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Table 2 Causes of UCD-related hyperammonemia

From: Efficacy and safety of i.v. sodium benzoate in urea cycle disorders: a multicentre retrospective study

Causes

Patients

N = 61 (%)

Episodes

N = 95 (%)

OTCD

20 (32.8 %)

35 (36.8 %)

ASSD

18 (29.5 %)

30 (31.6 %)

CPSD

15 (24.6 %)

18 (18.9 %)

ASLD

3 (4.9 %)

4 (4.2 %)

ARGD

1 (1.6 %)

2 (2.1 %)

NAGSD

1 (1.6 %)

2 (2.1 %)

Others

3 (4.9 %)*

4 (4.2 %)

  1. *Suspected OTCD without a molecular basis N = 2, HHH syndrome N = 1
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172