ライフサイエンスコーパス: ATTR

1 ATTR are caused by aggregation of transthyretin (TTR), a

2 ATTR can result from substitution of valine for isoleuci

3 subjects with autopsy-documented AL (n=10), ATTR (n=10), and nonamyloid controls (n=10) using (18)F-

4 Forty-five subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) underwent (99m)Tc-P

5 e subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) underwent (99m)Tc-PYP planar and single-ph

6 ign and rationale behind the ongoing phase 3 ATTR-ACT study (Tafamidis in Transthyretin Cardiomyopath

7 olume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to

8 mural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR).

9 Hereditary transthyretin amyloid (ATTR) amyloidosis is a rare but fatal autosomal dominant

10 ular pattern, whereas transthyretin amyloid (ATTR) more commonly showed patchy deposits.

11 m transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, th

12 The transthyretin amyloidoses (ATTR) are invariably fatal diseases characterized by pro

13 Transthyretin cardiac amyloidosis (ATTR) is an underrecognized cause of heart failure (HF)

14 Transthyretin amyloidosis (ATTR) is a heterogeneous disorder with multiorgan involv

15 Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failu

16 Familial transthyretin amyloidosis (ATTR) is associated with extracellular deposition of wil

17 Hereditary transthyretin amyloidosis (ATTR) is usually characterised by a progressive peripher

18 ed for hereditary transthyretin amyloidosis (ATTR) since 1990.

19 ted with familial transthyretin amyloidosis (ATTR), a hereditary degenerative disease.

20 Lkappa, ALlambda, transthyretin amyloidosis (ATTR), and Abeta amyloid deposits in tissue sections.

21 loidosis (AL) and transthyretin amyloidosis (ATTR).

22 hereditary transthyretin (TTR) amyloidosis (ATTR).

23 to study systemic amyloidosis of type AL and ATTR affecting the heart and should be investigated furt

24 apir binds specifically to myocardial AL and ATTR amyloid deposits.

25 um of cardiac involvement in systemic AL and ATTR amyloidosis.

26 apir specifically binds to myocardial AL and ATTR deposits in humans and offers the potential to scre

27 F-florbetapir uptake was noted in all AL and ATTR samples and in none of the control samples.

28 sthyretin cardiac amyloidosis (also known as ATTR cardiac amyloidosis) is an increasingly recognized

29 Recently, a relationship between ATTR-fibril composition and cardiomyopathy has been note

30 >99% sensitive and 86% specific for cardiac ATTR amyloid, with false positives almost exclusively fr

31 ty and positive predictive value for cardiac ATTR amyloidosis of 100% (positive predictive value conf

32 noninvasive diagnostic criteria for cardiac ATTR amyloidosis that are applicable to the majority of

33 maging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited.

34 cintigraphy enables the diagnosis of cardiac ATTR amyloidosis to be made reliably without the need fo

35 d) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individu

36 Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3

37 ing was typical in all patients with cardiac ATTR.

38 rty-four patients with genetically confirmed ATTR were assessed; 37 were symptomatic; mean age at ons

39 The goal of this study was to describe ATTR in the United States by using data from the THAOS (

40 ensitivity and 92% specificity for detecting ATTR cardiac amyloidosis with an area under the curve of

41 m PYP) cardiac imaging noninvasively detects ATTR cardiac amyloidosis, but the accuracy of this techn

42 clinical parameters accurately discriminated ATTR V122I amyloidosis from nonamyloid HF in a case-matc

43 thotopic liver transplantation for familial (ATTR) amyloidosis, was developed.

44 ielded excellent discriminatory capacity for ATTR V122I amyloidosis (AUC = 0.97; 95% CI, 0.93-1.00),

45 gand, could be used as a diagnostic test for ATTR V122I amyloidosis.

46 New therapies are in testing for ATTR, whereas those for AL have followed multiple myelom

47 performed to identify optimal thresholds for ATTR V122I amyloidosis identification.

48 PYP cardiac imaging as a diagnostic tool for ATTR cardiac amyloidosis and its association with surviv

49 Tc-PYP cardiac imaging distinguishes AL from ATTR cardiac amyloidosis and may be a simple, widely ava

50 hod was successfully tested using serum from ATTR patients with known variants (Val30-->Met and Val12

51 d after liver transplantation for hereditary ATTR amyloidosis, although gastrointestinal symptom scor

52 inal manifestations are common in hereditary ATTR amyloidosis and are important for the patients' mor

53 Swedish patients with hereditary ATTR amyloidosis transplanted between 1990 and 2012 were

54 Twenty-four patients with hereditary ATTR amyloidosis who had undergone LTx and have had thei

55 ts with ATTR than in those with AL; however, ATTR is associated with higher cell volume, which sugges

56 er the curve 0.992, P<0.0001 for identifying ATTR cardiac amyloidosis.

57 In ATTR amyloidosis, survival correlated with NYHA function

58 patients have the T60A missense mutation in ATTR where tyrosine is replaced by adenine at position 6

59 ardiac involvement in relation to outcome in ATTR.

60 mmonest pattern of ventricular remodeling in ATTR.

61 independent predictor of patient survival in ATTR amyloidosis.

62 t of cardiomyopathy and heart failure in LTx ATTR amyloid patients is related to amyloid fibril compo

63 utcomes for a relatively large series of LTx ATTR patients with the Val30Met (mutation are available,

64 L (mean age, 62 years +/- 10), 44 had mutant ATTR (mean age, 68 years +/- 10), and 66 had wild-type A

65 ECV, mean AL was 0.54 +/- 0.07, mean mutant ATTR was 0.60 +/- 0.07, and mean wild-type ATTR was 0.57

66 , mean AL was 107 g/m(2) +/- 30; mean mutant ATTR was 137 g/m(2) +/- 29; and mean wild-type ATTR was

67 ith ATTR cardiac amyloidosis and 50 with non-ATTR cardiac amyloidosis [34 with AL amyloidosis and 16

68 ata from the largest international cohort of ATTR V122I patients, followed up at the UK National Amyl

69 Tc 99m PYP cardiac imaging for detection of ATTR cardiac amyloidosis.

70 A definitive diagnosis of ATTR depends on the detection and identification of TTR

71 A definitive diagnosis of ATTR relies on the detection and identification of TTR v

72 lgorithm may be useful for identification of ATTR V122I amyloidosis in elderly African American patie

73 identified RBP4 as a sensitive identifier of ATTR V122I amyloidosis (area under the curve [AUC] = 0.7

74 In Afro-Caribbean patients, ATTR V122I is an underappreciated cause of heart failure

75 tor cohort of 25 patients with biopsy-proven ATTR V122I amyloidosis recruited from September 1, 2009,

76 In the THAOS registry, ATTR in the United States is overwhelmingly a disorder o

77 s, and the hereditary and "senile systemic" (ATTR) variants from mutant and wild-type transthyretin (

78 frequent and causes more organ disease than ATTR.

79 The ATTR-ACT study will provide important insight into the e

80 The description of the ATTR neuropathy phenotype, especially in the T60A patien

81 ); P<0.001), and in the AL compared with the ATTR samples (2.48+/-0.40 versus 1.52+/-0.22 DPM/mm(2);

82 globulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardi

83 Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopa

84 oglobulin light-chain (AL) or transthyretin (ATTR) type-and healthy volunteers (n = 5) were investiga

85 age, 68 years +/- 10), and 66 had wild-type ATTR (mean age, 75 years +/- 7).

86 ition 122 (Val122Ile) (n = 91) and wild-type ATTR (n = 189).

87 t ATTR was 0.60 +/- 0.07, and mean wild-type ATTR was 0.57 +/- 0.06 versus 0.27 +/- 0.03 in healthy s

88 TR was 137 g/m(2) +/- 29; and mean wild-type ATTR was 133 g/m(2) +/- 27 versus 65 g/m(2) +/- 15 in he

89 Here, we present outcomes for non-Val30Met ATTR patients after LTx, as reported to the Familial Amy

90 hate ((99m)Tc-PYP) scintigraphy in AL versus ATTR.

91 9m PYP imaging of 171 participants (121 with ATTR cardiac amyloidosis and 50 with non-ATTR cardiac am

92 regression analyses among participants with ATTR cardiac amyloidosis showed that an H/CL ratio of 1.

93 hy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour)

94 ntly higher percentage of U.S. patients with ATTR amyloid seen at cardiology sites had wild-type dise

95 tively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119

96 observational cohort study of patients with ATTR attending the National Hospital Inherited Neuropath

97 ificity for differentiation of patients with ATTR cardiac amyloidosis (irrespective of genotype) from

98 ated with worse survival among patients with ATTR cardiac amyloidosis.

99 Patients with ATTR had a higher total cell volume than did healthy sub

100 eposition is more extensive in patients with ATTR than in those with AL; however, ATTR is associated

101 ns were similar between the 25 patients with ATTR V122I amyloidosis (mean [SD] age, 72.2 [7.4] years;

102 ejection fraction was lower in patients with ATTR V122I amyloidosis (mean [SD], 40% [14%] vs 57% [14%

103 BP4 concentration was lower in patients with ATTR V122I amyloidosis compared with nonamyloid controls

104 frican American patients and 9 patients with ATTR V122I amyloidosis comprised the validation cohort.

105 Patients with ATTR V122I had the worst prognosis compared with other c

106 tracellular volume, whereas in patients with ATTR, the increase is extracellular, with an additional

107 Subjects with ATTR cardiac amyloid had a significantly higher semiquan

108 ailable method for identifying subjects with ATTR cardiac amyloidosis, which should be studied in a l

109 In addition, eight healthy subjects with ATTR mutations (mean age, 47 years +/- 6) and 47 healthy