de Gheldere Antoine, Ndjoko Roger, Docquier Pierre-Louis, Mousny Maryline, Rombouts Jean-Jacques
Department of Orthopaedic Surgery, Cliniques universitaires Saint-Luc, UCL, Brussels, Belgium.
Acta Orthop Belg. 2006 Dec;72(6):741-7.
Sickle cell disease is the most frequent haemoglobinopathy in the world. It affects mostly African descent, but is also present in whites in Greece, Turkey, Italy and India. The responsible gene is autosomal co-dominant and only individuals homozygous for the gene are symptomatic. The condition is characterised by haemolytic anaemia crises and cardio-pulmonary, digestive, neurological, ocular and osteo-articular manifestations. Osteo-articular complications are frequent and may compromise harmonious growth. This retrospective study reports the osteo-articular complications associated with sickle-cell disease encountered in our institution from 1975 to 2004. Orthopaedic complications were reported in 79 patients out of 325 who were followed with sickle-cell disease.
镰状细胞病是世界上最常见的血红蛋白病。它主要影响非洲裔人群,但在希腊、土耳其、意大利和印度的白人中也有出现。致病基因是常染色体共显性的,只有该基因的纯合个体才会出现症状。该病的特征是溶血性贫血危象以及心肺、消化、神经、眼部和骨关节表现。骨关节并发症很常见,可能会影响正常生长发育。这项回顾性研究报告了1975年至2004年在我们机构中遇到的与镰状细胞病相关的骨关节并发症。在325例接受镰状细胞病随访的患者中,有79例报告了骨科并发症。