Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematolog...Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematological indices. This study was designed to determine prevalence of minor and silent carries parents that have children suffering from major β-thalassemia, compare it with national protocol about prevention of thalassemia. Methods: A blood sample was taken from parents of all major thalassemic patients covered by TaleghaniHospitalin Gorgan (n = 195), CBC and Hemoglobin electrophoresis were done. Data were analyzed using SPSS software. Results: Amongst 196 parents one case have normal level of MCV, MCH, RBC, HbA, HbF and mentzer = 22.05 (0.51%) that diagnosed as alpha triplication/N by real time PCR, RFLP informative. The means of hematological indices were based on the national protocol. Conclusion: Present results showed that there are a few cases of thalassemia disorders with normal MCV, MCH, RBC, Mentzer index and Hb electrophoresis which could be missed in routine and pre-marital screening tests, resulted in a thalassemia child that it is possible in every screening test. Generally, indices were according to range of the national guideline.展开更多
文摘Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematological indices. This study was designed to determine prevalence of minor and silent carries parents that have children suffering from major β-thalassemia, compare it with national protocol about prevention of thalassemia. Methods: A blood sample was taken from parents of all major thalassemic patients covered by TaleghaniHospitalin Gorgan (n = 195), CBC and Hemoglobin electrophoresis were done. Data were analyzed using SPSS software. Results: Amongst 196 parents one case have normal level of MCV, MCH, RBC, HbA, HbF and mentzer = 22.05 (0.51%) that diagnosed as alpha triplication/N by real time PCR, RFLP informative. The means of hematological indices were based on the national protocol. Conclusion: Present results showed that there are a few cases of thalassemia disorders with normal MCV, MCH, RBC, Mentzer index and Hb electrophoresis which could be missed in routine and pre-marital screening tests, resulted in a thalassemia child that it is possible in every screening test. Generally, indices were according to range of the national guideline.
文摘目的探讨红细胞指数用于鉴别缺铁性贫血(iron deficiency anemia,IDA)与地中海贫血特质(thalassemia trait,TT)患者的应用价值和最佳界值。方法选取2017年10月1日至2021年3月31日北京和睦家医院确诊为IDA或TT的中国患者40例。根据血常规结果,计算8种红细胞指数,分析其对于鉴别IDA或TT的灵敏度与特异度、约登指数,根据ROC探讨其在我国患者中的适合界值。结果40例患者中有22例IDA和18例TT。Jayabose、Green and King和Ricerca 3种红细胞指数用于鉴别IDA和TT的约登指数分别为0.667、0.667和0.682,ROC的AUC分别为0.990、0.995和0.992,均为十分可靠的指标。同时,Jayabose、Green and King和Ricerca 3种红细胞指数的最适合界值分别为251.69、79.56和3.91。调整界值后,Jayabose、Green and King和Ricerca 3种红细胞指数鉴别IDA和TT的准确率均提高至97.5%。结论含有红细胞分布宽度指标的红细胞指数对于鉴别我国小细胞低色素性贫血患者的病因具有很高的应用价值,值得临床推广。
