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Table 4 Results of diagnostic blood samples in 19 non-HAE children

From: Clinical characteristics and real-life diagnostic approaches in all Danish children with hereditary angioedema

ID

sex

Born

Age when HAE was refuted

Cord blood samples [reference values for adults]

Peripheral complement measurements [reference values for adults]

Genetic testing

Non1

m

1996

4.8 y

n.d.

4.8 y

fC1INH 121 [70–130]%

 

n.d.

Non2

m

1997

1.5 y

n.d.

1.5 y

C1INH 162 [80–140]units

fC1INH 168 [70–130]%

 

n.d.

Non3

m

1997

18.1 y

n.d.

2.9 y

Reported as normal, but missing

 

18.1 y

Family mutation ruled out

Non4

f

1997

14.9 y

n.d.

<14.6 y (exact age unknown)

Reported as normal (missing)

14.9 y

C1INH 0.33 [0.21–0.39]g/L

fC1INH 120 [70–130]%

C4 0.18 [0.1–0.4]g/L

n.d.

Non5

m

1999

15.7 y

n.d.

0.1 y

fC1INH 81 [70–130]%

 

15.7 y

Family mutation ruled out

Non6

m

1999

16.1 y

n.d

1 y

fC1INH 166 [70–130]%

 

16.1 y

Family mutation ruled out

Non7

f

1999

1.9 y

n.d.

1.9 y

fC1INH 110 [70–130]%

 

n.d.

Non8

f

2000

6.4 y

n.d.

6.4 y

C1INH 0.27 [0.21–0.39]g/L

fC1INH 106 [70–130]%

C4 0.2 [0.1–0.4]g/L

 

n.d.

Non9

m

2000

5.9 y

n.d.

4.3 y

C1INH 0.34 [0.21–0.39]g/L

fC1INH 129 [70–130]%

C4 0.11 [0.1–0.4]g/L

5.9 y

C1INH 0.34 [0.21–0.39]g/L

fC1INH 135 [70–130]%

C4 0.19 [0.1–0.4]g/L

n.d.

Non10

m

2002

9.1 y

n.d.

9.1 y

C1INH 0.32 [0.21–0.39]g/L

fC1INH 116 [70–130]%

C4 0.27 [0.1–0.4]g/L

 

n.d.

Non11

m

2003

3.2 y

n.d.

1.5 y

C1INH 0.38 [0.21–0.39]g/L

fC1INH 117 [70–130]%

C4 0.13 [0.1–0.4]g/L

3.2 y

C1INH 0.32 [0.21–0.39] g/L

fC1INH 134 [70–130]%

C4 0.22 [0.1–0.4]g/L

n.d.

Non12

m

2008

1.3 y

n.d.

1.3 y

C1INH 0.4 [0.21–0.39]g/L

fC1INH 174 [70–130]%

C4 0.33 [0.1–0.4]g/L

 

n.d.

Non13

m

2008

1 y

Taken, but not analyzed

0.8 y

C1INH 0.41 [0.21–0.39]g/L

fC1INH 179 [70–130]%

C4 0.31 [0.1–0.4]g/L

1 y

C1INH 0.35 [0.21–0.39]g/L

fC1INH 143 [70–130]%

C4 0.39 [0.1–0.4] g/L

n.d.

Non14

m

2009

4.4 y

n.d

2.4 y

C1INH 0.3 [0.21–0.39]g/L

fC1INH 130 [70–130]%

C4 0.26 [0.1–0.4]g/L

4.4 y

C1INH 0.26 [0.21–0.39]g/L

fC1INH 123 [70–130]%

C4 0.24 [0.1–0.4]g/L

5.3 y

Family mutation ruled out

Non15

f

2009

1 y

C1INH 0.2 [0.21–0.39]g/L

C4 0.14 [0.1–0.4]g/L

1 y

C1INH 0.43 [0.21–0.39]g/L

fC1INH 168 [70–130]%

C4 0.21 [0.1–0.4]g/L

 

1 y

Family mutation ruled out

Non16

m

2010

0.3 y

C1INH 0.17 [0.21–0.39]g/L

C4 0.16 [0.1–0.4]g/L

1.4 y

C1INH 0.57 [0.21–0.39]g/L

fC1INH 252 [70–130]%

C4 0.5 [0.1–0.4]g/L

 

0.3 y

Family mutation ruled out

Non17

m

2013

0 y

Taken for complement measurement, but mishandled.

Instead used for genetic testing.

  

0 y

Family mutation ruled out

Non18

f

2014

0 y

n.d.

  

<0 y

Chorionic villous sampling.

Family mutation ruled out

Non19

f

2015

0 y

Genetic testing, family mutation ruled out

  

0 y

Family mutation ruled out

  1. Complement values were equivocal (bold font) or normal compared with adult normal range
  2. N.d. not determined