Literature DB >> 30819279

Clinical profile and quality of life of Puerto Ricans with hereditary angioedema.

Yanira M Arce-Ayala1, Yaritza Diaz-Algorri2, Timothy Craig3, Cristina Ramos-Romey4.   

Abstract

Background: There is limited data on clinical characteristics and quality of life (QoL) of Hereditary angioedema (HAE) patients in Puerto Rico. Objective: Create an epidemiological and clinical profile of patients with HAE and assess the burden of this condition on the general health of Puerto Ricans suffering the disease.
Methods: A cross-sectional study was performed in 32 Puerto Rican patients with HAE. To obtain data on sociodemographic characteristics, clinical symptoms and treatment, a clinical survey based on a Spanish adaptation of the "HAEA Patient Summit Questionnaire 2015" was used. To measure QoL, the SF-36v2 questionnaire was used. IBM SPSS 22.0 (IBM, NY, USA) and QualityMetric Health Outcomes™ Scoring Software 4.5 was utilized for statistical analysis.
Results: The most common type of HAE was Type I (41.9%). In 1 year, 32.3% of patients present 2-3 attacks per month. The most common locations were: abdomen and face. Seventy-eight percent of patient developed laryngeal edema but only 6.5% needed tracheotomy. Fifty-eight percent of patients were using prophylaxis therapy; C1 esterase inhibitor (68.7%) being the most common. Eighty-one percent of patients were using rescue medication; Icatibant (80.8%) being the most common. Compared with US population norms of quality of life, patients with HAE in Puerto Rico reported significantly lower scores, both in the physical component (62%) and in the mental components (58%). The most affected parameters were emotional (38.52), physical (39.19) and social (39.79) components. Gender differences were observed in our sample; females scored below males in all components except for general health.
Conclusion: Puerto Rican Hispanics showed a similar epidemiologic and clinical profile to previous studies, however; higher frequency of attacks was prominent. Our study demonstrates a substantial and noteworthy decrease in quality of life in HAE patients and an increase risk for depression, particularly among woman.

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Mesh:

Year:  2019        PMID: 30819279     DOI: 10.2500/aap.2019.40.4200

Source DB:  PubMed          Journal:  Allergy Asthma Proc        ISSN: 1088-5412            Impact factor:   2.587


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5.  Effectiveness and safety of lanadelumab in ethnic and racial minority subgroups of patients with hereditary angioedema: results from phase 3 studies.

Authors:  Timothy J Craig; Rafael H Zaragoza-Urdaz; H Henry Li; Ming Yu; Hong Ren; Salomé Juethner; John Anderson
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6.  A catalog of the genetic causes of hereditary angioedema in the Canary Islands (Spain).

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  6 in total

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