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质量问题——重型地中海贫血中造血干细胞移植与输血及螯合疗法的比较

Quality Matters - Hematopoietic Stem Cell Transplantation versus Transfusion and Chelation in Thalassemia Major.

作者信息

Patel Shivani, Swaminathan Venkateswaran Vellaichamy, Mythili V Sr, Venkatadesikalu M Sr, Sivasankaran Meena, Jayaraman Dhaarani, Balasubramaniam R, Uppuluri Ramya, Raj Revathi

机构信息

Pediatric Hematology and Blood and Marrow Transplantation, Apollo Speciality Cancer Hospital, Chennai, India. Correspondence to: Dr Shivani Patel, Apollo Cancer Institute, 320 - Anna Salai, Teynampet Chennai 600 035, India.

Pediatric Hematology and Blood and Marrow Transplantation, Apollo Speciality Cancer Hospital, Chennai, India.

出版信息

Indian Pediatr. 2018 Dec 15;55(12):1056-1058.

PMID:30745477
Abstract

OBJECTIVE

To compare quality of life of children with thalassemia major who have undergone stem cell transplantation with those on regular transfusion.

METHODS

The study included 40 children who underwent transplantation and 40 children and 20 adults on regular transfusion and iron chelation therapy. The quality of life assessment was done using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale.

RESULTS

The mean total summary score, psychosocial summary score and physical score was 92, 91 and 92.8, respectively in transplant group and 83, 82.7 and 83.6, respectively in children in transfusion group. The adult group on transfusion showed overall poorer scores of 74.9, 76 and 73.9, respectively. The average scores in all domains were significantly (P<0.05) lower and drop steeply in second decade in transfusion group.

CONCLUSIONS

Allogeneic stem cell transplantation improves quality of life in thalassemia major.

摘要

目的

比较接受干细胞移植的重型地中海贫血患儿与接受常规输血治疗的患儿的生活质量。

方法

该研究纳入了40名接受移植的儿童以及40名接受常规输血和铁螯合治疗的儿童和20名成人。使用儿童生活质量量表4.0通用核心量表进行生活质量评估。

结果

移植组的平均总综合得分、心理社会综合得分和身体得分分别为92、91和92.8,输血组儿童的相应得分分别为83、82.7和83.6。输血的成人组总体得分较低,分别为74.9、76和73.9。输血组所有领域的平均得分均显著较低(P<0.05),且在第二个十年中急剧下降。

结论

异基因干细胞移植可改善重型地中海贫血患者的生活质量。

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