VARIABILITY OF SICKLE-CELL DISEASE IN THE EASTERN PROVINCE OF SAUDI-ARABIA
VARIABILITY OF SICKLE-CELL DISEASE IN THE EASTERN PROVINCE OF SAUDI-ARABIA
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DOI：
10.1016/s0022-3476(89)80440-8
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发表时间：
1989-06-01
影响因子：
5.1
通讯作者：
NIAZI, GA
中科院分区：
文献类型：
--
作者：
ELMOUZAN, MI;ALAWAMY, BH;NIAZI, GA
镰状细胞病的临床和血液学特征在两组患者不同的祖先起源，但生活在沙特阿拉伯东部省，提出。28名来自西南地区的患者与相同数量的来自东部地区的患者进行性别、年龄和诊断匹配。所有患者在出生时都被诊断出该疾病，并定期进行随访研究。尽管出生和生活在相同的环境（东部省份），但西南地区的患者具有更严重的临床特征，血红蛋白水平持续较低，网状红细胞计数较高，提示溶血更严重。我们的结论是，在沙特阿拉伯东部省份，至少存在不同形式的镰状细胞病，并且儿童早期疾病的变异性更可能与遗传因素有关，而不是环境因素。
The clinical and hematologic features of sickle cell disease in two groups of patients of different ancestral origin, but living in the Eastern Province of Saudi Arabia, are presented. Twenty-eight patients of southwestern origin were matched for gender, age, and diagnosis with an equal number of patients of eastern origin. The disease was diagnosed in all patients at birth, and follow-up study was done on a regular basis. Despite being born and living in the same environment (Eastern Province), patients of southwestern origin had more severe clinical features, consistently lower hemoglobin levels, and higher reticulocyte counts, suggesting morre severe hemolysis. We conclude that there are at least distinct forms of sickle cell disease in the Eastern Province of Saudi Arabia and that the variability of the disease in early childhood is more likely related to genetic than to environmental factors.