中国实用儿科杂志

中国实用儿科杂志

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小儿严重肝病并棘刺红细胞症7例临床分析

朱春江，李雄，张明，杨方源   

  1. 桂林医学院附属医院儿科，广西桂林 541001
  • 收稿日期:2005-11-20 修回日期:2006-03-06 出版日期:2006-05-06 发布日期:2006-05-06

Severe liver disease combined with spur cell anemia: a review of 7 cases.

Zhu Chunjiang,Li Xiong，Zhang Ming，et al.   

  1. Department of Pediatrics,Guilin Medical College Affiliated Hospital,Guilin 541001,China
  • Received:2005-11-20 Revised:2006-03-06 Online:2006-05-06 Published:2006-05-06

摘要： 目的探讨严重肝病并棘刺红细胞症的临床特点。 方法对2001年1月至2005年5月间桂林医学院附属医院儿科收治的7例严重肝病并棘刺红细胞症患儿的症状、体征，血细胞光镜检查，肝功能，血脂检查，预后，并结合文献资料进行总结分析。 结果7例中男4例，女3例，年龄5个月至9岁，主要临床表现有黄疸(6例)、出血(3例)、贫血(7例)、肝肿大(6例)或肝硬化(1例)、脾肿大(3例)，外周血中棘刺红细胞占所有红细胞的85%~944%，肝功能损害明显，血脂均有不同程度的改变。 结论严重肝病并棘刺红细胞症临床罕见，这7例患儿的共同特点是：(1)原发病各异；(2)病程长；(3)有程度不等的贫血，红细胞平均容积(MCV)以增大为主；(4)外周血中均有多少不等的棘刺红细胞；(5)肝功能损害明显；(6)血脂均有多项改变；(7)预后不良。

关键词: 严重肝病, 棘刺红细胞

Abstract: AbstractObjectiveTo explore the clinical characteristics of severe liver disease combined with spur cell anemia in 7 children. MethodsThe clinical data of 7 patients with such disease admitted to the pediatric ward in our hospital between January 2001 to March 2005 were analyzed.The data included symptoms、 signs、 laboratory examinations ( blood smear、 liver function test and serum cholesterol ) and the literatures,etc. ResultsSeven patients consisted of 4 males and 3 females and the age ranged from 5 months to 9 years.The children manifested as jaundice(6 cases)、 hemorrhage (3 cases)、anemia(7 cases)、large liver(6 cases) or cirrhosis(1 case) and megalospleen(3 cases).Blood smear showed that spur cell was 85%~9438% of the whole erythrocytes in peripheral blood.Liver functions were destroyed badly.Serum cholesterols were abnormal. ConclusionThe clinical characteristics of the children are summed up as:although the original disease might be different,the children are all characterized as long course、moderate to mild anemia、spur cell in peripheral blood、destroyed liver function、abnormal plasma cholesterol and bad prognosis.

Key words: Spur cell