hemoglobinopathies/cefalee

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Regional cerebral blood flow in childhood headache.

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Regional cerebral blood flow (rCBF) was measured in 16 cranial regions in 23 children and adolescents with frequent headaches using the non-invasive Xenon-133 inhalation technique. Blood flow response to 5% carbon dioxide (CO2) was also determined in 21 patients, while response to 50% oxygen was

[Stroke in a child due to hemoglobinopathy AS].

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BACKGROUND Falciform cell anaemia is a genetically determined haemoglobinopathy which in homozygote form (HbSS) is accompanied by neurological disorders in a quarter of the patients, mainly in the form of cerebral ischemia. Some authors consider the heterozygote (HbAS) form to be asymptomatic,

[Splenic infarction at high altitude, Huaraz-Peru (3,100 masl)].

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We report three cases of splenic infarction in healthy men for the first time that amounted to high altitudes, observed in the hospital "Victor Ramos Guardia" Huaraz (3100 m). Case 1 (1995) of 55 years, born in Cuba, from Lima, caucasian suddenly presented acute abdominal pain in epigastrium,

Skull infarction and epidural hematomas in a patient with sickle cell anemia.

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OBJECTIVE Epidural hematomas are unusual manifestations of sickling disorders. We report a patient with sickle cell anemia and multiple skull infarctions associated with epidural hematomas. The association of skull infarctions and epidural hematomas in sickling hemoglobinopathies is

Erythrocytosis in a child due to Hb Andrew-Minneapolis [β144(HC1)Lys→Asn (AAG>AAT or AAC)] associated with a Spanish (δβ)(0)-thalassemia.

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We report a rare association of δβ-thalassemia (δβ-thal) and a hemoglobin (Hb) variant with high oxygen affinity in a Spanish newborn. The proband had no Hb A and showed microcytosis and hypochromia; the peripheral blood smear was compatible with a thalassemia trait. Molecular studies revealed that

Moyamoya syndrome associated with sickle cell trait in a child.

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Moyamoya syndrome is a chronic, progressive occlusion of cerebrovascular arteries that leads to the development of characteristic collateral vessels. It is usually accompanied with sickle cell disease and other hemoglobinopathies. We report a 7-year-old boy, who admitted to our clinic with headache,

Symptomatic erythrocytosis associated with a compound heterozygosity for Hb Lepore-Boston-Washington (δ87-β116) and Hb Johnstown [β109(G11)Val→Leu, GTG>TTG].

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Hb Johnstown [β109(G11)Val→Leu, GTG>TTG] has previously been described as a high oxygen affinity variant in a heterozygous state and in combination with β(0)-thalassemia (β(0)-thal). Because the variant does not separate from Hb A by routine methods it may be easily missed unless clinical suspicion

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

An Atypical Case of Methemoglobinemia due to Self-Administered Benzocaine.

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Acquired methemoglobinemia is an uncommon hemoglobinopathy that results from exposure to oxidizing agents, such as chemicals or medications. Although, as reported in the adult population, it happens most often due to prescribed medication or procedural anesthesia and not due to easily accessed

Phase I study of the orally administered butyrate prodrug, tributyrin, in patients with solid tumors.

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Butyrates have been studied as cancer differentiation agents in vitro and as a treatment for hemoglobinopathies. Tributyrin, a triglyceride with butyrate molecules esterified at the 1, 2, and 3 positions, induces differentiation and/or growth inhibition of a number of cell lines in vitro. When given

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