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Table 1 (abstract O-28) Laboratory study at diagnosis

From: 13th C1-inhibitor deficiency and angioedema workshop—2023

Patient number

Plasma C1-INH function (> 50%)

Antigenic C1-INH (16.0–33.0 mg/dL)

Serum C4 levels (14.0–60.0 mg/dL)

Serum C1q levels (> 100 µg/dL)

Autoantibodies anti C1INH (antiC1INHAbs)

Immunocomplexes C1INH–antiC1INHAb

SERPING1 gene study

1

50

4.8

1.67

60

ND

ND

ND

2

10.4

27.5

7.71

60

ND

ND

ND

3

11

9.8

 

138

ND

ND

ND

4

40

9.89

2

58

Negative

ND

ND

5

18

2.8

1.4

11.5

IgG

ND

ND

6

14.06

17.9

16.4

120

Negative

ND

Negative

7

25.67

16.2

 

157.95

IgG

ND

Negative

8

6

15

1.56

0

IgA

Negative

Negative

9

10.77

5.82

1.56

0

IgA, IgG

ND

Negative

10

21.61

13

35.9

233.16

Negative*

Negative*

Negative

11

33.46

12.1

1.66

32.13

Negative

IgG

Negative

12

19.64

4.62

5.32

111.88

IgA, IgM

Negative

Negative

13

25.96

3.99

1.5

71.82

Negative

IgA, IgG

Negative

14

11.38

5.49

3.53

58.11

IgM

Negative

Negative

15

7.9

5.24

ND

115.58

IgG

Negative

Negative

  1. ND: Not done.
  2. *Patient 10 had positive autoC1-INHabs several years later.