Síndrome de Wiskott-Aldrich en España: incidencia, mortalidad y sesgo de género durante 21 años

Abstract

BackgroundWiskott-Aldrich syndrome (WAS) is a rare X-linked disorder considered to predominantly affect males. ObjectiveThis study aims to investigate the incidence and intrahospital death associated with WAS in Spain as well as the gender bias. MethodsA 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. ResultsOur results revealed that the mean annual incidence of WAS in Spain was 1.1 per 10,000,000 inhabitants (CI95% 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% inWAS, being most of the deaths associated with brain hemorrhage or infection. ConclusionsWAS, a rare disease, is diagnoses at later ages in women and the mortality wasfound in males mostly associated with brain hemorrhage and infection.