Please use this identifier to cite or link to this item:
Title: Gender differences in Hereditary Hemorrhagic Telangiectasia severity
Author: Mora Luján, José María
Iriarte, Adriana
Alba, Esther
Sánchez Corral, M. A.
Cerdà, Pau
Cruellas, Francesc
Ordi, Q.
Corbella, Xavier
Ribas, J.
Castellote Alonso, José
Riera Mestre, Antoni
Keywords: Malalties rares
Rare diseases
Issue Date: 24-Feb-2020
Publisher: BioMed Central
Abstract: Background: Gender differences in organ involvement and clinical severity have been poorly described in hereditary hemorrhagic telangiectasia (HHT). The aim of this study was to describe differences in the severity of HHT manifestations according to gender. Methods: Severity was measured according to Epistaxis Severity Score (ESS), Simple Clinical Scoring Index for hepatic involvement, a general HHT-score, needing for invasive treatment (pulmonary or brain arteriovenous malformations -AVMs- embolization, liver transplantation or Young's surgery) or the presence of adverse outcomes (severe anemia, emergency department -ED- or hospital admissions and mortality). Results: One hundred forty-two (58.7%) women and 100 (41.3%) men were included with a mean age of 48.9 ± 16.6 and 49 ± 16.5 years, respectively. Women presented hepatic manifestations (7.1% vs 0%) and hepatic involvement (59.8% vs 47%), hepatic AVMs (28.2% vs 13%) and bile duct dilatation (4.9% vs 0%) at abdominal CT, and pulmonary AVMs at thoracic CT (35.2% vs 23%) more often than men. The Simple Clinical Scoring Index was higher in women (3.38 ± 1.2 vs 2.03 ± 1.2), and more men were considered at low risk of harboring clinically significant liver disease than women (61% vs 25.3%). These differences were mantained when considering HHT1 and HHT2 patients separetely. Duodenal telangiectasia were more frequent in men than women (21% vs 9.8%). Invasive treatments were more frequently needed in women (28.2% vs 16%) but men needed attention at the ED more often than women (48% vs 28.2%), with no differences in ESS, HHT-score, anemia hospital admissions or mortality. Conclusions: HHT women showed more severe hepatic involvement than men, also among HHT1 and HHT2 patients. Women had higher prevalence of pulmonary AVMs and needed invasive procedures more frequently, while men needed attention at the ED more often. These data might help physicians to individualize HHT patients follow-up.
Note: Reproducció del document publicat a:
It is part of: Orphanet Journal of Rare Diseases, 2020, vol. 15, p. 63
Related resource:
ISSN: 1750-1172
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Ciències Clíniques)

Files in This Item:
File Description SizeFormat 
699249.pdf563.08 kBAdobe PDFView/Open

This item is licensed under a Creative Commons License Creative Commons