title: [Liver and heart iron deposition status in patients with β thalassemia major: a multicenter study].
abstract: OBJECTIVE
  To observe the status of iron deposition in patient with β thalassemia major, and to formulate appropriate treatment strategies.
  
  
  METHODS
  The data of status of transfusion and chelation in 135 patients aged from 6 years and 4 months to 17 years and 11 months with β thalassemia major were collected and analyzed. Serum ferritin levels were determined and cardiac and hepatic iron deposition was determined using MRI T2(*) technology.
  
  
  RESULTS
  Of the 135 cases studied, 66 were male, and 69 were female, their average age was 12.1 years. Serum ferritin (SF) was determined for 111 cases, it varied from 1 086.8 µg/L to 15 011.5 µg/L. Among them, 16 cases had SF level <2 000 µg/L (14.5%) , in 41 cases SF were between 2 000 and 4 000 µg/L (36.0%) ;in 54 cases SF >4 000 µg/L (48.7%) . Liver MRI T2(*) results showed that in only 8 cases (5.9%) iron content in the liver was in normal range, 19 cases (14.9%) showed mild liver iron deposition;34 (25.2%) moderate and 74 (54.8%, the youngest one was only 6 years and 4 months of age) had severe iron deposition respectively. Cardiac MRI T2(*) showed that in 89 cases (65.9%) iron content in the heart was in normal range;19 cases (14.1%) had mild cardiac iron deposition and 27 (20.0%) presented severe iron deposition (the youngest one was only 9 years and 3 months of age) . SF level was obviously related to liver and cardiac iron deposition (MRI T2(*)) r and P value were -0.284, 0.003 and -0.374, 0.000 respectively. In 108 cases regular transfusion and chelation were delayed due to financial problem. The late and insufficient dosage administered and irregular chelation caused the higher SF level and the severe iron deposition.
  
  
  CONCLUSIONS
  The survival status of β thalassemia major in China is worrisome. Majority of them had not received regular transfusion and chelation. Liver and cardiac iron deposition occur early and had a high incidence.
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Changgang
authors.initials: C
authors.lastname: Li
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Sixi
authors.initials: S
authors.lastname: Liu
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Feiqiu
authors.initials: F
authors.lastname: Wen
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Hongying
authors.initials: H
authors.lastname: Gao
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Guangfu
authors.initials: G
authors.lastname: Chen
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Chunfu
authors.initials: C
authors.lastname: Li
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Xuedong
authors.initials: X
authors.lastname: Wu
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Jianpei
authors.initials: J
authors.lastname: Fang
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Wenge
authors.initials: W
authors.lastname: Hao
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Riyang
authors.initials: R
authors.lastname: Liu
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Xinhua
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: Chiuwing Winnie
authors.initials: CW
authors.lastname: Chu
authors.affiliation: Department of Hematology and Oncology, Shenzhen Children's Hospital, Shenzhen 518026, China.
authors.forename: WingYan
authors.initials: W
authors.lastname: Au
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000009:adverse effects / Q000627:therapeutic use; D019190:Iron Overload / Q000453:epidemiology* / Q000209:etiology; D008099:Liver / Q000378:metabolism*; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000378:metabolism*; D011859:Radiography; D012189:Retrospective Studies; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000628:therapy
chemical_list: D007502:Iron Chelating Agents; D005293:Ferritins; D007501:Iron