Clinical and haematological diversity of sickle cell disease in Saudi children.
Clinical and haematological diversity of sickle cell disease in Saudi children.
复制标题
沙特儿童镰状细胞病的临床和血液学多样性。
作者：
M. El
镰状细胞病(SCD)表现为从轻微疾病到丧失工作能力的广泛的临床行为。在这项研究中，我们试图调查沙特阿拉伯不同地区SCD的临床多样性。对来自东部省份的41名SCD儿童的血液学参数和临床表现进行了比较，并与来自西南部省份(SWP)的51名儿童的结果进行了比较。东部省份患者的严重程度指数(SI)为2~11，平均为4.5；西南部省份患者的SI为2~18，平均为9.5。此外，手足综合征、血管闭塞危象的发生、需要输血和住院次数的增加也使西南地区SCD患者的临床表现与东部地区的SCD患者有所不同。西南部省份的SCD儿童的红细胞水平、总血红蛋白和红细胞压积较低。Hb A2水平显著升高，而Hb F、红细胞压积(PCV)、平均红细胞体积(MCV)和平均红细胞血红蛋白浓度(MCHC)无显著差异。HBF水平对SWP患者的血液学指标无明显影响。结论：沙特人群中的SCD在临床和血液学上具有异质性。
Sickle cell disease (SCD) exhibits itself in a broad spectrum of clinical behaviour ranging from a mild disease to an incapacitating condition. In this study, we have attempted to investigate the clinical diversity of SCD in different regions of Saudi Arabia. The results of haematological parameters and clinical manifestations in 41 children with SCD from the eastern province where the disease is mild, were compared with results obtained in 51 children from the south-western province (SWP), where the disease has been shown to be more severe. The severity index (SI) of patients from the eastern province ranged from 2 to 11 with a mean of 4.5 and in patients from the south-western province, the SI ranged from 2 to 18 with a mean of 9.5. In addition, the occurrence of hand and foot syndrome, vaso-occlusive crisis, and increased frequency of requirement of blood transfusion and hospitalization differentiated the clinical presentation of SCD in the patients from south-western province from those in the eastern province. Red blood cell level, total haemoglobin and packed cell volume were lower in the SCD children from the south-western province. Haemoglobin A2 level was significantly higher, while haemoglobin F, packed cell volume (PCV), mean corpuscular volume (MCV), and mean corpuscular haemoglobin concentration (MCHC) did not show any significant differences. HbF level did not influence the haematological parameters significantly in the SWP patients. It is concluded that the SCD in Saudi population is heterogeneous clinically and haematologically.