目的 分析重型β-地中海贫血(β-TM)儿童的甲状腺功能与铁过载的关系,改善患儿的甲状腺功能。方法 2018年1—12月在珠海市妇幼保健院治疗的44例β-TM儿童,用化学发光法检测其游离T3(FT3)、游离T4(FT4)、促甲状腺素(TSH)、血清铁蛋白(SF),了解β-TM儿童甲状腺功能减退发生率、分析SF与TSH之间的关系。结果 44例β-TM患儿中甲状腺功能减退(PH)共0例,亚临床型甲状腺功能减退共6例(13.6%),SF与TSH之间呈正相关(r=0.399,P＜0.05),亚临床甲状腺功能减退患儿的SF水平[(5 933.00±2 405.41)μg/L]明显增高(t=-3.174,P＜0.05),不规范祛铁组患儿有较高的SF、TSH水平[(5 322.50±1 956.37)μg/L、(9.95±9.27)mU/ml]和亚临床型甲状腺功能减退发生率(33.3%)(t=6.186、2.678,χ2=8.273,P＜0.05)。结论 β-TM患儿甲状腺功能与SF、不规范祛铁有关,需规范祛铁防止甲状腺功能障碍。
Abstract
Objective To study the relationship between thyroid function and iron overload in children with β-thalassemia major(β-TM) and to improve the thyroid function. Methods From January to December 2018,44 children with β-TM were treated in Zhuhai Municipal Maternity and Child Health Care Hospital.Their free T3(FT3), free T4(FT4), thyrotropin(TSH) and serum ferritin(SF) were detected by chemiluminescence.The incidence of hypothyroidism in children was investigated and the relationship between SF and TSH was analyzed. Results Among 44 children with β-TM, there were 0 case of hypothyroidism and 6 cases of subclinical hypothyroidism(13.6%).There was a positive correlation between SF and TSH(r=0.399,P＜0.05).The level of SF [(5 933.00±2 405.41)μg/L]in subclinical hypothyroidism children was significantly increased(t=-3.174,P＜0.05).In irregular iron removal group,there were higher levels of SF,TSH[(5 322.50±1 956.37)μg/L,(9.95±9.27)mU/ml] and incidence of subclinical hypothyroidism(33.3%)(t=6.186,2.678,χ2=8.273,P<0.05). Conclusions Thyroid function in children with β-TM is related to SF and irregular iron removal.It is necessary to regulate iron removal to prevent thyroid dysfunction.
关键词
重型β-地中海贫血 /
儿童 /
甲状腺功能减退 /
铁过载
Key words
β-thalassemia major /
children /
hypothyroidism /
iron overload
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参考文献
[1] Telfer PT, Warburton F, Christou S,et al.Improved survival in thalassemia major patients on switching from desferrioxamine to combined chelation therapy with desferrioxamine and deferiprone[J].Haematologica,2009,94(12):1777-1778.
[2] De Sanctis V,Elsedfy H,Soliman AT,et al.Clinical and biochemical data of adult Thalassemia major patients(TM)with multiple endocrine complications(MEC)versus TM patients with normal endocrine functions:a long-term retrospective study(40 years) in a tertiary care center in Italy[J].Mediterr J Hematol Infect Dis,2016,8(1):e2016022.
[3] Bonifazi F,Conte R,Baiardi P,et al.Pattern of complications and burden of disease in patients affected by beta thalassemia major[J].Curr Med Res Opin,2017,33(8):1525-1533.
[4] 中华医学会儿科学分会血液学组.重型β地中海贫血的诊断和治疗指南[J].中华儿科杂志,2018,56(10):724-729.
[5] Angelucci E,Barosi G,Camaschella C,et al.Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders[J].Haematologica,2008,93(5):741-752.
[6] 杨锡强,易著文.儿科学[M].北京:人民卫生出版社,2006:489-493.
[7] De Sanctis V,Soliman A,Candini G,et al.High prevalence of central hypothyroidism In adult patients with β-thalasemia major[J].Georgian Med News,2013,222:88-94.
[8] Haghpanah S,Jelodari S,Ksramifar H,et al.The frequency of hypothyroidism and its relationship with HCV positivity in patients with thalassemia major in southern Iran[J].Acta Biomed,2018,89(1):55-60.
[9] Soliman AT,Al Yafei F,Al-Naimi L,et al.Longitudinal study on thyriod function in patients with thalassemia major:high incidence of central hypothyroidism by 18 years[J].Indian Endocrinol Metab,2013,17(6):1090-1095.
[10] Borgna-Pignatti C,Rugolotto S,De Stefano P,et al.Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine [J].Haematologica,2004,89(10):1187-93.
[11] De Sanctis V,Soliman A,Campisi S,et al.Thyroid disorders in thalassaemia:An update[J].Curr Trends Endocrinol,2012,6:17-27.
[12] Rindang CK,Batubara JRL,Amalia P,et al.Some aspects of thyroid dysfunction in Thalassemia major patients with severe iron overload[J].Paediatr Indones,2011,51:66-72.
[13] De Sanctis V, Soliman AT, Elsedfy H, et al.Growth and endocrine disorders in thalassemia:the international network on endocrine complications in thalassemia (I-CET) position statement and guidelines[J].Indian J Endocrinol Metab,2013,17:8-18.
[14] Eshragi P,Tamaddoni A,Zarifi K,et al.Thyriod function in major thalassemia patients:is it related to height and chelation therapy?[J].Caspian J Intern Med,2011,2(1):189-193.
[15] Sharma R,Seth A,Chandra J,et al.Endocrinopathies in adolescents with thalassemia major receiving oral iron chelation therapy[J].Paediatr Int Child Health,2016,36(1):22-27.
[16] 许吕宏,方建培,张亚停,等.重型β地中海贫血患儿体内铁负荷磁共振成像T2*检测的临床分析[J].中华妇幼临床医学杂志:电子版,2011,7(5):341-344.
[17] Angelucci E,Brittenham GM,McLaren CE,et al.Hepatic iron concentration and total body iron stores in thalassemia major[J].New Engl J Med,2000,343:327-331.
[18] 赵雪丽.桂西地区重型β地中海贫血儿童生长发育及铁过载状况的调查研究[D].南宁:广西医科大学,2015.
[19] Filosa A, Vitrano A, Rigano P, et al.Long-term treatment with deferiprone enhances left ventricular ejection function when compared to deferoxamine in patients with thalassemia major[J].Blood Cells Mol Dis,2013,51(2):85-88.
[20] 陈娟娟,陈光福,高红英.不同铁螯合剂治疗重型β地中海贫血铁过载的临床研究[J].中国小儿血液与肿瘤杂志,2017,22(4):185-189.
[21] Pepe A, Meloni A, Rossi G,et al.Cardiac and hepatic iron and ejection fraction in thalassemia major:multicentre prospective comparison of combined deferipro-ne and deferoxamine therapy against deferiprone or deferoxamine monotherapy[J].J Cardiov Magn Reson,2013,88(8):652-526.
[22] Haghpanah S,Zarei T,Zahedi Z,et al.Compliance and satisfaction with deferasirox (Exjade(R)) compared with deferoxamine in patients with transfusion-dependent beta-thalassemia [J].Hematology(Amsterdam,Netherlands),2014,19(4):187-191.
[23] Poggi M,Sorrentino F,Pugliese P,et al.Longitudinal changes of endocrine and bone disease in adults with β-thalasemia major receiving different iron chelators over 5 years[J].Ann Hamatol,2016,95(5):757-763.
[24] Kwiatkowski JL.Current recommendations for chelation for transfusion-depen- dent thalassemia[J].Ann N Y Acad Sci,2016,1368(1):107-114.
[25] 曾庆青.广西重型β地中海贫血患者疾病经济负担及对策研究[D].南宁:广西医科大学,2016.
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