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1/20. Fatal haemorrhagic infarct in an infant with homocystinuria.

    Thrombotic and thromboembolic complications are the main causes of morbidity and mortality in patients with homocystinuria. However, it is unusual for thrombosis and infarction to be the presenting feature leading to investigation for homocystinuria and cerebrovascular lesions in the first year of life. We describe a previously healthy 6-month-old infant who presented with a large middle-cerebral-artery territory infarction and died of massive brain swelling. homocystinuria due to cystathionine beta-synthase (CBS) deficiency was diagnosed by metabolite analysis and confirmed by enzymatic activity measurement in a postmortem liver biopsy. homocystinuria should be considered in the differential diagnosis of venous or arterial thrombosis, regardless of age, even in the absence of other common features of the disease. We recommend systematic metabolic screening for hyperhomocysteinemia in any child presenting with vascular lesions or premature thromboembolism.
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ranking = 1
keywords = thromboembolism
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2/20. Thromboembolism originated from the pulmonary artery stump after Fontan operation.

    Cerebral thromboembolism is a rare but serious complication after Fontan operation. This is the report of a patient who underwent a successful intracardiac thrombectomy for cerebral thromboembolism after Fontan operation. A 2-year-old girl was referred to us with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, and a ventricular septal defect. Although she underwent a successful Fontan operation and division of the main pulmonary artery, she developed a cerebrovascular event at 3 weeks after the operation. echocardiography demonstrated a large thrombus within the residue of the main pulmonary artery, and suggested that the thrombus had migrated into the systemic circulation by way of the ventricular septal defect. At 2 weeks after the cerebrovascular event, she underwent thrombectomy and excision of the pulmonary valve. Although she has developed slight left-sided hemiparesis, she is leading a normal life at 1 year after the operation.
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ranking = 2
keywords = thromboembolism
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3/20. Immediate postoperative thrombolytic therapy: an aggressive strategy for neurologic salvage when cerebral thromboembolism complicates carotid endarterectomy.

    A 42-year-old man with a high-grade left internal carotid artery (ICA) stenosis demonstrated on a duplex scan was referred to us. A cerebral arteriogram confirmed a greater than 90% left internal carotid stenosis, but with the unexpected finding of a moderate amount of thrombus in the proximal ICA. He underwent emergent left carotid endarterectomy, but during the operation, only a small amount of thrombus was identified as adherent to the atherosclerotic plaque. he awakened in the operating room with a dense right hemiplegia and aphasia. Immediate reexploration demonstrated a patent endarterectomy site, a distal thromboembolectomy was performed without extraction of thrombus, and urokinase (250,000 Units) was infused into the distal ICA. He reawakened with an unchanged right hemiplegia and aphasia. The patient then underwent an urgent postoperative carotid and cerebral arteriogram that demonstrated an embolus to the middle cerebral artery. he was treated with the superselective infusion of urokinase (500,000 Units), with almost complete resolution of the clot. Over the course of the next 48 hours, the patient made a nearly complete neurologic recovery, and he was discharged from the hospital with only a slight facial droop. At 2 months' follow-up he was completely neurologically healthy. To our knowledge this is the first reported case of urokinase administered in the immediate postoperative period in the angiography suite to treat a thromboembolus complicating a carotid endarterectomy.
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ranking = 4
keywords = thromboembolism
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4/20. Metastatic unknown primary tumor presenting in pregnancy as multiple cerebral infarcts.

    BACKGROUND: Cancer presenting during pregnancy is a rare event. There are no reports of an unknown primary tumor presenting during pregnancy. CASE: A 35-year-old primigravida presented at 16 weeks' gestation with multiple cerebral infarcts. After a negative workup she was discharged on anticoagulation therapy, only to return at 29 weeks with diffuse, metastatic cancer of unknown primary origin. After an elective 32-week delivery she received aggressive chemotherapy but ultimately died 5 months later. CONCLUSION: Malignancy should be included in the differential diagnosis of unusual, unexplained cases of thromboembolism in pregnancy.
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ranking = 1
keywords = thromboembolism
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5/20. thrombolytic therapy for delayed, in-hospital stroke after cardiac surgery.

    Five patients who had delayed stroke after cardiac surgery underwent intraarterial administration of a fibrinolytic agent for thromboembolism (n = 4) or thrombosis (n = 1) of the cerebral artery. Complete recanalization of the occluded artery was obtained in 3 patients and partial recanalization in 2. Additional angioplasty for basilar artery stenosis was performed in 1 patient. No patients exhibited rebleeding into the pericardial space or wound bleeding. All patients survived with moderate or full functional recovery. Immediate cerebral angiography and local thrombolysis may improve functional outcome and survival in patients with postcardiotomy cerebral thromboembolism.
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ranking = 2
keywords = thromboembolism
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6/20. Retrograde cerebral perfusion with circulatory arrest in aortic arch aneurysms.

    Two surgical cases of aortic arch aneurysms are presented. Retrograde (venoarterial) cerebral perfusion during circulatory arrest under deep hypothermia was performed to evacuate air and debris in cerebral vessels and preserve cerebral tissue. On postoperative day 1, the patients were conscious and alert with no neurological deficit. This technique is simple and can be applied during standard cardiopulmonary bypass. The technique is useful to avoid cerebral air and thromboembolisms.
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ranking = 1
keywords = thromboembolism
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7/20. Cerebral thrombosis in a newborn with a congenital deficiency of antithrombin III.

    An Israeli Arab family with type I antithrombin III (AT-III) deficiency with several affected symptomatic members in three generations is reported. The propositus presented with deep vein thrombosis and pulmonary emboli associated with gestation. The propositus infant presented at the age of 2 weeks with superior sagittal and rectus sinus thrombosis. Hereditary AT-III deficiency should be considered in infants with cerebral thrombosis, especially if they have a family history of thromboembolism. The role of prophylactic therapy by AT-III concentrates in these infants should be further assessed.
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ranking = 1
keywords = thromboembolism
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8/20. Cerebral thromboembolism due to antithrombin iii deficiency in two children.

    Despite numerous well-described causes of stroke in infancy and childhood, a significant proportion remains unexplained. venous thromboembolism is a common complication in adult patients undergoing surgery, and after severe trauma, but not in otherwise healthy children less than 10 years old. However, it may also occur spontaneously without recognizable cause. It has been known for a long time that some patients are particularly prone to venous thrombosis and in recent years great efforts have been made to identify the risk factors. The attention of haematologists has been focused on the possibility that certain abnormalities of coagulation may be associated with a tendency to thrombosis, but only in a few instances a clear causal relationship has been established. One such example is a deficiency of antithrombine III, but such a deficiency has hitherto been recognized as a cause for thrombosis in children only in very particular circumstances. We present two young children with stroke of which one was purely ischemic and the other ischemic with secondary hemorrhage. Both our patients showed an AT III deficiency. Patient one also had a cyanotic congenital heart disease with right to left shunting which made cerebral embolism originating from a thrombus in the iliac vein possible to occur. We consider her hematocrit values too low to be a predisposing factor for this thrombosis. AT III deficiency may be caused by several different mechanisms. Either it exists as a congenital (hereditary) or as an acquired disorder. In patient two the family history was positive for hereditary AT III deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)
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ranking = 5
keywords = thromboembolism
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9/20. Maternal and neonatal death associated with Eisenmenger's syndrome.

    pregnancy in patients with Eisenmenger's syndrome is known to be associated with a high incidence of maternal and perinatal death. Complications can occur before and during parturition but are most hazardous in the early postpartum period. If the exact causes of maternal death remain in most instances unknown, hypovolemia with augmented pulmonary to systemic shunt, thromboembolism and cardiac arrythmias are often mentioned as the primary causes of mortality. We report the unsuccessful management of a pregnant patient with Eisenmenger's disease despite all possible precautions as reported in the literature had been taken. We discuss these precautions and more specifically the type of anesthesia that is best given, the prophylactic methods of anticoagulation and the benefit of monitoring techniques in the management of such complex pathologies.
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ranking = 1
keywords = thromboembolism
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10/20. Cerebral haemorrhagic infarction in young patients with hereditary protein c deficiency: evidence for "spontaneous" cerebral venous thrombosis.

    Among 53 patients with hereditary protein c deficiency belonging to 20 families three women were encountered who, aged 27, 34, and 38 respectively, had had cerebral haemorrhagic infarction, probably due to intracranial venous thrombosis. All three had also had venous thrombosis of the leg and pulmonary embolism either before or after their cerebral infarction. One patient sustained cerebral infarction while receiving an oral contraceptive, but infarction in the two others occurred "spontaneously." One patient also had an intraventricular and subarachnoid haemorrhage during the induction phase of coumarin treatment, which was assumed to have resulted from haemorrhagic infarction of the chorioid plexus, analogous to coumarin provoked haemorrhagic skin necrosis in protein c deficiency. Hereditary protein c deficiency should be considered in young patients with acute or subacute cerebral symptoms, especially if they have a family or personal history of venous thromboembolism.
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ranking = 1
keywords = thromboembolism
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