Alkindi Salam, Al Zadjali Shoaib, Al Madhani Ali, Daar Shahina, Al Haddabi Hamood, Al Abri Qamariya, Gravell David, Berbar Tsouria, Pravin Sahaya, Pathare Anil, Krishnamoorthy Rajagopal
Sultan Qaboos University Hospital, Muscat, Sultanate of Oman.
Hemoglobin. 2010 Jan;34(2):135-44. doi: 10.3109/03630261003677213.
To evaluate the incidence of hemoglobinopathies in Omani subjects and to forecast its future burden on health resources, we initiated a prospective neonatal screening program in two major cities of the Sultanate of Oman. Consecutive cord blood samples from a total of 7,837 neonates were analyzed for complete blood counts and for hemoglobin (Hb) profile by high performance liquid chromatography (HPLC). No case with Hb H (beta4) was detected. We observed that the overall incidence of alpha-thalassemia (alpha-thal) was 48.5% [based on the presence of Hb Bart's (gamma4)] and the beta-globin-related abnormalities accounted for 9.5% of the samples (4.8% sickle cell trait, 2.6% beta-thal trait, 0.9% Hb E trait, 0.8% Hb D trait, 0.08% Hb C trait, 0.3% sickle cell disease and 0.08% homozygous beta-thal). This is also the first large study to establish reference ranges of cord red blood cell (RBC) indices for Omani neonates.
为评估阿曼人群中血红蛋白病的发病率,并预测其未来对卫生资源的负担,我们在阿曼苏丹国的两个主要城市启动了一项前瞻性新生儿筛查项目。对总共7837名新生儿的连续脐带血样本进行了全血细胞计数分析,并通过高效液相色谱法(HPLC)分析了血红蛋白(Hb)谱。未检测到Hb H(β4)病例。我们观察到,α地中海贫血(α-thal)的总体发病率为48.5%[基于Hb Bart's(γ4)的存在],β珠蛋白相关异常占样本的9.5%(镰状细胞性状4.8%、β地中海贫血性状2.6%、Hb E性状0.9%、Hb D性状0.8%、Hb C性状0.08%、镰状细胞病0.3%和纯合β地中海贫血0.08%)。这也是第一项为阿曼新生儿建立脐带红细胞(RBC)指数参考范围的大型研究。