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Vol. 223. Issue 5.
Pages 262-269 (May 2023)
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Vol. 223. Issue 5.
Pages 262-269 (May 2023)
Original article
21 years of Wiskott-Aldrich syndrome in Spain: incidence, mortality, and gender bias
Síndrome de Wiskott-Aldrich en España: incidencia, mortalidad y sesgo de género durante 21 años
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A. Guerrero Espejoa,
Corresponding author
guerreroespejo@gmail.com

Corresponding author.
, S. Tomás Dolsa, M.C. Gestalb
a Grupo de Investigación de Enfermedades Infecciosas, Faculty of Medicine and Dentistry, Catholic University of Valencia "San Vicente Mártir", Valencia, Spain
b Department of Microbiology and Immunology, LSU Health, Shreveport, LA, USA
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Figures (1)
Tables (3)
Table 1. Demographics of the admitted patients suffering WAS.
Table 2. Mean annual incidence of WAS by autonomous regions/cities with at least 1 case in 10 millions of inhabitants.
Table 3. Bivariant analysis of comorbidities in relation to the mortality of WAS in the hospital.
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Abstract
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.

Keywords:
Wiskott-Aldrich syndrome
Epidemiology
Incidence
Mortality
Female
Spain
Abbreviations:
WAS
WASP
XLT
Resumen
Introducción

El síndrome de Wiskott-Aldrich (SWA) es un trastorno raro ligado al cromosoma X que afecta predominantemente a los hombres.

Objetivo

Este estudio tiene como objetivo investigar la incidencia y muerte intrahospitalaria asociada al SWA en España, así como su sesgo de género.

Métodos

Se realizó un estudio epidemiológico retrospectivo de base poblacional de 97 pacientes con SWA diagnosticados en hospitales españoles entre 1997 y 2017, a través del Sistema Nacional del Conjunto Mínimo Básico de Datos al alta hospitalaria.

Resultados

Nuestros resultados revelaron que la incidencia media anual de SWA en España fue de 1,1 por 10 000 000 habitantes (IC95% 0,45−2,33). El riesgo relativo fue mayor en hombres que en mujeres (2,42). El diagnóstico de SWA se produce a edades más tardías en las mujeres (mediana de edad de 47 años) en comparación con los hombres (mediana de edad de 5,5 años). Solo hombres ingresaron al hospital al menos en 10 ocasiones diferentes y todas las muertes se detectaron en hombres. La tasa de mortalidad intrahospitalaria fue del 9,28% en WAS, siendo la mayoría de las muertes asociadas a hemorragia cerebral o infección.

Conclusiones

El SWA, una enfermedad rara, se diagnosticó en edades más tardías en mujeres y la mortalidad, mayoritariamente asociada a hemorragia cerebral e infección, afectó a hombres.

Palabras clave:
Síndrome de Wiskott-Aldrich
Epidemiología
Incidencia
Mortalidad
Mujer
España

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