Cissé R, Wandaogo A, Tapsoba T L, Chateil J F, Ouiminga R M, Diard F
CHU de Ouagadougou, Burkina Fasor.
Dakar Med. 1999;44(1):40-4.
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.
镰状细胞贫血是世界上分布最广泛的定性血红蛋白病之一。在一项对波尔多儿童医院观察到的16例镰状细胞贫血患者的回顾性研究中,7例患者出现骨髓增生,10例患者出现血管闭塞,4例患者出现感染。骨髓增生:目前的表现为骨质脱矿(47%),皮质变薄(40.5%)。病变累及长骨。血栓形成:目前的表现为骨质脱矿(86.3%),病变累及长骨,1例首次出现右股骨头梗死。我们发现了一些特殊表现。2例“手足综合征”,1例缺血性无菌性关节炎和1例椎体“阶梯状”。观察到3例沙门氏菌全骨髓炎和1例结核性脊椎间盘炎。作者分析了这种血红蛋白病的主要放射学表现,并提醒人们即使在低流行国家也有必要了解这种疾病。