Hereditary angioedema: quality of life in Brazilian patients
OBJECTIVE: Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier España
2013-01-01
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| Series: | Clinics |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322013000100014 |
| _version_ | 1818115126917595136 |
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| author | Maria Abadia Consuelo M. S. Gomide Eliana Toledo Solange Oliveira Rodrigues Valle Regis A. Campos Alfeu T. França Nieves Prior Gomez Heitor Franco Andrade Jr. Teresa Caballero Anete S. Grumach |
| author_facet | Maria Abadia Consuelo M. S. Gomide Eliana Toledo Solange Oliveira Rodrigues Valle Regis A. Campos Alfeu T. França Nieves Prior Gomez Heitor Franco Andrade Jr. Teresa Caballero Anete S. Grumach |
| author_sort | Maria Abadia Consuelo M. S. Gomide |
| collection | DOAJ |
| description | OBJECTIVE: Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous, gastrointestinal and laryngeal edema. The unpredictability, intensity and other factors associated with the disease impact the quality of life of hereditary angioedema patients. We evaluated the quality of life in Brazilian hereditary angioedema patients. METHODS: Patients older than 15 years with any severity of hereditary angioedema and laboratory confirmation of C1 inhibitor deficiency were included. Two questionnaires were used: a clinical questionnaire and the SF-36 (a generic questionnaire). This protocol was approved by the Ethics Committee of Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. RESULTS: The SF-36 showed that 90.4% (mean) of all the patients had a score below 70 and 9.6% had scores equal to or higher than 70. The scores of the eight dimensions ranged from 51.03 to 75.95; vitality and social aspects were more affected than other arenas. The internal consistency of the evaluation was demonstrated by a Cronbach's alpha value above 0.7 in seven of the eight domains. CONCLUSIONS: In this study, Brazilian patients demonstrated an impaired quality of life, as measured by the SF-36. The most affected domains were those related to vitality and social characteristics. The generic SF-36 questionnaire was relevant to the evaluation of quality of life; however, there is a need for more specific instruments for better evaluation. |
| first_indexed | 2024-12-11T04:01:40Z |
| format | Article |
| id | doaj.art-6b364684388745cd8de95773f816b0d8 |
| institution | Directory Open Access Journal |
| issn | 1807-5932 1980-5322 |
| language | English |
| last_indexed | 2024-12-11T04:01:40Z |
| publishDate | 2013-01-01 |
| publisher | Elsevier España |
| record_format | Article |
| series | Clinics |
| spelling | doaj.art-6b364684388745cd8de95773f816b0d82022-12-22T01:21:38ZengElsevier EspañaClinics1807-59321980-53222013-01-016818183Hereditary angioedema: quality of life in Brazilian patientsMaria Abadia Consuelo M. S. GomideEliana ToledoSolange Oliveira Rodrigues ValleRegis A. CamposAlfeu T. FrançaNieves Prior GomezHeitor Franco Andrade Jr.Teresa CaballeroAnete S. GrumachOBJECTIVE: Hereditary angioedema is a serious medical condition caused by a rare autosomal dominant genetic disorder and it is associated with deficient production or dysfunction of the C1 esterase inhibitor. In most cases, affected patients experience unexpected and recurrent crises of subcutaneous, gastrointestinal and laryngeal edema. The unpredictability, intensity and other factors associated with the disease impact the quality of life of hereditary angioedema patients. We evaluated the quality of life in Brazilian hereditary angioedema patients. METHODS: Patients older than 15 years with any severity of hereditary angioedema and laboratory confirmation of C1 inhibitor deficiency were included. Two questionnaires were used: a clinical questionnaire and the SF-36 (a generic questionnaire). This protocol was approved by the Ethics Committee of Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. RESULTS: The SF-36 showed that 90.4% (mean) of all the patients had a score below 70 and 9.6% had scores equal to or higher than 70. The scores of the eight dimensions ranged from 51.03 to 75.95; vitality and social aspects were more affected than other arenas. The internal consistency of the evaluation was demonstrated by a Cronbach's alpha value above 0.7 in seven of the eight domains. CONCLUSIONS: In this study, Brazilian patients demonstrated an impaired quality of life, as measured by the SF-36. The most affected domains were those related to vitality and social characteristics. The generic SF-36 questionnaire was relevant to the evaluation of quality of life; however, there is a need for more specific instruments for better evaluation.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322013000100014Hereditary AngioedemaQuality of LifeQuestionnairesSF-36Asphyxia |
| spellingShingle | Maria Abadia Consuelo M. S. Gomide Eliana Toledo Solange Oliveira Rodrigues Valle Regis A. Campos Alfeu T. França Nieves Prior Gomez Heitor Franco Andrade Jr. Teresa Caballero Anete S. Grumach Hereditary angioedema: quality of life in Brazilian patients Clinics Hereditary Angioedema Quality of Life Questionnaires SF-36 Asphyxia |
| title | Hereditary angioedema: quality of life in Brazilian patients |
| title_full | Hereditary angioedema: quality of life in Brazilian patients |
| title_fullStr | Hereditary angioedema: quality of life in Brazilian patients |
| title_full_unstemmed | Hereditary angioedema: quality of life in Brazilian patients |
| title_short | Hereditary angioedema: quality of life in Brazilian patients |
| title_sort | hereditary angioedema quality of life in brazilian patients |
| topic | Hereditary Angioedema Quality of Life Questionnaires SF-36 Asphyxia |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322013000100014 |
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