beta-thalassemia/seizures

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Arterial ischemic stroke in a child with beta-thalassemia trait and methylentetrahydrofolate reductase mutation.

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Genetic and acquired disorders that foster a procoagulable state represent risk factors for stroke in childhood. Although an increased incidence of thromboembolic complications has been reported in patients with thalassemia, severe cerebral thromboembolism has rarely been observed in patients with

Antiphospholid antibody syndrome and Hb E/Beta thalassemia disease post-allogeneic stem cell transplantation.

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We report a 10-year-old male with Hb E/Beta thalassemia disease who developed chronic graft-versus-host disease (cGVHD) of antiphospholipid antibody syndrome after successful allogeneic stem cell transplantation (SCT). He exhibited a recurrent ischemic stroke on day 368 post-SCT while on

Beta-thalassemia major complicated by intracranial hemorrhage and critical illness polyneuropathy.

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Intracranial hemorrhage (ICH) is rarely seen in patients with thalassemia. A seven-year-old male, known case of beta-thalassemia major, on irregular packed cell transfusions (elsewhere) and non-compliant with chelation therapy, presented with congestive cardiac failure (Hb-3 gm/dl). He received

Generalized tonic-clonic seizures in a thalassemic patient with hypoparathyroidism and brain calcinosis.

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Acquired hypoparathyroidism (HPT) is a not uncommon complication in patients with b-thalassemia major. The insufficient production of parathyroid hormone is mainly due to iron overload in parathyroid glands. We report a 22-year-old female thalassemic patient referred to our Unit for hypogonadism.

Hypoparathyroidism with extensive intracerebral calcification in patients with beta-thalassemia major.

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We report marked intracerebral calcification in eight thalassemic patients with hypoparathyroidism, followed regularly at the Haematology Research Center, Department of Pediatrics, Shiraz, Iran. Their mean age was 16.8 years (range 12-21 years). Six of the eight patients with thalassemia were

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

Novel valproic acid derivatives with hemoglobin F inducing activity.

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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.

Diffuse intracerebral calcification in a beta-thalassaemia major patient with hypoparathyroidism: a case report.

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Beta-thalassemia major is a complex medical problem found worldwide. Endocrinopathies are some of the most frequent dysfunctions found in these patients. Iron overload in different organs is responsible for multiple endocrine complications particularly in the absence of adequate chelation therapy.

Status epilepticus amauroticus and posterior reversible encephalopathy syndrome in children.

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BACKGROUND Transient cortical visual impairment is a common aura of occipital lobe seizures. However, status amauroticus for hours has rarely been described as a manifestation of occipital lobe status epilepticus. OBJECTIVE To describe status amauroticus caused by focal status epilepticus of

Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease.

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OBJECT Pediatric patients with sickle cell disease (SCD) and moyamoya syndrome (MMS) are at significant risk for cerebrovascular accidents despite chronic transfusion therapy. Encephaloduroarteriosynangiosis (EDAS) and encephalomyoarteriosynangiosis (EMAS) are additional therapeutic options for

High incidence of severe cyclosporine neurotoxicity in children affected by haemoglobinopaties undergoing myeloablative haematopoietic stem cell transplantation: early diagnosis and prompt intervention ameliorates neurological outcome.

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BACKGROUND Neurotoxicity is a recognized complication of cyclosporine A (CSA) treatment. The incidence of severe CSA-related neurological complications following hematopoietic stem cell transplantation (HSCT) is 4-11%. METHODS We describe 6 cases of CSA related neurotoxicity out of 67 matched

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