First Census of Patients with Hereditary Angioedema in the Canary Islands.

Hereditary angioedema (HAE) is a rare genetic condition whose main symptoms are recurrent swelling in the skin, mucosa, and internal organs. Recent studies suggested that the regulation of the inflammatory response and the complement cascade are two of the pathways significantly enriched in the Canary Islands, Spain. Here, we describe the first HAE patient series in this region. Forty-one patients (33 F, 8 M) and nine healthy relatives belonging to twenty-nine families were recruited for this study, obtaining their clinical and demographic features using a data collection form, as well as blood samples for biochemical analysis. The mean age of patients was 36.8 years (ranging from 4 to 72 years). Positive family history of HAE was reported in 13 patients (32.5%), and a mean diagnosis delay of 7.9 (±12.5) years was estimated, ranging from months to 50 years. Cutaneous edema was the most common symptom (53.6%), while airway symptoms was present in 11 patients. Prophylactic treatment was indicated for 23 patients, while 14 also require on-demand rescue treatment. We estimate a minimum prevalence of 1.25:100,000 for HAE due to C1-INH deficiency or dysfunction in the Canary Islands, which is higher than the estimates for mainland Spanish populations. HAE continues to be a disease poorly recognized by health care professionals due to its confusing symptoms, leading to longer diagnosis delay. Altogether, the evidence reinforces the need for a rapid and accurate diagnosis and precision medicine-based studies to improve the patient's quality of life.

Safety and effectiveness of a microcrystalline tyrosine-associated mite extract immunotherapy for allergic rhinitis.

Aim: To assess the safety and effectiveness of an allergen immunotherapy (AIT) with a microcrystalline tyrosine-associated mite allergoid in real-world patients with allergic rhinitis (AR). Materials & methods: Retrospective, multicenter study assessing the safety of AIT in patients aged 5 to 65 years with AR, with or without asthma, sensitized to mites. Secondary objective was effectiveness, measured as unscheduled visits to healthcare centers and emergency rooms, rhinitis and asthma evolution, medication use and patients' and physicians' disease perception 12 months before and after treatment. Results: The 306 patients evaluated, with a mean (standard deviation) age of 29.68 (14.66) years, received different treatment compositions and regimens, and 25 (8.2%) experienced nonserious adverse reactions. Unscheduled visits to the specialist and emergency room admissions significantly decreased after immunotherapy (mean [standard deviation] 2.11 [1.95] and 0.3 [0.93] vs 0.66 [1.09] and 0.02 [0.2], before and after treatment, respectively). Rhinitis and asthma classification ('AR and its impact on asthma' and 'Guía Española para el Manejo del Asma', respectively) significantly changed (p < 0.0001 for all classifications), showing symptom reduction after AIT. Median (interquartile range)-combined rhinitis and combined asthma medication scores significantly decreased (4.0 [1.33, 7.0] vs 0.25 [0, 10.0]; p < 0.0001 and 6.94 [1.5, 6.0] vs 0.67 [0, 4.67]; p < 0.0001) within 12 months before and after starting AIT, respectively. Conclusion: AIT with microcrystalline tyrosine-associated mite allergoid appears to be safe and effective in treating rhinitis caused by mites.