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Table 3 Baseline characteristics of patients who had post-treatment data recorded in PMS database

From: Application of a scoring system in Japanese patients diagnosed with atypical hemolytic uremic syndrome to assess the relationship between the score and clinical responses to eculizumab

 

Partial response

Complete response

 

Met

Not met

p

Met

Not met

p

N

174

9

 

56

128

 

TMA score items

 Hb < 10.0 g/dL*

169

(97.1)

9

(100)

1.000

55

(98.2)

124

(96.9)

1.000

 PLT < 150 × 109/L

171

(98.3)

8

(88.9)

0.184

56

(100)

124

(96.9)

0.315

 Renal failure

162

(93.1)

6

(66.7)

0.027

55

(98.2)

113

(88.3)

0.042

 Extrarenal organ failures

114

(65.5)

4

(44.4)

0.283

36

(64.3)

83

(64.8)

1.000

Positive aHUS score items

Past history of TMA

16

(9.2)

2

(22.2)

0.217

4

(7.1)

14

(10.9)

0.591

 1 time

16

(9.2)

2

(22.2)

 

4

(7.1)

14

(10.9)

 

 ≥ 2 times

0

(0)

0

(0)

 

0

(0)

0

(0)

 

Family history of TMA

10

(5.7)

0

(0)

1.000

3

(5.4)

7

(5.5)

1.000

 1 family member

8

(4.6)

0

(0)

 

2

(3.6)

6

(4.7)

 

 ≥ 2 family members

2

(1.1)

0

(0)

 

1

(1.8)

1

(0.8)

 

Age at TMA onset

 < 10 years

46

(26.4)

3

(33.3)

0.438

23

(41.1)

26

(20.3)

0.003

 ≥ 10 to < 63 years

91

(52.3)

3

(33.3)

 

27

(48.2)

67

(52.3)

 

 ≥ 63 years

37

(21.3)

3

(33.3)

 

6

(10.7)

35

(27.3)

 

Trigger

42

(24.1)

2

(22.2)

1.000

9

(16.1)

35

(27.3)

0.132

Exclusion aHUS score items

 Bloody stool

22

(12.6)

0

(0)

0.602

5

(8.9)

17

(13.3)

0.468

 ADAMTS13 < 10%

2

(1.1)

0

(0)

1.000

1

(1.8)

1

(0.8)

0.517

 Underlying diseases

26

(14.9)

3

(33.3)

0.154

1

(1.8)

28

(21.9)

 < 0.001

 D-dimer > 20 μg/mL

0

(0)

0

(0)

0

(0)

0

(0)

 WBC > 16,000/mL

2

(1.1)

1

(11.1)

0.141

1

(1.8)

2

(1.6)

1.000

Total score, median (range)

10

(3–16)

9

(3–14)

0.155

11

(6–14)

10

(3–16)

 < 0.001

  1. Values are n (%), unless stated otherwise
  2. *Description used in post-marketing surveillance: microangiopathic hemolytic anemia (defined from following items: Hb < 10 g/dL, LDH increase, haptoglobin decrease, presence of schistocyte)
  3. Wilcoxon’s rank sum test and Fisher’s test were used, as appropriate
  4. Abbreviations: ADAMTS13 a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, aHUS atypical hemolytic uremic syndrome, Hb hemoglobin, PLT platelet count, sCr serum creatinine, TMA thrombotic microangiopathy, ULN upper limit of normal, WBC white blood cell count