儿童遗传性球形红细胞增多症8例临床分析  

Clinical Analysis of Eight Children with Hereditary Spherocytosis

作　　者：顾岚[1] 蒋瑾瑾[1] 

机构地区：[1]解放军第二军医大学附属长海医院儿科,上海200433

出　　处：《临床军医杂志》2005年第5期585-586,共2页Clinical Journal of Medical Officers

摘　　要：目的分析儿童遗传性球形红细胞增多症的临床特点。方法对第二军医大学附属长海医院儿科1998年1月—2004年6月收治的8例儿童遗传性球形红细胞增多症临床资料进行回顾分析。结果8例遗传性球形红细胞增多症患儿临床症状和体征中以尿色深、皮肤黄染和脾大常见;入院前有1例诊断不明,7例误诊;均经过补铁、输血和激素治疗;部分性脾栓塞、脾切除疗效显著。结论儿童遗传性球形红细胞增多症误诊率高,如临床上发现有不明原因的反复溶血的患儿,尤其是有相关家族史或常规治疗无效者,应尽快到有检测条件的医院检查,以便获得正确的治疗。Objective To analyze the clinical feature of children hereditary spherocytosis （HS）. Methods Retrospectively. analyzing the data of 8 cases with HS from janl, 1998 to Sep Results In the eight cases of HS, the most common signs were jaundice and hepatosplenomegaly. One case did not have diagnostically clear results, and seven ones were misdiagnosed. All were treated with iron supply, blood transfusion and hormone therapy. Partial splenic embolisation and spleneetomy were quite effective on HS. Conclusion HS has a high possibility of misdiagnosis. When doctors find that haemolytic anemia, whose causes are unknown, continually occurs in the patients, they should be sent to hospital with qualifled equipment for physical examination as soon as posssible in order to get correct diagnosis and treatment.

关 键 词：儿童 遗传性球形红细胞增多症 溶血性贫血 

分 类 号：R559[医药卫生—血液循环系统疾病]