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Table 2 Characteristics of initial hyperammonemic episodes in all patients

From: Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001–2013

 

CPS1D

OTCD

ASSD

All patients

Number of patients

12

23

27

63

First hyperammonemic crisis ≥ 150 μmol/L

 

N = 20a

 

N = 60a

   No

3 (15.0 %)

6 (22.2 %)

9 (15.0 %)

   Yes

12 (100.0 %)

17 (85.0 %)

21 (77.8 %)

51 (85.0 %)

 Peak concentration [μmol/L]

  Minimum

203

385

161

161

  Maximum

2627

4050

2690

4050

  Mean

1257

1875

1084

1378

  SD

929

1105

682

950

  150 – < 250

1 (8.3 %)

1 (4.8 %)

2 (3.9 %)

  250 – < 500

2 (16.7 %)

1 (5.9 %)

3 (14.3 %)

6 (11.8 %)

   ≥ 500

9 (75.0 %)

16 (94.1 %)

17 (81.0 %)

43 (84.3 %)

 Duration of crisis

  1 day

3 (17.6 %)

3 (14.3 %)

6 (11.8 %)

  2–7 days

8 (66.7 %)

9 (52.9 %)

16 (76.2 %)

34 (66.7 %)

  8–14 days

3 (25.0 %)

4 (23.5 %)

2 (9.5 %)

9 (17.6 %)

  15–19 days

1 (8.3 %)

1 (5.9 %)

2 (3.9 %)

  1. Peak concentration of ammonia is the highest level of plasma ammonia during the initial crisis. SD Standard deviation
  2. athree missing values