The Silent Threat of Hereditary Angioedema in Children: Single Center Experience

Kılavuz G. Ü., Tuncay H. A., Karakaya B., Kiğılı G., Gürbüz S., Süleyman A., ...Daha Fazla

European Academy of Allergy and Clinical Immunology (EAACI) Annual Congress 2025, Glasgow, İngiltere, 13 - 16 Haziran 2025, (Yayınlanmadı)

Yayın Türü: Bildiri / Yayınlanmadı
Basıldığı Şehir: Glasgow
Basıldığı Ülke: İngiltere
İstanbul Üniversitesi Adresli: Evet

Özet

Background

In children, diagnosis of hereditary angioedema (HAE) is often delayed due to the rarity of the condition, overlapping symptoms with other diseases, and lack of awareness among healthcare providers. This study presents clinical features, diagnostic challenges, and management outcomes of pediatric HAE cases.

Methods

The study included 24 cases diagnosed with HAE, followed at Istanbul Faculty of Medicine, Division of Pediatric Immunology and Allergy. Demographic, laboratory, and clinical data of the patients were analyzed, and all patients were assessed using the Angioedema Quality of Life Scale (AE-QoL) as well as the 4-week and 3-month Angioedema Control Tests (AECT).

Results

Our study included 24 patients (15 F/9 M) and the mean current age was 14.5 ±4.6 years. The mean age at first outpatient visit was 9.3 ±4.6 years, and the mean age at diagnosis was 8.9 ±7.2 years. Symptoms first appeared at a mean age of 6.3 ±3.9 years, with a median delay of 0.94 years (IQR 0.29–3.7) from symptom onset to diagnosis. Family history was present in 22(92%) patients.

Nineteen patients (79%) had Type 1 HAE, 4 (17%) had Type 2, and 1 (4%) had Type 3. The median time for attack resolution was 60 hours (IQR 48–84). Over the past month, the median number of attacks was 0.75 (IQR 0.20–2.75), and over the past year, it was 6 (IQR 2.5–14.75). All patients used icatibant during acute attacks, and 6 (25%) received long-term prophylaxis, though only 2 (4%) are currently on this treatment.

Trauma and stress were the most common triggers. According to AECT scores, 14 patients (58%) were well-controlled and 10 (42%) poorly controlled over the past 4 weeks, 12 patients (50%) were well-controlled, and 12 patients (50%) were poorly controlled over the 3 months.

There was no significant difference in the number of monthly and yearly attacks among the HAE types, respectively (p=0.8, p=0.5).

Conclusion

Our findings emphasize the importance of early diagnosis, family screening, and raising awareness of HAE among pediatricians to ensure timely and effective management. Addressing these gaps can lead to improved outcomes and a better understanding of HAE.