Síndrome de Wiskott-Aldrich en España: incidencia, mortalidad y sesgo de género durante 21 años / 21 years of Wiskott-Aldrich syndrome in Spain: incidence, mortality, and gender bias

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 (AU)

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 (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. Conclusions WAS, a rare disease, is diagnoses at later ages in women and the mortality wasfound in males mostly associated with brain hemorrhage and infection (AU)

21 years of Wiskott-Aldrich syndrome in Spain: incidence, mortality, and gender bias.

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.

Prevención de la transmisión intrauterina del virus de la hepatitis B en procedimientos invasivos / Prevention of intrauterine hepatitis B virus infection in invasive procedures: a case report

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Resistencia antibiótica de Streptococcus pyogenes: ¿qué está pasando? / Antibiotic resistance to Streptococus pyogenes: what´s happening?

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[The risk factors associated with colonization and bacteremia in non-tunnelled central venous catheters]. / Factores de riesgo asociados a colonización y bacteriemia en catéteres venosos centrales no tunelizados.

OBJECTIVE: To identify risk factors for colonization and bacteremia among patients with non-tunnelled central venous catheters. MATERIALS AND METHODS: A prospective study was conducted of a cohort of patients carrying non-tunnelled central venous catheters. Different parameters were obtained and the degree of its association with colonization of the distal portion of the catheter or with bacteremia associated with colonization was estimated. The CDC (centers for Disease Control) diagnostic criteria of colonization and catheter-related bacteremia were used. RESULTS: A total of 118 catheters were eventually analyzed, corresponding to 114 patients, with a catheterization mean time of 14 +/- 8 days (mean +/- SD); out of these 114 patients, 51 were colonized and in 22 the presence of associated bacteremia was confirmed. The parameters associated with a higher risk for catheter colonization included length of colonization, femoral location, number of lumina and a vital prognosis lower than one month. All these factors, with the exception of the increase in the number of lumina, showed an independent association with colonization on the multivariate analysis [catheterization length (in weeks): OR 1.46; 95% CI: 1.0-2.11; femoral location: OR 3.73; 95% CI: 1.16-11.9; vital prognosis lower than one month: OR 12.7; 95% CI: 1.4-112.7]. As for risk for catheter-related bacteremia, the univariate analysis showed an association with catheterization length and a vital prognosis lower than one month; the latter was the only factor that maintained an independent association in the multivariate analysis (OR 5.75; 95% CI: 1.17-28.27). CONCLUSION: The present study documents the relevance of prolonged catheterization as a consistent risk for colonization of non-tunnelled central venous catheters. This risk increases independently in canalization at femoral site and particularly among severely ill patients. The presence of these factors allows the identification of a high risk population for the development of catheter related bacteremia.

Factores de riesgo asociados a colonización y bacteriemia en catéteres venosos centrales no tunelizados / Risk factors associated with colonization and bacteremia in non-tunnelled central venous catheters

Objetivo. Identificar factores de riesgo de colonización y de bacteriemia en pacientes con catéteres venosos centrales no tunelizados. Material y métodos. Se estudió de forma prospectiva una cohorte de pacientes portadores de catéteres venosos centrales no tunelizados. Se recogieron diferentes variables y se calculó la magnitud de su asociación con la colonización del segmento distal del catéter o con bacteriemia asociada a dicha colonización. Se utilizaron los criterios diagnósticos de colonización y bacteriemia asociada a catéter establecidos por los Centers for Diseases and Control. Resultados. Se analizaron finalmente 118 catéteres, correspondientes a 114 pacientes, cuya media de cateterización fue de 14 ñ 8 días (media ñ DE), de los que 51 resultaron colonizados y de los que en 22 se confirmó la presencia de bacteriemia asociada. Las variables asociadas con un mayor riesgo de colonización del catéter fueron la duración de cateterización, la localización femoral, el número de luces y un pronóstico vital inferior a un mes; todos ellos, salvo el incremento en el número de luces, demostraron una asociación independiente con colonización en el análisis multivariante [duración de cateterización (en semanas): OR 1,46; IC95 por ciento: 1,0-2,11; localización femoral: OR 3,73; IC95 por ciento: 1,16-11,9; pronóstico vital inferior a un mes: OR 12,7; IC95 por ciento: 1,4-112,7]. En relación al riesgo de bacteriemia asociada a catéter, el análisis univariante demostró asociación con la duración de la cateterización y el pronóstico vital inferior a un mes; este último es el único que se mantuvo asociado de forma independiente en el análisis multivariante (OR 5,75; IC95 por ciento: 1,17-28,27). Conclusión. El presente trabajo confirma la importancia de la cateterización prolongada como un claro riesgo de colonización de los catéteres venosos centrales no tunelizados, incrementándose este riesgo de forma independiente en las canalizaciones a nivel femoral, y sobre todo en los pacientes graves. La presencia de estos factores nos permite identificar una población de alto riesgo para el desarrollo de bacteriemia asociada a catéter (AU)

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Borreliosis de Lyme: una enfermedad que la diagnostica quien piensa en ella / Lyme borreliosis: a disease diagnosed by somebody who thinks of it

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