Barkawi M, Bashir N, Sharif L
Pediatrics Department, Princess Basma Hospital, Irbid, Jordan.
Trop Geogr Med. 1991 Jan-Apr;43(1-2):94-9.
A family case report of sickle cell-thalassemia syndrome shows three problems concerning this disease. Firstly, the problem of differentiating sickle cell-thalassemia from the sickle cell disease; secondly, the wide range varieties in clinical picture; thirdly and finally contradiction in the criteria of prognosis. In this report sickle cell-thalassemia case was confirmed by constructing a hemoglobin electrophoresis pedigree for the patient. Clinical varieties and prognosis of sickle cell-thalassemia in this family are discussed.
一篇镰状细胞-地中海贫血综合征的家族病例报告显示了关于这种疾病的三个问题。首先,是将镰状细胞-地中海贫血与镰状细胞病区分开来的问题;其次,临床表现存在广泛的多样性;第三,也是最后一点,预后标准存在矛盾。在本报告中,通过为患者构建血红蛋白电泳谱系确诊了镰状细胞-地中海贫血病例。本文讨论了该家族中镰状细胞-地中海贫血的临床多样性及预后情况。