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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The effects of low doses of aspirin on the frequency and severity of painful vaso-occlusive crises were evaluated in children with sickle hemoglobinopathies. Aspirin was compared with placebo in 49 patients in a double-blind crossover study. Careful monitoring of patients revealed an average of 1.1 painful crises per patient year. During the 21 months of study, 70% of patients had a maximum of two painful crises, and 25% experienced four or more. The frequency and severity of crises were not affected by aspirin therapy. In view of aspirin's demonstrated effect on platelet function, we suggest that platelets do not contribute to the initiation or progression of the vaso-occlusive process.
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PMID:Trial of low doses of aspirin as prophylaxis in sickle cell disease. 684 40

Three cases studies from the Regional Hemoglobinopathy Laboratory at St. Joseph's Hospital in Hamilton are presented. These cases demonstrate the diagnostic value of a comprehensive hemoglobinopathy screen, family studies, and appropriate clinical information in solving complex hemoglobinopathy problems. The first case is a 79-year-old woman found to have small amounts of an unknown hemoglobin migrating electrophoretically in the position of hemoglobin S. The second case describes a boy of Greek extraction with a thalassemic blood picture but otherwise normal hemoglobinopathy screen. The third case involves a 70-year-old man with a previously normal hemoglobinopathy screen who subsequently showed evidence of alpha thalassemia.
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PMID:Hemoglobinopathies: three illustrative case studies. 786 51

Medroxyprogesterone acetate (MPA) - a safe depot contraceptive - is shown previously to reduce painful crises of sickle cell anemia, which is parallel with the recent findings showing progesterone induction of fetal hemoglobin genes. This would be a way to reduce transfusions for late term thalassemia major and sickle cell-disease cases with no chances left for a stem cell transplantation. In these patients, transfusional hemosiderosis causes irreversible damage to many organs despite the available iron-chelating agents. Pharmacological strategies either target the conformal structure of the defective adult hemoglobin or aim to activate fetal hemoglobin concentrations. The only concern on MPA may be its thromboembolic risks, which may be uncoupled with agents acting both anti-coagulant and inductive on the blood oxygen-carrying affinity. Such agents could be valproic acid and aspirin. Valproic acid is being safely used to treat epilepsy and its histone acetylating function may lead its induction of fetal hemoglobin. Aspirin was shown to increase oxygen affinity of hemoglobin via acetylating lysine residues and its general acetylating activity on proteins such as histones makes it also an interesting candidate to activate fetal hemoglobin. We propose that combining MPA with clinically available doses of valproic acid and aspirin would be beneficial in terms of both reduced coagulation risks and increased oxygen affinity to decrease the transfusions and to improve the prognosis in late-phase hemoglobin disorders.
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PMID:Medroxyprogesterone - valproic acid - aspirin. MVA regime to reduce transfusion associated mortality in late-term hemoglobinopathies. Hypothesis and rationale. 1716 47