21 years of Wiskott-Aldrich syndrome in Spain : incidence, mortality, and gender bias
Copyright © 2023. Published by Elsevier España, S.L.U..
BACKGROUND: Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder considered to predominantly affect males.
OBJECTIVE: This study aims to investigate the incidence and intrahospital death associated with WAS in Spain as well as the gender bias.
METHODS: A population-based retrospective epidemiological study of 97 WAS patients that were diagnosed in Spanish hospitals between 1997 and 2017 was conducted by using data from the National Surveillance System for Hospital Data.
RESULTS: Our results revealed that the mean annual incidence of WAS in Spain was 1.1 per 10,000,000 inhabitants (IC95% 0,45-2,33). The relative risk was higher in male than female (2.42). WAS diagnosis occurs at later ages in women (median age of 47 years) compared to men (median age of 5.5 years). Only male were admitted to the hospital at least in 10 different occasions and all deaths were detected in men. The intra-hospital death rate was of 9.28% in WAS, being most of the deaths associated with brain hemorrhage or infection.
CONCLUSIONS: WAS, a rare disease, is diagnoses at later ages in women and the mortality was found in males mostly associated with brain hemorrhage and infection.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:223 |
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| Enthalten in: |
Revista clinica espanola - 223(2023), 5 vom: 08. Mai, Seite 262-269 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Guerrero Espejo, A [VerfasserIn] |
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| Links: |
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| Themen: |
Epidemiología |
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| Anmerkungen: |
Date Completed 03.05.2023 Date Revised 12.05.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.rceng.2023.03.004 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM354777610 |
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| 500 | |a Date Revised 12.05.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2023. Published by Elsevier España, S.L.U. | ||
| 520 | |a BACKGROUND: Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder considered to predominantly affect males | ||
| 520 | |a OBJECTIVE: This study aims to investigate the incidence and intrahospital death associated with WAS in Spain as well as the gender bias | ||
| 520 | |a METHODS: A population-based retrospective epidemiological study of 97 WAS patients that were diagnosed in Spanish hospitals between 1997 and 2017 was conducted by using data from the National Surveillance System for Hospital Data | ||
| 520 | |a RESULTS: Our results revealed that the mean annual incidence of WAS in Spain was 1.1 per 10,000,000 inhabitants (IC95% 0,45-2,33). The relative risk was higher in male than female (2.42). WAS diagnosis occurs at later ages in women (median age of 47 years) compared to men (median age of 5.5 years). Only male were admitted to the hospital at least in 10 different occasions and all deaths were detected in men. The intra-hospital death rate was of 9.28% in WAS, being most of the deaths associated with brain hemorrhage or infection | ||
| 520 | |a CONCLUSIONS: WAS, a rare disease, is diagnoses at later ages in women and the mortality was found in males mostly associated with brain hemorrhage and infection | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Epidemiology | |
| 650 | 4 | |a Epidemiología | |
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| 650 | 4 | |a Female | |
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| 650 | 4 | |a Mortalidad | |
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