Budd-Chiari syndrome in association with polycystic disease of the liver and kidneys.
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Biomedical subjects
Publications and source records attributed to D Samson.
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The projected outcome of allogeneic BMT for myeloma based on the EBMT data is as follows: for patients transplanted after first line therapy there is a 30% risk of transplant related death, a 55% chance of being alive at 5 years and a 35% chance of being alive in complete remission at 5 years. If BMT is performed later in the course of the disease, the risk of transplant related death is increased and the chance of long term relapse-free survival correspondingly reduced. Conversely, ABMT has a low mortality risk, but there is currently no evidence that ABMT using unpurged marrow can produce long term cure as no series has shown any evidence of a plateau in remission duration. The early results of maintenance interferon are encouraging but longer follow-up is needed to determine whether the proportion of patients in continuing remission at 5 years will approach that seen after allogeneic BMT. Early results of peripheral blood stem cell transplantation are also encouraging but longer follow-up is required. It remains extremely important when comparing results of ABMT with chemotherapy alone to compare similar patient groups, bearing in mind that patients who have autologous transplantation in first response are by definition responders with good performance status and without significant renal failure. In order to address this problem in a prospective manner, a randomised study has been planned by the EORTC in collaboration with the EBMT. In this study, patients with adequate renal function will be randomised at diagnosis to chemotherapy followed by either autologous BMT or continuing chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)
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The authors report their experience with surgical resection of 108 previously embolized arteriovenous malformations (AVM's). Embolization was performed via only transfemoral catheterization in 70 lesions and via the surgical exposure of feeding vessels in 32. The remaining six patients were referred for resection following silicone sphere embolization elsewhere. Materials used included polyvinyl alcohol (PVA) foam, platinum microcoils, detachable silicone balloons, surgical silk, a mixture of 33% ethanol and microfibrillar collagen, and isobutyl cyanoacrylate (IBCA). It is believed that proximal arterial occlusion with balloons is an inferior choice for preresection embolization, because the technical difficulty of placement is high and the nidus of the AVM is unaffected. Vascular coagulation and section and AVM retraction are more difficult with IBCA; therefore, this is also considered an inferior choice. Among the materials studied, the combination of PVA for distal occlusion and microcoils for proximal occlusion appears to be the superior choice. Fewer complications (stroke or hemorrhage) are seen when intraarterial Amytal (amobarbital) testing is used to guide the embolization. Data regarding toxicity, oncogenicity, and vascular metabolism or recanalization associated with PVA, IBCA, and n-butyl cyanoacrylate are reviewed.
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In preoperative embolization of cerebral arteriovenous malformation (AVM), one difficulty frequently encountered is assessment of the risk of ischemic deficit, posed by proceeding with embolization. One technique to assist prediction of tolerance of devascularization involves superselective injection of sodium amytal into cerebral arteries, prior to and during embolization. Since September, 1985, we have performed 119 embolization procedures in 77 patients with cerebral AVM. Of these, 89 procedures in 60 patients involved superselective intracerebral catheterization and embolization with either polyvinyl alcohol (PVA) alone or in combination with platinum microcoils, surgical silk, or a mixture of ethanol and microfibrillar collagen. In 50 of these procedures, we superselectively administered sodium amytal intraarterially immediately prior to embolization, for purposes of functional testing. Groups of patients undergoing testing were compared to groups not tested prior to embolization, for development of transient or permanent neurologic deficits or cerebral hemorrhage, subsequent to embolization. Significantly better results, both in absolute numbers and severity of complications, were found when testing was employed. No complications of amytal administration were seen. In four cases, embolization was altered or discontinued based on development of a deficit at the time of amytal administration. We conclude that the brief anesthetic effect of intraarterial testing with sodium amytal is a safe and important adjunct during preoperative embolization of cerebral AVMs.
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Endovascular embolization procedures have undergone dramatic evolution and improvement in recent years. Despite these advances, controversy remains regarding the optimal role of these procedures in treating cerebral arteriovenous malformations (AVM's) and whether their purpose should be as a presurgical adjunct or as primary therapy. This controversy risks fragmentation between disciplines in the broader efforts to improve management of cerebrovascular disorders. The authors report seven cases of life-threatening hemorrhages that occurred during staged invasive therapy for AVM's which illustrate the value of a unified team approach to optimize patient care. Each patient underwent at least one embolization procedure using polyvinyl alcohol particles, followed in two cases by the occlusion of proximal feeding vessels by platinum microcoils and in one case by the attempted detachment of an endovascular balloon. In three patients, catheter penetration into the subarachnoid space resulted in subarachnoid hemorrhage. One patient suffered rupture of a large feeding vessel during balloon inflation. The final three patients sustained intracranial hemorrhage 2 hours, 8 hours, and 5 days, respectively, following embolization. All but two patients underwent emergency craniotomy at the time of the complication. These cases underscore the advantages of interdisciplinary management optimizing decision-making and providing expeditious care when life-threatening complications develop.
Although gradual carotid artery occlusion is an accepted and effective treatment for some surgically inaccessible intracranial aneurysms, there are no specific guidelines to determine the optimal amount of carotid artery narrowing at each adjustment. The technique of transcranial Doppler ultrasound, however, allows continuous measurement of blood velocity in the middle cerebral artery as the carotid artery is narrowed, so that hemodynamic effects and development of collateral flow can be immediately assessed at the bedside at each adjustment of the carotid artery diameter. This case report describes the use of transcranial Doppler to guide the rate of carotid occlusion in a patient with an unclippable giant aneurysm of the carotid artery. Sudden carotid occlusion and gradual occlusion at the usual rate were not tolerated by the patient, but repeatedly tightening the clamp until the first signs of attenuation of the Doppler signal allowed an expedient occlusion without complication. Transcranial Doppler ultrasound can provide a useful dynamic guide to gradual therapeutic carotid occlusion.
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Hydroxyethyl methacrylate (HEMA) has been advocated as a polymerizing solution with which to prevent deflation of detachable balloons in interventional neuroradiology. It is pertinent to know if unpolymerized HEMA would have untoward effects if accidentally released into the carotid artery by balloon rupture or deflation. Seven mongrel dogs underwent transfemoral catheterization of the common carotid artery and subsequent injection of HEMA solution in volumes of 1 cc in five dogs, 2 cc in one, and 4 cc in one. Angiography performed at the time of injection revealed evidence of intravascular thrombosis as well as possible spasm. Three surviving animals were sacrificed at 48 hours; the brains were fixed and examined histopathologically. One brain was normal and one was autolyzed and could not be examined. Five of the seven animals had histopathologically documented cerebral infarctions of varying size. No foreign substance was seen within the blood vessels to suggest intravascular polymerization. The animals injected with 2 or 4 cc HEMA solution did not survive 48 hours. Literature review reveals little documentation of the toxicology of intravascular HEMA. With its increasing popularity as a compound for polymerization in detachable balloons introduced into the brain, further investigations are warranted to understand the physical properties of the compound and potential risks of its use.
In order to determine possible risk factors and to assess the value of platinum microcoils added to polyvinyl alcohol particles in preoperative embolization of cerebral arteriovenous malformations in adults, we reviewed our experience with this procedure. Between September 1985 and June 1989, we performed embolizations in 54 patients with cerebral arteriovenous malformations. Of these, procedures in 51 adults involved the use of polyvinyl alcohol particles, either alone (n = 29) or in combination with platinum microcoils (n = 21). A complication during catheterization precluded embolization in another patient. Beginning as flow-directed embolizations via carotid artery catheterizations (n = 12), newer catheters allowed progression to superselective intracerebral catheterizations (n = 38). Embolization has led to shorter surgical procedures, more clearly defined operative margins, and less bloody operative fields. We have not found recanalization to significantly hinder embolization results with polyvinyl alcohol when resection is undertaken within 1-4 weeks of embolization. Its relative safety and ease of manipulation at surgery argue for its use. We found no significant increase in complications based on patient age, venous drainage of the arteriovenous malformation, or the circulation embolized. Embolization results in cerebral arteriovenous malformations were improved with superselective catheterization and most improved with the combined use of polyvinyl alcohol for nidus embolization followed by occlusion of the feeding vessel with microcoils.
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32 previously untreated patients with multiple myeloma received vincristine, doxorubicin ('Adriamycin'), and dexamethasone (VAD) as first-line therapy. The overall response rate was 84%, with 28% of all patients entering complete remission. Response was rapid, with near-maximum response occurring after two courses of treatment and rapid improvement in bone-marrow function. Median response duration was 18 months and this seemed to be unaffected by initial prognostic criteria or by degree of remission achieved. The projected median survival was 44 months, with 75% of all patients and 83% of responders being alive at 2 years. Side-effects due to steroids were common, but there was only 1 treatment-related death. The high response rate and lack of toxicity offer an advantage over other forms of initial treatment, although other strategies will be necessary to prolong the duration of response.
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