Clinical Case Analysis Of Thalassemia Intermedi

Objective: To understand the iron overload and gene distribution of patients with intermediate thalassemia in this region;The relationship between iron overload and liver function,hematological parameters and calcium and phosphorus metabolism was analyzedα、β Clinical characteristics of intermediate thalassemia.Methods: Using the case management system and outpatient case collection of the Affiliated Hospital of Youjiang Medical College for Nationalities,47 patients with thalassemia intermedia who met the inclusion and exclusion criteria in the Affiliated Hospital of Youjiang Medical College from 2016 to 2021 were used to collect the liver function of the inpatients.Related indexes(alanine aminotransferase,aspartate aminotransferase,total bilirubin,direct bilirubin,indirect bilirubin,γ-glutamyl transpeptidase),related indexes of hematological parameters(hemoglobin,MCH,MCV),blood calcium,blood phosphorus,and the general condition of the patient,thalassaemia gene,hemoglobin electrophoresis results and other indicators.Forty-seven patients with thalassemia intermedia who met the inclusion criteria were divided into iron overload group(ferritin greater than 1000ng/m L)and non-iron overload group(ferritin less than 1000ng/m L).Phosphorus metabolism.To further explore the relationship between liver function-related indicators,degree of anemia,blood calcium and blood phosphorus and iron overload in patients with thalassemia intermedia,and compare serum ferritin,blood cell parameters,liver function,blood pressure and iron overload under different genotypes(α and β thalassemia intermedia).Whether there are differences in calcium,blood phosphorus,etc.,parallel Pearson correlation analysis between serum ferritin(SF)and alanine aminotransferase,aspartate aminotransferase,total bilirubin,γ-glutamyl transpeptidase,hematological parameters,blood calcium,blood The correlation between phosphorus and age,and in addition,according to the different ways of causing α-thalassemia intermedia gene mutation,it can be divided into deletion Hb H disease and non-deletion hemoglobinopathy,and compare the serum ferritin,blood cell parameters,liver function between the two groups,blood calcium,blood phosphorus and other differences.Results:(1)Among the 47 cases of Ti,the ethnic groups were mainly Zhuang nationality(31 cases)(66%),Han nationality(9 cases)(19.1%),Buyi Nationality(5 cases)(10.6%),Yao nationality(2 cases)(4.3%),which were divided into 38 intermediate types α Thalassemia and 9 cases of intermediate type β Thalassemia,38 cases of intermediate type α There are three genotypes in thalassemia,non deletion genotype:-α CS /--sea 25 cases(65.8%),deletion genotype:-α 3.7 /--sea 8 cases(21.1%)-α 4.2 /--sea 5 cases(13.1%).(2)In the study of 47 cases of Ti,liver function related indexes(alt,AST,TBIL,DBIL,ibil γ-GT)was significantly higher than that of non iron overload group(p<0.05).Anemia in iron overload group was more severe than that in non iron overload group(p<0.05),MCH in iron overload group was less than that in non iron overload group(p>0.05),and MCV in iron overload group was greater than that in non iron overload group(p<0.05).Ca in non iron overload group was higher than that in iron overload group,the difference was not statistically significant(p>0.05).P in non iron overload group was higher than that in iron overload group,the difference was statistically significant(p<0.05).(3)In 47 cases of Ti,β-The related indexes of liver function in Ti group were lower than those in HBH group(p<0.05).β-The Hb and MCH of Ti were higher than those of HBH(p<0.05)β-MCV of Ti was lower than that of HBH(p>0.05).β-There was no significant difference in CA between Ti and HBH(p>0.05)β-The P of Ti was higher than that of HBH(p<0.05).(4)Among the 38 cases of HBH,the MCV of non deletion type HBH was significantly higher than that of deletion type HBH(p<0.05),but there was no significant difference between Hb and MCH(p>0.05).Non deletion SF was higher than deletion SF,and the difference was not statistically significant(p>0.01)There was no significant difference in Ca and P between deletion type and non deletion type HBH group(p>0.05),and the related indexes of liver function(alt,AST,TBIL,DBIL,ibil γ-GT),the difference was not statistically significant(p>0.05)(5)SF and age,TBIL,DBIL,ibil,alt,AST γ-GT and MCV were significantly positively correlated(r=0.459,0.481,0.532,0.442,0.692,0.586,0.685)(p<0.01),and negatively correlated with HB(r=-0.382)(p<0.01),but had no significant statistical significance with blood Ca,blood p and MCH(p>0.01);Conclusions: 1.iron overload often causes a certain degree of liver function damage in patients with intermediate thalassemia.There are differences in hematological parameters between iron overload group and non iron overload group in patients with intermediate thalassemia.Compared with non iron overload group,iron overload group has heavier anemia and larger hemoglobin volume.2.in this region,non deletion type is the main type of Hb H disease,and the degree of anemia and iron overload is greater than that of deletion type.Non deletion type of Hb H disease is mainly-α CS /--sea is the main disease,while deletion type of HBH is the main disease-α 3.7 /--sea genotype is dominant.