ライフサイエンスコーパス: thalassemia major

1 condition as severe as Cooley's anemia (beta-thalassemia major).

2 re reviewed including 11 (41%) children with thalassemia major.

3 progenitor cells (HPCs) in adults with beta-thalassemia major.

4 ents in the management of patients with beta-thalassemia major.

5 s between basic and clinical studies of beta-thalassemia major.

6 dy-state bone marrow from patients with beta-thalassemia major.

7 iac iron overload causes most deaths in beta-thalassemia major.

8 esonance for heart failure and arrhythmia in thalassemia major.

9 cal severity of sickle cell disease and beta-thalassemia major.

10 rload are the leading cause of death in beta-thalassemia major.

11 ng therapeutic option for subjects with beta-thalassemia major.

12 e now have created an adult model of beta(0)-thalassemia major.

13 acterized by a severe, transfusion-dependent thalassemia major.

14 31 chronically transfused patients with beta-thalassemia major and collected samples immediately befo

15 Conclusion ECV is significantly increased in thalassemia major and is associated with myocardial iron

16 and arrhythmia from myocardial siderosis in thalassemia major and is superior to serum ferritin and

17 ion therapy can rescue mice affected by beta-thalassemia major and modify both the absorption and dis

18 leading cause of death for young adults with thalassemia major and related disorders has been cardiac

19 Transfusional therapy for thalassemia major and sickle cell disease can lead to ir

20 g cardiac magnetic resonance (MR) imaging in thalassemia major and to investigate the relationship be

21 curred in the second decade in patients with thalassemia major, and this experience has driven expect

22 Sickle cell disease and beta-thalassemia major are clinically significant hereditary

23 endent (TD) hereditary anemias such as alpha-thalassemia major are incontrovertible, the evidence sup

24 ion factor-15 (GDF-15) in patients with beta-thalassemia major before and after transfusion, in the c

25 beta-Thalassemia major causes ineffective erythropoiesis and

26 A 34-year-old man with thalassemia major complained of nyctalopia and decreased

27 H disease, beta-thalassemia intermedia, beta-thalassemia major/Cooley's anemia).

28 Hepcidin levels in patients with beta-thalassemia major dynamically reflect competing influenc

29 al incidence of melioidosis in children with thalassemia major from 2001 to 2010 was 140 per 100 000/

30 transplanted more than 20 years ago for beta-thalassemia major had a different health-related quality

31 The experience of HCT for persons with beta-thalassemia major has been successfully extended to SCD.

32 Treatment of patients with beta-thalassemia major has improved dramatically during the p

33 e previously described animal models of beta thalassemia major, homozygous gammabeta(0) mice switch f

34 to manage iron overload in diseases such as thalassemia major; however, the use of conventional iron

35 We report here the correction of alpha-thalassemia major hydrops fetalis in transgene-free iPS

36 n controlling iron overload in patients with thalassemia major is highly variable and may partly depe

37 roval and informed consent, 30 patients with thalassemia major (mean age +/- standard deviation, 34.6

38 e patients (sickle cell anemia, n = 32; beta-thalassemia major, n = 6; and bone marrow failure, n = 5

39 nts included malignancy, sickle cell anemia, thalassemia major, nonmalignant hematological conditions

40 A preclinical humanized mouse model of beta thalassemia major or Cooley anemia (CA) was generated by

41 total of 197 consecutive patients with beta-thalassemia major or intermedia with at least 10 years o

42 e, paroxysmal nocturnal hemoglobinuria, beta-thalassemia major, or thalassemia intermedia.

43 We studied 107 pediatric thalassemia major patients (61 boys, median age 14.4 yea

44 oietin levels were found in the plasma of 67 thalassemia major patients compared with 29 control subj

45 ifferentiated from transduced HSCs from beta-thalassemia major patients demonstrated improved globin

46 magnetic resonance and serum ferritin in 652 thalassemia major patients from 21 UK centers with 1442

47 examined pancreatic and cardiac iron in 131 thalassemia major patients over a 4-year period.

48 jection fraction and endothelial function in thalassemia major patients with mild to moderate cardiac

49 eek) for myocardial iron removal in 197 beta-thalassemia major patients with myocardial siderosis (T2

50 In spleen specimens of thalassemia major patients, a higher level of heparanase

51 e should be monitored on an ongoing basis in thalassemia major patients, and the rate of transfusiona

52 (CMR) plays a key role in the management of thalassemia major patients, but few data are available i

53 yopathy are common in chronically transfused thalassemia major patients, occurring in the second and

54 al evolution of cardiac and liver iron in 38 thalassemia major patients, using previously validated m

55 tiparametric CMR approach can occur early in thalassemia major patients.

56 ction, and fibrosis in a cohort of pediatric thalassemia major patients.

57 rogenitor cells with correction of the human thalassemia major phenotype.

58 e and a partial, variable correction of beta-thalassemia major phenotype.

59 Twenty-four children with beta-thalassemia major received abatacept at a dose of 10 mg/

60 ular magnetic resonance in 167 patients with thalassemia major receiving standard maintenance chelati

61 Patients affected by beta-thalassemia major require lifelong transfusions because

62 Individuals with beta-thalassemia major require regular lifelong Red Blood Cel

63 beta-Thalassemia major results from severely reduced or absen

64 uch disorders as hereditary hemochromatosis, thalassemia major, sickle cell disease, aplastic anemia,

65 Patients with thalassemia major (Thal) frequently have low plasma zinc

66 A total of 255 patients with beta-thalassemia major (TM) and beta-thalassemia intermedia (

67 PAH) remains a concern in patients with beta-thalassemia major (TM) and intermedia (TI); however, stu

68 Patients with beta-thalassemia major (TM) and other refractory anemias requ

69 e analyzed the outcomes of 485 patients with thalassemia major (TM) or sickle cell disease (SCD) rece

70 -induced compromised fertility in women with thalassemia major (TM) was evaluated in 26 adult TM fema

71 L)-6, and IL-8 in biofluids of patients with thalassemia major (TM) with or without gingivitis.

72 ally low in approximately 40% of adults with thalassemia major (TM), suggesting myocardial iron depos

73 and treatment of cardiac dysfunction in beta-thalassemia major (TM).

74 till a major cause of death in patients with thalassemia major (TM).

75 pathies, sickle cell disease (SCD), and beta-thalassemia major (TM).

76 We sought to determine whether, in beta-thalassemia major, transfusion-mediated inhibition of er

77 in children with transfusion-dependent beta-thalassemia major undergoing a myeloablative allogeneic

78 Thalassemia major was a major risk factor for melioidosi

79 ron chelation therapy in 2010, no child with thalassemia major was diagnosed with melioidosis in 2011

80 and efficacy of deferiprone in patients with thalassemia major, we have extended the treatment period

81 ependent iron-loaded adult patients (38 with thalassemia major) were treated with the orally active i

82 tin insulator in transfusion-dependent human thalassemia major, where it would be ultimately relevant

83 n several of these disorders, including beta-thalassemia major, which is characterized by a defective