目的:探讨换血术对高胆红素血症新生儿血液内环境的影响。方法:33例采用外周动静脉同步换血术,19例采用O型红细胞+AB型血浆,14例采用新鲜的(3 d内)与患儿同型ACD全血,平均换血量156.9±30.4 m l/kg。检测换血前中后血常规、血气、...目的:探讨换血术对高胆红素血症新生儿血液内环境的影响。方法:33例采用外周动静脉同步换血术,19例采用O型红细胞+AB型血浆,14例采用新鲜的(3 d内)与患儿同型ACD全血,平均换血量156.9±30.4 m l/kg。检测换血前中后血常规、血气、电解质、血糖,计量资料用t检验对比分析。结果:换血后可出现贫血,白细胞及血小板均明显下降(P<0.001),而且在换血中已显著下降(P<0.001)。血电解质方面,血钾显著下降,磷也有明显降低。血糖的改变为双向性。血气除PaCO2有一过性显著升高,其余pH、PaO2、H2CO3均无明显差异(P>0.05)。结论:该组换血资料提示:换血对新生儿特别是早产儿容易造成白细胞及血小板明显下降而发生感染或出血。如使用较新鲜血,换血后往往出现血钾下降甚至低血钾症,造成糖代谢紊乱是双向性的,经常规补碱后不易出现代谢性酸中毒。展开更多
BACKGROUND Erythropoietic protoporphyria(EPP)is a rare genetic disorder stemming from ferrochelatase gene mutations,which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes,skin,bone marrow ...BACKGROUND Erythropoietic protoporphyria(EPP)is a rare genetic disorder stemming from ferrochelatase gene mutations,which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes,skin,bone marrow and liver.Although porphyria-related severe liver damage is rare,its consequences can be severe with limited treatment options.CASE SUMMARY This case study highlights a successful intervention for a 35-year-old male with EPP-related liver impairment,employing a combination of red blood cell(RBC)exchange and therapeutic plasma exchange(TPE).The patient experienced significant symptom relief and a decrease in bilirubin levels following multiple PE sessions and an RBC exchange.CONCLUSION The findings suggest that this combined approach holds promise for managing severe hepatic impairment in EPP.展开更多
文摘目的:探讨换血术对高胆红素血症新生儿血液内环境的影响。方法:33例采用外周动静脉同步换血术,19例采用O型红细胞+AB型血浆,14例采用新鲜的(3 d内)与患儿同型ACD全血,平均换血量156.9±30.4 m l/kg。检测换血前中后血常规、血气、电解质、血糖,计量资料用t检验对比分析。结果:换血后可出现贫血,白细胞及血小板均明显下降(P<0.001),而且在换血中已显著下降(P<0.001)。血电解质方面,血钾显著下降,磷也有明显降低。血糖的改变为双向性。血气除PaCO2有一过性显著升高,其余pH、PaO2、H2CO3均无明显差异(P>0.05)。结论:该组换血资料提示:换血对新生儿特别是早产儿容易造成白细胞及血小板明显下降而发生感染或出血。如使用较新鲜血,换血后往往出现血钾下降甚至低血钾症,造成糖代谢紊乱是双向性的,经常规补碱后不易出现代谢性酸中毒。
文摘BACKGROUND Erythropoietic protoporphyria(EPP)is a rare genetic disorder stemming from ferrochelatase gene mutations,which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes,skin,bone marrow and liver.Although porphyria-related severe liver damage is rare,its consequences can be severe with limited treatment options.CASE SUMMARY This case study highlights a successful intervention for a 35-year-old male with EPP-related liver impairment,employing a combination of red blood cell(RBC)exchange and therapeutic plasma exchange(TPE).The patient experienced significant symptom relief and a decrease in bilirubin levels following multiple PE sessions and an RBC exchange.CONCLUSION The findings suggest that this combined approach holds promise for managing severe hepatic impairment in EPP.
