Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report about one case of left branchial arterial thrombosis in a 6-year-old child with thalassemia-sickle cell disease. In their opinion, although this hemoglobinopathy usually causes microthrombosis, its existence at the same time as that of the brachial thrombosis does not seem to be a coincidence. The actual origin of this disease remains to be found, as well as the role that thrombocytosis may play. The authors emphasize the relative rarity of thrombolytic arterial diseases in African Negroes and advise practitioners to recognize them, because their diagnosis, often established late in our climates, has severe consequences.
J Mal Vasc 1992
PMID:[Acute thrombosis of the brachial artery and beta-thalassemia-sickle cell anemia]. 161 6

We report a case of pulmonary hypertension (PH) in a 35-year old patient with beta-thalassemia major; he had commenced blood transfusions after the age of 4 years and had been splenectomised at the age of 6 years. PH clinical presentation was not uncommon. Hemodynamic study revealed precapillary PH with high cardiac output; vasodilators agents led to significant pulmonary responsiveness. In beta-thalassemia, whereas congestive heart failure is common and due to cardiac hemosiderin deposition, PH appears to be non rare but its etiopathogenic mechanism remain unclear and probably non univoqual. Hypoxemia as well as hemodynamic changes related to chronic anemia including increased pulmonary flow might play an important role. Management should include blood transfusions to correct anemia, the indication and the choice of vasodilator agents need to be evaluated.
Rev Mal Respir 2000 Jun
PMID:[Pulmonary hypertension in patient with beta-thalassemia major]. 1095 65