HEREDITARY ANGIOEDEMA WITH C1 ESTERASE DEFICIT IN CHILDREN AND ADOLESCENTS
Keywords:
Hereditary Angioedema, C1 Esterase Inhibitor, Bradykinin, ChildrenAbstract
Hereditary angioedema is a disease of autosomal dominant inheritance, characterized by the quantitative and / or functional deficiency of C1 esterase inhibitor. It is characterized by angioedema attacks involving the upper airway skin and / or mucosa as well as the intestinal mucosa in the absence of urticaria due to bradykinin accumulation. The diagnosis is often late, the clinical manifestations in children usually develop before the age of six. This study aimed to analyze 5 cases of children and adolescents with Hereditary Angioedema with C1 esterase inhibitor deficiency, followed at the Professor Alberto Antunes University Hospital Immunology and Allergy Outpatient Clinic, from October 2016 to April 2019. data was used a script previously structured with sociodemographic and clinical data. The database was based on the Microsoft Excel version 2017 program. Among the patients, four (80%) were male, aged between six and fifteen years. The age at which the diagnosis was made ranged from 04 to 14 years (mean 8.6 years). The average time elapsed between the first seizure and diagnosis was 2.6 years. All patients had a family history. In two patients (40%) the seizures were severe. In four patients (80%) facial edema was the main clinical manifestation. Four patients (80%) were seen in the emergency room more than once. Three patients (60%) reported that the duration of the seizure ranges from 2 to 5 days. Regarding the diagnosis, all patients had low serum C4 and quantitative and functional C1 esterase levels below the normal range. We observed an early diagnosis favored by family history and screening laboratory tests for diagnosis.
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References
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