| Objective To study the general growth and development status and gonadal hormone levels of patients with severe thalamic-thalamic-gonadal anemia,and to understand the hypothalamic-pituitary-gonadal axis status of thalamic-thalamic-gonadal axis status in patients with severe thalamic-thalamic-gonadal axis status.To study the iron overload status in these children,and to explore whether iron overload has any effect on the hypothalamic-pituitary-gonadal axis.Methods This study collected and analyzed clinical cases of severe beta thalassaemia in children diagnosed by genotyping in the department of hematology,the first affiliated hospital of guangxi medical university from January 2012 to January 2017.A total of 91 children(48 males and 43 females,2-20 years old)with transfusion dependent severe beta thalassemia were excluded,and 63 children were selected as non-thalassemia group(33 males and 30 females,2-17 years old).Growth and development(height,weight,GH)and gonadal hormone levels(FSH,LH,testosterone,estradiol)between the two groups were analyzed,as well as iron overload and sex hormone levels before and after puberty in the thalassemia group.Results 1)Compared with the non-thalassaemia group,the height and weight of patients with thalassemia major were lower.Ages in the < 8 stage,34 cases of male β-thalassemia major 4 cases(11.76%)of patients with height is lower than the average height of 2 sd,4 cases(11.76%)patients with weight is lower than the average weight of 2 sd,30 cases of female β-thalassemia major patients have 8 cases(26.67%)was lower than the average height of 2 sd,6patients(20.00%),the weight is lower than the average weight of 2 sd,the non-thalassaemia group of 17 cases of men in 2 cases(11.76%)height is lower than the average height of 2 sd,Short stature was observed in 2(14.29%)of the14 women in the non-thalassemia group,and low body weight was not observed in the non-thalassemia group.When age <8 years,there was no significant difference in the incidence of short stature and low body weight between theβ-thalassemia major and the non-thalassemia group.At age ≥8 years,10(71.43%)of the 14 male patients withβ-thalassemia major presented with short stature and 4(28.57%)with low-birth-weight 。 Short stature was found in 7(53.85%)and 5(38.46%)of the 13 women with thalassemia major,and 3(18.75%)of the 16 men in the non-thalassemia group and 5(31.25%)of the 16 women in the non-thalassemia group.No low body weight was found in the non-thalassemia group.When age ≥8 years,the incidence of short stature and low-birth-weight was higher in men in theβ-thalassemia major group compared with those in the non-thalassemia group(p<0.05).2)Among the 91 cases ofβ-thalassemia major children,57 cases(62.6%)were in the state of severe iron overload.3)When age <8 years,GH levels in the β-thalassemia major group were lower than those in the non-thalassemia group(P < 0.05).When the age was ≥8 years old,GH,FSH and LH levels showed that theβ-thalassemia major group was lower than the non-thalassemia group(P <0.05).There was no significant difference between the TESTO level of the maleβ-thalassemia major group and the non-thalassemia group at different ages(P>0.05).Comparison of GH,FSH and LH in thalassemia major men of different ages with those in non-thalassemia group.When <8 years old,GH and LH in the male β-thalassemia major group were lower than those in non-impoverished group.When ≥8 years old,GH,FSH and LH in the maleβ-thalassemia major were lower than those in the non-thalassemia group.There was no difference in the mean ESTRDL hormone level between women of different age groups in theβ-thalassemia major group and the non-thalassemia group(P>0.05).Compared with the non-thalassemia group,when the age was <8 years old,the GH level in the thalassemia major female group was lower than that in the non-thalassemia female group.When the age was ≥8 years old,GH,FSH and LH in the female thalassemia major group were significantly lower than those in the non-thalassemia group.5)In terms of TESTO hormone levels,there was no difference in the average TESTO levels of men with heavy iron overload in different age groups and those without heavy iron overload.Compared with the non-heavy iron overload group,when the age was <8 years old,the GH of the male heavy iron overload group was lower than that of the non-heavy iron overload group.When age ≥8 years old,there were no differences in GH,FSH and LH levels between the two groups。In terms of ESTRDL hormone levels,there was no difference in average ESTRDL levels between women with heavy iron overload and those without heavy iron overload in different age groups.There was no significant difference in GH,FSH and LH in different age groups between the heavy iron overload group and the non-heavy iron overload group.Conclusion 1)Most children with thalassemia are short and have low gonadal hormone levels in adolescence.Early monitoring,early diagnosis,early intervention and early treatment of the hypothalamic-pituitary-gonadal axis and growth and development in patients with thalamic anemia are recommended.2)High incidence of severe iron overload in patients with severe thalassemia.Early use of iron dispelling therapy and early clinical intervention are recommended.3)We no significant correlation between iron overload and gonadal hormone levels was observed in patients with β-thalassemia major in this study. |