Akademik Veri Yönetim Sistemi
EUROPEAN JOURNAL OF PEDIATRICS, cilt.185, sa.9, ss.1-7, 2025 (Hakemli Dergi)
Alpha-gal syndrome (AGS) remains underdiagnosed in the pediatric population despite increasing awareness. This study comprehensively evaluates diagnostic delays, age-specific clinical presentations, environmental risk factors, and novel biomarkers for anaphylaxis prediction in childhood-onset AGS. This study examined 39 childhood-onset AGS cases diagnosed at the Ondokuz Mayıs University Pediatric Allergy and Immunology Clinic, with symptom onset at 18 years of age or younger. Patients underwent comprehensive clinical evaluation including alpha-gal specific IgE testing (≥ 0.35 kU/L), skin prick tests, and oral food challenge tests when deemed necessary. Diagnostic delay was defined as the time interval between symptom onset and definitive diagnosis. Clinical phenotypes, environmental exposures, laboratory parameters, and vitamin D levels were systematically evaluated. Among 39 patients (69.2% male), the median age of symptom onset was 7 years (range: 1-18), with a median diagnostic delay of 2 years (range: 0-35). Diagnostic delay exceeded 2 years in 43.6% of patients, with 12.8% experiencing extreme delay (> 10 years). Anaphylaxis occurred in 76.9% of patients and was predominantly early-onset (≤ 6 h: 61.5%). Tick bite history was present in 84.6% of patients, hazelnut orchard exposure in 69.2%, and family history of meat allergy in 53.8%. Alpha-gal IgE levels were significantly higher in males (10.10 ± 8.39 vs. 5.31 ± 2.70 ng/mL, p < 0.05) and patients with anaphylaxis (9.42 ± 8.04 vs. 5.99 ± 2.96 ng/mL, p = 0.048). Our novel findings revealed significantly lower vitamin D levels in anaphylactic patients (15.58 vs. 20.31 ng/mL, p = 0.049). Fresh meat prick tests demonstrated superior sensitivity (100%) compared to commercial extracts (51.3%). Patients without concurrent allergic disease showed significantly higher rates of diagnostic delay (52.9% vs. 13.6%, p = 0.008).
Conclusions: Childhood-onset AGS presents with age-dependent clinical variability and diagnosis is frequently delayed until adulthood. The high sensitivity of fresh meat extracts in diagnosis and the association between vitamin D deficiency and anaphylaxis are clinically noteworthy. Familial clustering suggests genetic predisposition, emphasizing the importance of family screening and early diagnostic approaches in endemic regions.