Recurrent angioedema in childhood: hereditary angioedema or histaminergic angioedema?

Abstract

The exact rate of prevalence of pediatric angioedema is not well known, and any reported rate will change with the presence of urticaria, or due to a specific cause of angioedema. The lifetime prevalence of non-hereditary angioedema was shown to be 4.9%-7.4% in two large nationwide studies. 1,2 However, information about the prevalence of recurrent histaminergic angioedema (HA) and its clinical presentation in children is limited. Considering only patients with hereditary angioedema (HAE), it has been reported that approximately 1 in 50 000 individuals is affected, with a range of 1:10 000-1:150 000 worldwide. 3 Hereditary angioedema is a rare genetic disease characterized by recurrent episodes of submucosal or subcutaneous swelling. The clinical manifestations are highly variable, from asymptomatic cases to life-threatening attacks. The more common forms, HAE type I (85% of patients) and type 2 are caused by autosomal dominant mutations in the gene encoding the C1 inhibitor (C1-INH), leading

Identification of novel biomarkers to distinguish bradykinin‐mediated angioedema from mast cell‐/histamine‐mediated angioedema

“…With regard to locations and symptoms, significant differences could not be identified consistent with published data 21,33,34,41,42 …”

“…Significantly increased Tie-2 and VE-Cadherin levels were characteristic for HAE compared to ACE-AE, CSU-AE, and controls (Figure 1E With regard to locations and symptoms, significant differences could not be identified consistent with published data. 21,33,34,41,42 In summary, no distinct diagnostic relationship was found, not even for BK-versus histamine-mediated angioedema, despite the finding that some clinical characteristics were more frequently associated with a certain angioedema type, there was no diagnostic clue, not even for BK-versus histamine-mediated angioedema. Regarding clinical characteristics at visit 1, our limited study has evaluated only past events and includes only small populations.…”

Pediatric Angioedema without Wheals: How to Guide the Diagnosis

“…To the best of our knowledge, only the prospective study (95 patients) by Ertoy Karagol et al and the retrospective study (42 patients) by Ocak et al deepen this theme in the pediatric age [9,38]. Specifically, these studies included subjects affected by histamine-mediated AEwW, including in their cohorts patients affected by AE with poor response to antihistamines, the simultaneous presence of urticaria, or positive family history of AE [9,38]. Cross-sectional studies on pediatric populations identified a prevalence of 1.6% of isolated AE [9].…”

“…Little is known about the prevalence, clinical presentation, etiology, management, and follow-up of pediatric-acquired or idiopathic isolated histamine-mediated AE. To the best of our knowledge, only the prospective study (95 patients) by Ertoy Karagol et al and the retrospective study (42 patients) by Ocak et al deepen this theme in the pediatric age [9,38]. Specifically, these studies included subjects affected by histamine-mediated AEwW, including in their cohorts patients affected by AE with poor response to antihistamines, the simultaneous presence of urticaria, or positive family history of AE [9,38].…”

Angioedema and Fatty Acids

“…In mast-cell-induced angioedema, IgE-mediated angioedema is closely associated with a histamine-mediated mechanism, which is a Type I allergic reaction to drugs and external allergens [13,14]. ACE inhibitor is the major representative trigger of non-IgE-mediated angioedema.…”