Clinical Features Of Pediatric Hereditary Angioedema

Abstract

RationaleThere is a paucity of data describing the clinical course of hereditary angioedema (HAE) in children. The purpose of this study was to identify and characterize children with HAE in our medical center.MethodsElectronic medical records from the past 10 years at Cincinnati Children’s Hospital Medical Center and Bernstein Allergy Group were searched for ICD-9 code 277.6 (Other deficiencies of circulating enzyme), in patients less than 18 years of age. Exclusion criteria included laboratory data not supportive of type I or type II HAE, or age at diagnosis greater than 18 years. Chart review was performed to ascertain the clinical presentation of patients, and missing data was collected by telephone interviews with patient families. Descriptive statistics were performed using SAS version 9.3.ResultsTwenty-one patients were identified. The mean age was 13.6 years (2.9-24.4), 71% were male, 86% had an HAE family history and 95% were Caucasian. The mean age of symptom onset and diagnosis was 6.5 (2-12) and 6.3 years (1-16), respectively. Five patients diagnosed were still asymptomatic. The most common angioedema attack sites were abdominal, peripheral and laryngeal, which occurred at least once in 70%, 55%, and 20% of children, respectively. The mean number of lifetime hospitalizations and ER visits was 0.8 (0-6) and 1.4 (0-12), respectively. Only 15% of patients required prophylaxis therapy.ConclusionsIn our pediatric HAE patient population, the age of onset is younger than the average age of 11.2 years previously reported, with abdominal being more common than peripheral attack sites. RationaleThere is a paucity of data describing the clinical course of hereditary angioedema (HAE) in children. The purpose of this study was to identify and characterize children with HAE in our medical center. There is a paucity of data describing the clinical course of hereditary angioedema (HAE) in children. The purpose of this study was to identify and characterize children with HAE in our medical center. MethodsElectronic medical records from the past 10 years at Cincinnati Children’s Hospital Medical Center and Bernstein Allergy Group were searched for ICD-9 code 277.6 (Other deficiencies of circulating enzyme), in patients less than 18 years of age. Exclusion criteria included laboratory data not supportive of type I or type II HAE, or age at diagnosis greater than 18 years. Chart review was performed to ascertain the clinical presentation of patients, and missing data was collected by telephone interviews with patient families. Descriptive statistics were performed using SAS version 9.3. Electronic medical records from the past 10 years at Cincinnati Children’s Hospital Medical Center and Bernstein Allergy Group were searched for ICD-9 code 277.6 (Other deficiencies of circulating enzyme), in patients less than 18 years of age. Exclusion criteria included laboratory data not supportive of type I or type II HAE, or age at diagnosis greater than 18 years. Chart review was performed to ascertain the clinical presentation of patients, and missing data was collected by telephone interviews with patient families. Descriptive statistics were performed using SAS version 9.3. ResultsTwenty-one patients were identified. The mean age was 13.6 years (2.9-24.4), 71% were male, 86% had an HAE family history and 95% were Caucasian. The mean age of symptom onset and diagnosis was 6.5 (2-12) and 6.3 years (1-16), respectively. Five patients diagnosed were still asymptomatic. The most common angioedema attack sites were abdominal, peripheral and laryngeal, which occurred at least once in 70%, 55%, and 20% of children, respectively. The mean number of lifetime hospitalizations and ER visits was 0.8 (0-6) and 1.4 (0-12), respectively. Only 15% of patients required prophylaxis therapy. Twenty-one patients were identified. The mean age was 13.6 years (2.9-24.4), 71% were male, 86% had an HAE family history and 95% were Caucasian. The mean age of symptom onset and diagnosis was 6.5 (2-12) and 6.3 years (1-16), respectively. Five patients diagnosed were still asymptomatic. The most common angioedema attack sites were abdominal, peripheral and laryngeal, which occurred at least once in 70%, 55%, and 20% of children, respectively. The mean number of lifetime hospitalizations and ER visits was 0.8 (0-6) and 1.4 (0-12), respectively. Only 15% of patients required prophylaxis therapy. ConclusionsIn our pediatric HAE patient population, the age of onset is younger than the average age of 11.2 years previously reported, with abdominal being more common than peripheral attack sites. In our pediatric HAE patient population, the age of onset is younger than the average age of 11.2 years previously reported, with abdominal being more common than peripheral attack sites.