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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。
Objective To investigate the clinical features of severe liver disease and thorny cell syndrome. Methods From January 2001 to May 2005 in Guilin Medical College Hospital, pediatric patients admitted to seven cases of severe liver disease and children with thrombocytopenia symptoms, signs, blood cell light microscopy, liver function, blood lipids, prognosis, and combined Literature data analysis. Results There were 4 males and 3 females in 7 cases, ranging in age from 5 months to 9 years old. The main clinical manifestations were jaundice (6 cases), hemorrhage (3 cases), anemia (7 cases), hepatomegaly (6 cases) or Liver cirrhosis (1 case), splenomegaly (3 cases), peripheral blood thorn erythrocytes accounted for 8.5% to 94.4% of all red blood cells, liver damage, blood lipids have varying degrees of change. Conclusions Severe liver disease and thorny thrombocytopenia are rare in clinic. The common features of these seven children are: (1) different primary diseases; (2) longer duration; (3) varying degrees of anemia, mean corpuscular volume ) To increase the main; (4) There are varying degrees of peripheral blood thorn erythrocytes; (5) Liver damage was significant; (6) There are many changes in blood lipids; (7) The prognosis is poor.