hemoglobinopathies/seizures

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Extended therapy with intravenous arginine butyrate in patients with beta-hemoglobinopathies.

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BACKGROUND Enhanced production of fetal hemoglobin lessens the severity of beta-thalassemia and sickle cell disease. Intravenous infusion of arginine butyrate can increase the number of reticulocytes containing fetal hemoglobin in patients with these disorders, and it has induced a substantial

[A familial case of DRPLA diagnosed by an autopsy associated with hemoglobinopathy (Hb Takamatsu)].

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The patient first noticed general muscle stiffness at the age of 36. Two years later, she suffered from a tonic-clonic seizure which brought her to a hospital for the first time. Choreoathetoid movement, ataxia and cognitive deficit were apparent. At the age of 44, tonic-clonic seizures became more

Neurological complications of hemoglobin SC disease.

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The records of 68 patients with hemoglobin SC disease and 68 age- and sex-matched control patients were reviewed for neurological problems. A significant increase in retinopathy, stupor/coma, and seizures was noted in the hemoglobin SC group. Hemiplegia, noted in two young patients, was probably

Risk factors for epilepsy: case-control study in Nigerians.

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In a case-control study we compared 155 Nigerians affected by epilepsy, with an equal number of controls matched for age and sex, with the aim of identifying risk factors of epilepsy. Febrile convulsions and head trauma (with odds ratios of 11.0 and 13.0, respectively) were significant risk factors

An unusual cause of cerebellar ataxia in an immunocompromised elderly patient.

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BACKGROUND Parvovirus B19 is a single-stranded DNA virus belonging to the family Parvoviridae, genus Erythrovirus. PVB19 infection is most common in the pediatric population, manifesting as erythema infectiosum. In patients with hemoglobinopathy, PVB19 infection is known to cause aplastic anemia.

Association of sickle cell disease, priapism, exchange transfusion and neurological events: ASPEN syndrome.

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Priapism and acute neurological events are believed to be unrelated complications of sickle cell hemoglobinopathy. We describe a syndrome based on our experience and a review of the literature of significant neurological events after partial exchange transfusion to treat priapism in sicklemic

Busulfan.

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OBJECTIVE To review the current published studies evaluating the pharmacokinetics, clinical efficacy, safety, and toxicity of busulfan in pediatric and adult patients. METHODS English-language literature published between 1953 and 1993 was analyzed; pertinent literature was

Pediatric Hypovitaminosis D: Molecular Perspectives and Clinical Implications.

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Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets,

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