Severity score for hereditary hemorrhagic telangiectasia
| Autor: | Latino, GA, Kim, H, Nelson, J, Pawlikowska, L, Young, W, Faughnan, ME, Brain Vascular Malformation Consortium HHT Investigator Group |
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| Rok vydání: | 2014 |
| Předmět: |
Adult
Intracranial Arteriovenous Malformations Male Genetics & Heredity congenital hereditary and neonatal diseases and abnormalities Other Medical and Health Sciences Bleeding Arteriovenous Malformation Middle Aged Severity of Illness Index Brain Disorders Cross-Sectional Studies Rare Diseases Telangiectasia otorhinolaryngologic diseases Humans Female Osler-Weber-Rendu Hereditary Hemorrhagic Disease severity Hereditary Hemorrhagic Telangiectasia Retrospective Studies Severity score |
| Zdroj: | Orphanet J Rare Dis, vol 9, iss 1 |
| Popis: | BACKGROUND: A disease severity score in hereditary hemorrhagic telangiectasia (HHT) would be a useful tool for assessing burden of disease and for designing clinical trials. Here, we propose the first known HHT severity score, the HHT-score. METHODS: Demographics and disease characteristics were collected for the first 525 HHT patients recruited to the HHT Project of the Brain Vascular Malformation Consortium (BVMC). HHT-score was calculated based on presence of: organ arteriovenous malformations (maximum 3 points); chronic bleeding (maximum 2 points); and severe organ involvement (maximum 2 points). Points were summed and patients categorized as having mild (0-2), moderate (3-4) or severe (5-7) disease. The occurrence of "any adverse outcome" was evaluated for association with HHT-score categories. RESULTS: The frequency of "any adverse outcome" was significantly different across the three groups (49.6% in mild, 65.8% in moderate and 89.5% in severe, p |
| Databáze: | OpenAIRE |
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