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Query: UMLS:C0038454 (stroke)
 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine cases have been presented in detail to illustrate some of the varied causes of sudden neurological deficit in childhood: arteriovenous malformation, cryptic hamartoma, berry aneurysm, mycotic aneurysm, intraspinal arteriovenous malformation, brain tumor, migraine, arteritis, and multiple sclerosis. The Boston Children's Hospital experience with aneurysms and intracranial arteriovenous malformation has been summarized. It is noteworthy that a cutaneous hemangioma overlay one cranial and one intraspinal arteriovenous malformation. One small but deep cerebral arteriovenous malformation apparently destroyed itself after its second hemorrhage. Not only have multiple sclerosis and a brain tumor mimicked a vascular lesion, but a series of vascular accidents was misdiagnosed first as multiple sclerosis then as a thalamic tumor. The many possible causes of childhood strokes has been thoroughly cataloged in the Report of the Joint Committee for Stroke Facilities in 1973 (11). Children may be more susceptible to strokes because of congenital abnormalities such as congenital heart disease, hemophilia, and sickle cell anemia, or by diseases which more commonly occur in this age group, such as leukemia. The likelihood of brain abscess in cyanotic congenital heart disease is stressed. Arteriographic studies in our series have been safe; however, there have been reports of probable worsening of symptoms in children with multiple cerebral occlusive lesions in the presence of homocystinuria.
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PMID:Strokes in children. 98 45

Psychological reactions and their 'hierarchies' regarding the own health status were analysed in a sample of 81 survivors of the Bonn Longitudinal Study on Aging and in different clinical samples (patients suffering from hemophilia, renal failure, heart infarction, stroke, and schizophrenia). The longitudinal analysis of the health-related response hierarchy points to a high degree of consistency of these reactions over time. Comparing the response hierarchies of the aged sample as related to different areas of life stress (such as housing, income, family, health problems), the author finds a high degree of discriminative competence in coping with stress. The same is true for the response hierarchies of the patient groups. This high degree of situation-specific selectivity in patterns of responses to chronical disease should be studied more intensively from theoretical as well as clinical-practical points of view.
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PMID:[Reactions to health stress in middle and advanced adulthood]. 648 77

We report the use of recombinant VIIa (rFVIIa) in the treatment of five ICHs in two factor VIII-deficient patients with inhibitors. In four of five ICHs, rFVIIa was the only factor replacement used at doses of 60-135 micrograms/kg every 2-4 hr for 12-14 days. Hemostasis at the primary site of bleeding was achieved in all cases, and all patients survived with no permanent neurologic deficits. However, the patient who received the highest dose of rFVIIa during the first 4 days of therapy developed clinical symptoms consistent with a cerebral vascular accident of the brainstem characterized by acute onset of truncal ataxia and upward-gaze nystagmus on day 8 of rFVIIa therapy. While receiving rFVIIa therapy for treatment of these five ICHs, four treatment courses were complicated by bleeding at sites other than the primary site, including two episodes of localized oozing at central line insertion sites, two episodes of hemarthrosis, and two episodes of epistaxis. Antifibrinolytic therapy with tranexamic acid was effective in two of these episodes. Laboratory evaluation revealed shortening of the PT, variable shortening without normalization of the APTT, peak factor VII activity levels 7-30-fold higher than normal baseline values, and normal antithrombin III (ATIII) and alpha 2-antiplasmin levels. In four of five ICHs, there was a 20-40% decrease in fibrinogen levels from baseline. The decrease in fibrinogen was accompanied by an increase in fibrin degradation products in 3/5 episodes and a 15-35% decrease in plasminogen activity levels in 2/5 episodes. Tissue factor pathway inhibitor (TFPI) levels remained stable and in the normal range. Although rFVIIa is an effective new therapy for the treatment of ICH in hemophilia patients with inhibitors, its optimal use with respect to safety and efficacy requires further clinical study.
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PMID:Recombinant activated factor VII (rFVIIa) therapy for intracranial hemorrhage in hemophilia A patients with inhibitors. 804 14

Vascular endothelial cells are critical participants in maintaining blood flow, with the ability to respond rapidly to injury. We have outlined above how the regulated secretion of a variety of hemostatic and inflammatory mediators contributes to these nearly instantaneous responses. The WPB are the most prominent of these regulated secretory granules, and there is growing evidence of additional granules that release their contents under a variety of conditions. The mechanisms responsible for the targeting of proteins to regulated secretory granules, and of exocytosis of these granules are being elucidated. EC appear to share some characteristics with other secretory cell types, but also are likely to have unique properties related to the storage and secretion of large multimeric proteins such as VWF and multimerin. Understanding these mechanisms may lead to new strategies for treating coronary artery disease, stroke, sickle cell disease, and hemophilia through drugs that modulate sorting and secretion, or by gene transfer approaches that introduce therapeutic molecules into the WPB for regulated release.
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PMID:Regulated secretion in endothelial cells: biology and clinical implications. 1181 99

Porcine factor VIII (pFVIII), which is used to control bleeding in patients with congenital or acquired haemophilia who have high-titre neutralizing antibodies to human FVIII, is not known to increase the risk of arterial or venous thrombosis. We have recently encountered a patient with acquired haemophilia who developed a thrombotic left middle cerebral artery distribution stroke while being treated with pFVIII. To our knowledge, this is the first such reported thrombotic event. We speculate that platelet activation induced by pFVIII may have contributed to thrombosis and suggest that pFVIII be used with caution in elderly patients with pre-existing cardiovascular risk factors.
Haemophilia 2002 Jan
PMID:Thrombotic stroke associated with the use of porcine factor VIII in a patient with acquired haemophilia. 1188 66

Historically, the leading cause of death among persons with haemophilia and other congenital coagulation disorders was uncontrolled bleeding. Mortality was associated with severe deficiency of coagulation factors VIII or IX and especially with high-titre antifactor neutralizing antibodies (inhibitors). The catastrophic contamination of plasma donor pools with human immunodeficiency virus (HIV) resulted in acquired immunodeficiency syndrome replacing haemorrhage as the leading cause of death among persons with haemophilia. Rather little has been written, however, about mortality among those not infected with HIV. The objective of this study was to identify conditions associated with all-cause mortality among HIV-uninfected patients who were followed for a mean of 8.8 years in the Multicentre Hemophilia Cohort Study. Among the 364 children (mean age 8 years), there were four deaths; two related to cancer, one to trauma, and the fourth to haemorrhage, end-stage liver disease and sepsis. Among the 387 HIV-uninfected adults (mean age 35 years) there were 29 deaths, with haemorrhage the leading cause of death, followed by hepatic, stroke and cancer deaths. Prognostic factors for all-cause mortality among the adults included haemophilia Type A with neutralizing antibodies [age-adjusted relative rate (RR) 3.1, 95% confidence interval (CI) 1.4-6.9] and serologic evidence of both hepatitis B and C virus (RR 4.1, 95% CI 0.97-17.6). Although hepatitis C viral load was slightly lower in patients with hepatitis B virus surface antigenaemia, it was unrelated to vital status. We conclude that causes of death and prognostic factors for current HIV-uninfected haemophilia patients are similar to those noted before the HIV epidemic. Better understanding, prevention and control of neutralizing antibodies and hepatitis infections may substantially improve longevity for people with haemophilia.
Haemophilia 2002 Sep
PMID:Factors predictive of death among HIV-uninfected persons with haemophilia and other congenital coagulation disorders. 1219 76

Stroke is a major health problem worldwide, causing high morbidity and mortality. Intracerebral hemorrhage (ICH) accounts for 15% of stroke cases in the US and Europe and up to 30% in Asian populations. It is less treatable than other forms of stroke and causes higher morbidity and disability. Data suggest that early hematomy growth is the principal cause of early neurological deterioration after ICH. Prospective and retrospective studies indicate that early hematoma growth occurs in 18-38% of patients scanned within 3 h of ICH onset, and that hematoma volume is an important predictor of 30-day mortality. As hematoma growth in acute ICH is a dynamic process, intervention with ultra-early hemostatic therapy could lead to minimization and even prevention of early hematomy growth. Recombinant activated factor VII (rFVIIa, 'NovoSeven'), a powerful initiator of hemostasis, is approved for the treatment of bleeding in patients with hemophilia and inhibitors and may also promote hemostasis in patients with normal coagulation. rFVa acts locally at the bleeding site without activating systemic coagulation and may be a valuable therapy during the hyperacute stage of ICH. A randomized, double-blind, placebo-controlled, dose-ranging trial is currently in progress to investigate the potential of rFVIIa as an ultra-early hemostatic therapy to prevent or minimize hematoma growth in ICH patients without coagulopathy.
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PMID:Intracerebral hemorrhage: natural history and rationale of ultra-early hemostatic therapy. 1240 92

Although intracranial hemorrhage accounts for approximately 10 to 15% of all cases of stroke, it is associated with a high mortality rate. Bleeding disorders account for a small but significant risk factor associated with intracranial hemorrhage. In conditions such as hemophilia and acute leukemia associated with thrombocytopenia, massive intracranial hemorrhage is often the cause of death. The authors present a comprehensive review of both the physiology of hemostasis and the pathophysiology underlying spontaneous ICH due to coagulation disorders. These disorders are divided into acquired conditions, including iatrogenic and neoplastic coagulopathies, and congenital problems, including hemophilia and rarer diseases. The authors also discuss clinical features, diagnosis, and management of intracranial hemorrhage resulting from these bleeding disorders.
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PMID:Spontaneous intracerebral hemorrhage due to coagulation disorders. 1534 96

Specific treatment in the acute phase after cerebral haemorrhage is still not available. Corticosteroids and early surgical evacuation have been proven to be ineffective. In the initial 3 hours after a cerebral haemorrhage, the haematoma volume increases in one-third of all patients. Arresting this enlargement may be an effective strategy to improve outcome. Recombinant activated factor VII (rFVIIa) is used to treat bleeding in patients with haemophilia and has also been reported to reduce bleeding in patients without coagulopathy. In a recently published phase-II trial totalling 399 patients given placebo or one of three doses of rFVIIa, treatment with rFVIIa reduced haematoma volume 24 hours after administration. Treatment with rFVIIa was also superior to placebo for several secondary endpoints, including death, handicap and neurological deficits. However, more thromboembolic complications, including myocardial infarction and ischaemic stroke, occurred in the groups given rFVIIa. A new trial, with death or dependency as the primary outcome, has recently been started. The results are expected early in 2007.
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PMID:[Acute treatment with recombinant factor VII is probably indicated for intracerebral haematoma]. 1612 78

Myocardial infarction and other arterial thrombosis are commonly maintained to be rare in hemophilia patients. This, in general, seems true but the occurrence of a thrombotic event in hemophilia B is not exceptional. A thorough search of the literature has yielded 13 patients with myocardial infarction and 1 patient with a cerebrovascular accident. There were three fatalities. In five cases MI occurred after infusion of Prothrombin Complex Concentrates. In three additional patients the event occurred after infusion of plasma, Feiba or cryoprecipitate supernatant. Four patients had an antero-lateral infarction. Two had a non-Q infarction and one each showed a multiple or a posterior-inferior form. Several therapeutic coronary procedures (GABG and PTCA) were carried out in hemophilia B patients without undue complication providing adequate level of FIX were maintained. Heparin prophilaxis was used in all patients but one. The analysis of the literature indicates that (1) MI may occur in hemophilia B patients and (2) that invasive coronary artery therapeutic procedures may be carried out without complications.
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PMID:Myocardial infarction, other arterial thrombosis and invasive coronary procedures, in hemaophilia B: a critical evaluation of reported cases. 1613 95


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