With regard to the article entitled ‘‘Hereditary angioedema with normal C1 inhibitor associated with carboxypeptidase N deficiency ’’ (J Allergy Clin Immunol Global 2024;3:100223), the authors have notified the Editors of a necessary change in Table I and Fig 1. In Table I, Family A should be corrected with both asymptomatic mother II.3 and daughter III.1. Additionally, in Fig 1 Family C should be corrected by introducing the synonymous variant c.1299T that is carried by both sister III.1 and mother II.2, and c.582G also carried by sister III.1. The Results section is correct. The authors apologize for the errors. The corrected Table I and Fig 1 appear below.
Table I.
Clinical records
| Family ID | Patient | Sex | Symptoms∗ |
Trigger | Age of onset | Delay diagnostic | Treatment prophylaxis | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Peripheral | Abdominal | Laryngeal | Macroglossia | Urticaria | |||||||
| A | I.2 | F | Yes | Yes | No | No | No | Unknown | 40 y | 1 y | Tranexamic acid |
| II.1† | F | Yes | Yes | Yes | No | Yes | Pressure pruritus, triptorelin | 41 y | 2 y | Tranexamic acid, icatibant on demand | |
| II.2 | M | Yes | Yes | Yes | No | Yes | Unknown | — | — | Tranexamic acid | |
| II.3 | F | No | No | No | No | No | Unknown | — | — | None | |
| III.1 | F | No | No | No | No | No | Unknown | — | — | None | |
| B | I.1 | M | No | No | No | No | Yes | Unknown | 22 y | 75 y | None |
| I.2 | F | Yes | Yes | No | No | Yes | None | 27 y | 70 y | None | |
| II.1† | F | Yes | Yes | Mild | No | Yes | Spontaneous and/or cold | 30 y | 12 y | Tranexamic acid, montelukast, icatibant on demand | |
| III.1 | F | Yes | Yes | No | No | Yes | Unknown | 25 y | 1 y | Tranexamic acid, montelukast, icatibant on demand | |
| III.3 | F | No | No | No | No | Yes | Unknown | 17 y | 1 y | None | |
| IV.1 | M | No | Yes | No | No | Yes | Unknown | 12 y | 6 mo | None | |
| C | II.2 | F | Yes | Yes | No | No | Yes | Cold | 47 y | 6 mo | None |
| III.1 | F | No | Yes | No | No | Rare | Unknown | 17 y | 6 mo | None | |
| III.2† | F | — | Yes | No | No | Yes | Cold | 15 y | 6 mo | Tranexamic acid, icatibant on demand | |
| D | I.1 | M | No | Yes | No | No | Yes | Unknown | 16 y | 30 y | None |
| I.2 | F | No | No | No | No | Yes | Unknown | 12 y | 30 y | None | |
| II.1† | M | Yes | Yes | Yes | Rare | Chronic urticaria | Pressure, cold, fatigue | 18 y | 14 y | Tranexamic acid, montelukast, icatibant or C1-INH concentrate on demand | |
F, Female; M, male.
Urticarial lesions in CPN-deficient patients developed frequently, but not consistently, in association with angioedema attacks. An urticarial rash accompanied nearly 60% of symptomatic episodes of angioedema.
Family proband.