Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cerebrovascular disorders are an important cause of mortality and chronic morbidity in children. Ischemic stroke is more common than cerebral venous thrombosis and hemorrhagic stroke in children. Several medical disorders have been associated with stroke in children, and a thorough evaluation of underlying causes is needed to determine the best treatment and prevention strategy. The treatment and prevention of stroke in children is not well studied, and current recommendations are based on adult studies, nonrandomized trials, or expert opinion. Children with stroke require immediate, special attention and if possible should be stabilized and transferred to an institution that can offer pediatric neurovascular expertise and care. All children with stroke should be referred to or have their care managed by a pediatric neurologist. The treatment of stroke in adults is well studied, and when applicable this evidence should be considered in the treatment of children with stroke. Data from animal and adult stroke studies have demonstrated a benefit for the aggressive treatment of infection, fever, blood pressure, hypo/hyperglycemia, intracranial pressure, and seizures, and should be applied to children with stroke. The use of thrombolytic, antithrombotic, and antiplatelet therapies is based on adult studies, cohort studies, and/or expert opinion. Two consensus guidelines regarding the treatment of arterial ischemic stroke and cerebral venous thrombosis were recently published and recommend the use of anticoagulants or antiplatelet agents in the acute setting, depending on the underlying cause of stroke. The evidence for the primary prevention of stroke in children is restricted to sickle cell disease (SCD) and derived from the Stroke Prevention in Sickle Cell Study Project studies. Long-term chronic transfusion therapy to maintain hemoglobin S levels below 30% is indicated in children with SCD and intracranial stenosis. It has also been recently determined that chronic transfusion therapy should not be stopped in children with SCD and an increased risk for stroke. The recurrence rate of arterial ischemic stroke (AIS) in children ranges from 6% to 30% and is highest among children with recurrent transient ischemic attack, cardiac disease, arteriopathies, and metabolic and coagulation abnormalities. Recommendations for secondary prevention are based on adult studies and the underlying pathophysiology of the stroke. Antiplatelet therapy (aspirin 1-5 mg/kg/day) is recommended in most children with a history of AIS. Although there is minimal evidence to support its use in children, anticoagulation may be indicated in AIS associated with extracranial arterial dissection, prothrombotic disorders, cardiac disease, severe intracranial stenosis, and recurrent AIS while on antiplatelet therapy.
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PMID:Treatment and prevention of cerebrovascular disorders in children. 1622 70

The Kidd blood group is clinically significant since the Jk antibodies can cause acute and delayed transfusion reactions as well as hemolytic disease of newborn (HDN). In general, HDN due to anti-Jk(b) incompatibility is rare and it usually displays mild clinical symptoms with a favorable prognosis. Yet, we apparently experienced the second case of HDN due to anti-Jk(b) with severe clinical symptoms and a fatal outcome. A female patient having the AB, Rh(D)-positive blood type was admitted for jaundice on the fourth day after birth. At the time of admission, the patient was lethargic and exhibited high pitched crying. The laboratory data indicated a hemoglobin value of 11.4 mg/dL, a reticulocyte count of 14.9% and a total bilirubin of 46.1 mg/dL, a direct bilirubin of 1.1 mg/dL and a strong positive result (+++) on the direct Coomb's test. As a result of the identification of irregular antibody from the maternal serum, anti-Jk(b) was detected, which was also found in the eluate made from infant's blood. Despite the aggressive treatment with exchange transfusion and intensive phototherapy, the patient died of intractable seizure and acute renal failure on the fourth day of admission. Therefore, pediatricians should be aware of the clinical courses of hemolytic jaundice due to anti-Jk(b), and they should be ready to treat this disease with active therapeutic interventions.
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PMID:A fatal case of severe hemolytic disease of newborn associated with anti-Jk(b). 1647 82

Focal neocortical seizures, induced by injection of 4-aminopyridine, were imaged in the rat neocortex using the intrinsic optical signal, with incident light at various wavelengths. We observed focal, reproducible and prolonged reflectance drops following seizure onset, regardless of wavelength, in the ictal onset zone. A persistent drop in light reflectance with incident orange light, which corresponds to a decrease in oxygenated hemoglobin, was observed. We describe this phenomenon as an 'epileptic dip' as it is reminiscent of the 'initial dip' observed using the intrinsic optical signal, and also with blood oxygen level-dependent functional magnetic resonance imaging, after normal sensory processing, although with much longer duration. This persistent ictal ischemia was confirmed by direct measurement of tissue oxygenation using oxygen-sensitive electrodes.
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PMID:Intrinsic optical signal imaging of neocortical seizures: the 'epileptic dip'. 1654 14

An 8-year-old Yorkshire terrier developed acute onset coma and seizure after cranial trauma. Intracranial hemorrhage was suspected from the clinical signs and history. Low-field magnetic resonance (MR) imaging revealed a round mass within the right cerebral hemisphere, compressing the right lateral ventricle and displacing the longitudinal fissure to the left. The lesion was hypointense on T1-weighted images and hyperintense on T2-weighted images, consistent with an acute hemorrhage. MR imaging was performed every 24 h for 6 days from 1 h after the injury, and then on day 14 of hospitalization. With time, the signal intensity changed to hyperintense on Ti-weighted images. On T2-weighted images the center of the mass changed to hypointense, and then to hyperintense with a hypointense rim. These changes of signal intensity were related to hemoglobin oxidation.
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PMID:Sequential magnetic resonance imaging of an intracranial hematoma in a dog. 1655 45

Carbon monoxide arises during incomplete combustion of organic material, is incorporated into the circulation via the lungs and displaces oxygen from hemoglobin. Consecutively, symptoms of intoxication such as headache, vertigo, nausea, seizures and coma may result in a dose dependent fashion. Carbon monoxide is however also generated endogenously during heme degradation catalysed by heme oxgenase enzymes. The isoform hemeoxygenase-1 is inducible by oxidative stress and may mediate cytoprotection mainly attributable to endogenously produced carbon monoxide. Exogenous applied carbon monoxide has also been shown to confer protection in experimental studies. Meanwhile, in addition to the toxicological properties, antiinflammatory and cytoprotective effects of carbon monoxide have moved into the focus of scientific interest.
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PMID:[Carbon monoxide: toxic molecule with antiinflammatory and cytoprotective properties]. 1655 39

Pharmacological induction of hemoglobin F expression may be a promising approach for the treatment of beta-thalassemia and sickle cell disease. Valproic acid, a drug frequently used for the treatment of seizure disorders, has been shown to enhance fetal hemoglobin synthesis in erythroid cells. However, this effect is only modest and requires relative high concentrations. Therefore, the drug appears not to be applicable for the treatment of beta-globin chain disorders. Here, we describe the identification of novel valproic acid derivatives with potent hemoglobin F inducing activities at concentrations that presumably can be obtained in vivo.
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PMID:Novel valproic acid derivatives with hemoglobin F inducing activity. 1662 26

Cocaine abuse increases the risk of life-threatening neurological complications such as strokes and seizures. Although the vasoconstricting properties of cocaine underlie its cerebrovascular effects, the mechanisms underlying its neurotoxicity remain incompletely understood. Here, we use optical techniques to measure cerebral blood volume, hemoglobin oxygenation (S(t)O(2)), and intracellular calcium ([Ca(2+)](i)) to test the hypothesis that cocaine increases [Ca(2+)](i) in the brain. The effects of cocaine were compared with those of methylphenidate, which has similar catecholaminergic effects as cocaine (except for serotonin increases) but no local anesthetic properties, and of lidocaine, which has similar local anesthetic effects as cocaine but is devoid of catecholaminergic actions. To control for the hemodynamic effects of cocaine, we assessed the effects of cocaine in animals in which normal blood pressure was maintained by infusion of phenylephrine, and we also measured the effects of transient hypotension (mimicking that induced by cocaine). We show that cocaine induced significant increases ( approximately 10-15%) in [Ca(2+)](i) that were independent of its hemodynamic effects and of the anesthetic used (isofluorance or alpha-chloralose). Lidocaine but not methylphenidate also induced significant [Ca(2+)](i) increases ( approximately 10-13%). This indicates that cocaine at a dose within the range used by drug users significantly increases the [Ca(2+)](i) in the brain and its local anesthetic, but neither its catecholaminergic nor its hemodynamic actions, underlies this effect. Cocaine-induced [Ca(2+)](i) increases are likely to accentuate the neurotoxic effects from cocaine-induced vasoconstriction and to facilitate the occurrence of seizures from the catecholaminergic effects of cocaine. These findings support the use of calcium channel blockers as a strategy to minimize the neurotoxic effects of cocaine.
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PMID:Cocaine increases the intracellular calcium concentration in brain independently of its cerebrovascular effects. 1709 73

Children with sickle cell disease, a chronic hemolytic anemia, present with a wide variety of neurological syndromes, including ischemic and hemorrhagic stroke, transient ischemic attacks, 'soft neurological signs', seizures, headache, coma, visual loss, altered mental status, cognitive difficulties, and covert or 'silent' infarction. Those with ischemic stroke usually have stenosis or occlusion of the distal internal carotid and proximal middle cerebral arteries. Indefinite transfusion prevents recurrence in most patients who have had a stroke, and can prevent first stroke in those with high transcranial Doppler velocities. High white cell count, low hemoglobin and oxyhemoglobin desaturation predict neurological complications. Other risk factors for overt ischemic stroke include hypertension, previous transient ischemic attack, covert infarction and chest crisis. For hemorrhagic stroke, aneurysms are common in adults but not children, who often present with hypertension after transfusion or corticosteroids. Seizures are particularly common in patients with cerebrovascular disease and covert infarction; the latter is also associated with hyposplenism and infrequent pain. Factors associated with cognitive difficulties include thrombocytosis, infarction, large-vessel disease, and perfusion abnormality on neuroimaging. As well as investigating the role of genes and the possibility that hydroxyurea or blood pressure control reduce neurological complications, we should explore the modifiable effects of poor nutrition, chronic infection, hemolysis and oxyhemoglobin desaturation on stroke risk.
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PMID:Therapy insight: stroke risk and its management in patients with sickle cell disease. 1747 74

Optical topography is a new alternative for noninvasive functional brain mapping. It uses near infrared light projected from the scalp into the brain. The reflected light which is sampled 30 mm apart from the injection probe carries the information of hemoglobin concentration change of the regional brain tissue lying at the midpoint of probes. When mapped using 24 channels, we see the real time brain activities during various tasks allowing the head movements as well. It is utilized in measuring the language function, and diagnosis of epilepsy focus. Several such clinical applications are demonstrated. The brain activities are measured during finger tapping. The mapping during word generation task shows the activities of Broca's area indicating the hemispheric dominancy of language function. We correlated the results between optical topography measurement and amytal test, showing 85% matching according to the language dominancy. It also useful in identifying the epilepsy focus because the hemoglobin increases at the focus region in the initial phase of epileptic seizures. In our study, the feasibility of optical topography in focus diagnosis is confirmed in 85% of 27 cases. The advantages of optical topography are as follows. 1: It is portable to various clinical scenes including patient ward and outpatient clinic. 2: It can be used conjunction with fMRI and MEG. 3: It measures the brain activities even during the head movements.
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PMID:[Applications of optical topography in neurosurgery]. 1753 71

Screening for pulmonary hypertension (pHTN) has not yet become routine in sickle cell disease (SCD), despite clinical evidence of its high prevalence and associated mortality. Our objectives are to identify clinical conditions and laboratory findings predictive of/or associated with pHTN. One hundred twenty-five adult outpatients with Hb SS, SC, SOArab, Sbeta(0), or Sbeta(+) thalassemia, who underwent echocardiography and/or right heart catheterization due to cardiorespiratory symptoms, were studied. pHTN was identified in 36% (28/77) of SS/Sbeta(0) and in 25% (12/48) of SC/SOArab/Sbeta(+) patients studied. In SS/Sbeta(0) patients, pHTN was associated with low hemoglobin, low GFR, increasing age, no history of treatment with hydroxyurea and a history of leg ulcers, with trends for associations with higher total bilirubin, LDH levels, systolic systemic blood pressure, history of avascular necrosis, seizures, and cerebrovascular events. Twelve (40%) of the SS/Sbeta(0) patients with pHTN had >or= 1+ proteinuria. (P<0.039). The presence of proteinuria correlated with lower GFR and had a high positive predictive value (0.60) for pHTN in subjects with SS/Sbeta(0). The data also provided evidence that pHTN in this population is associated with right heart failure, with echocardiographic evidence of right ventricle enlargement and pericardial effusion. This study confirmed that even relatively mild elevations in pulmonary pressure are associated with high prospective mortality (hazard ratio: 15.9). We concluded that pHTN has a high prevalence in all Hb S related syndromes and is associated with increased mortality in SS/Sbeta(0). Kidney dysfunction, as indicated by proteinuria or decreased GFR, also represents sufficient reason to screen for pHTN.
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PMID:Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. 1772 99


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