Herediter Anjioödemli Çocuk Hastalarda Alerjik Hastalık Birlikteliğinin Araştırılması

Öz

Herediter anjiyoödem(HAE); C1 esteraz inhibitörün (C1-İNH) eksikliği veya disfonksiyonundan kaynaklanan; nadir görülen otosomal dominant bir hastalıktır. Herediter anjioödemle birlikte görülebilen hastalıklarla ilgili çalışmalarda alerjik hastalıkların sağlıklı popülasyona göre arttığı görülmüş, ancak şimdiye kadar bu konu ile ilgili bir çalışma yapılmamıştır. Bu çalışma herediter anjioödemli hastalarda atopik duyarlılık ve alerjik hastalıkların eşlik etme sıklığını belirlemek amacı ile yapıldı. Bu çalışmada 2019-2021 tarihleri arasında Mersin Şehir Hastanesi çocuk alerji polikliniğinde herediter anjioödem tanısı olan 32 hasta incelenmiştir. Bu gruptaki hastalarda atopik hastalıkların varlığı dosyalarından alınarak kaydedildi. Hastaların 6 (% 18,8)’sında astım, 18 (% 46,9)’inde rinit, 2 (% 6,2)’sinde ürtiker varlığı saptanırken, dermatit varlığına hastaların hiçbirinde rastlanılmadı. Fx5 testi hastalardan 5 (% 15,6)’inde pozitif iken, inhalan alerjen mix sps İgE 7 (% 25,9) hastada pozitif saptandı. IGE değerinin 12 (% 52,2) hastada 100 üstünde olduğu, eozinofil düzeyinin 16 (%50) hastada %4’ün üstünde olduğu gözlendi. Sonuç olarak bu çalışma ile herediter anjioödemli hastalarda atopik hastalık ve atopik duyarlılık birlikteliğinin sağlıklı popülasyona göre daha sık gözlendiğini düşünmekteyiz. Ancak doğrulamak için daha geniş hasta grubu ile çalışmaya ihtiyaç vardır.

Anahtar Kelimeler

• Hereditary angioedema
• allergic inflammation
• bradykinin
• Allergy
• Bradykinin

Analysis of the Association of Allergic Disease in Pediatric Patients with Hereditary Angioedema

Öz

Hereditary angioedema (HAE) is a rare autosomal dominant disease that is derived from the deficiency or dysfunction of C1 esterase inhibitor (C1-INH). In studies about the diseases that can accompany hereditary angioedema, allergic diseases occur more frequently compared to the healthy population but no studies on this issue have been carried out. This study was conducted in order to determine the frequency of the comorbidity of atopic sensitivity and allergic diseases in patients with hereditary angioedema. 32 patients who were diagnosed with hereditary angioedema in the Paediatric Allergy Polyclinic of Mersin City Hospital between 2019 and 2021 were included in the study. In these patients, the information about the existence of atopic diseases was obtained from their files and recorded. While asthma was observed in 6 (18.8%), allergic rhinitis in 18 (46.9%), and urticaria in 2 (6.2%) patients, dermatitis was not observed in any of the patients. While Fx5 test was positive in 5 (15.6%) patients, inhalant allergen mix sps IgE was positive in 7 (25.9%) patients. The IgE value was above 100 in 12 (52.2%) patients, and the eosinophil level was above 4% in 16 (50%) patients. In conclusion, we speculate in this study that the association of atopic disease and atopic sensitivity is observed more frequently in patients with angioedema than in the healthy population. However, a study with a larger sample is necessary to confirm this finding. 

Anahtar Kelimeler

• Allergy
• Bradykinin
• Hereditary angioedema,

Kaynakça

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