112. 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.
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ranking = 0.14303961484226
keywords = thrombosis, vein
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9/112. brain involvement in haemolytic-uraemic syndrome: MRI features of coagulative necrosis.

    We describe radiological demonstration of brain involvement in haemolytic-uraemic syndrome (HUS) in two siblings with a very different clinical course. While the brother presented with a mild, reversible encephalopathy, his sister developed high-signal lesions in the cortex, putamen and caudate nucleus on T1-weighted images, seen as dense areas on CT. biopsy revealed coagulative necrosis due to microthrombosis without haemorrhage, calcification or infection. These findings suggest a possible prognostic role for MRI in cases of encephalopathy due to HUS.
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ranking = 0.14285714285714
keywords = thrombosis
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10/112. Cerebral vein thrombosis and prothrombin gene (G20210A) mutation.

    Recently, prothrombin gene mutation G20210A has been associated with elevated thrombosis risk and rarely with cerebral vein thrombosis (CVT). Three patients are described who had this genetic predisposition and who developed CVT in an unusual constellation with other factors. In the first patient, the intake of valproic acid (VPA) may have played an aggravating role in the development of CVT; in the second patient diagnosis of coagulation disorder was made during pregnancy consultation 6 years after CVT; in the third patient the CVT occurred at the age of 78 years. In patients with CVT, coagulation-examinations should include tests for the prothrombin gene (G20210A) mutation.
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ranking = 0.8781529328452
keywords = thrombosis, vein thrombosis, vein
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