Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Drepanocytosis is one of the qualitative hemoglobinopathies which are the most widespread in the word. In a retrospective study on 16 drepanocytary patients observed at the Bordeaux Children's hospital, 7 patients showed medullar hyperplasia, 10 patients showed vaso-occlusive, and 4 patients showed infections. Medullar hyperplasia: bone demineralisation was the current appearance (47%), cortical thinning down (40.5%). They touch long bone. Thrombosis: bone demineralisation was the current appearance (86.3%) and touch long bone, 1 first appearance right femoral head infarct. Some particular appearances we found. 2 cases of "hand foot syndrome", 1 case ischemic aseptic arthritis and 1 case of vertebra "step staircase". Three salmonella pandiaphysite and 1 tuberculosis spondylodiscite were observed. The authors analyse the major radiological appearances of this hemoglobinopathy and recall the interest in knowing this disease even in low endemicity countries.
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PMID:[Contribution of medical imaging in osteoarticular manifestations of sickle cell anemia in the child]. 1079 85