ABSTRACT
Introducción y objetivos: La disección de la aorta torácica (DAT) es infrecuente en jóvenes y presenta características diferentes que en la población adulta. En este estudio se analizan las características clinicopatológicas de la muerte súbita por DAT de personas de 1-35 años. Métodos: Estudio poblacional multicéntrico basado en autopsias forenses realizadas en las provincias de Vizcaya (periodo 1991-2016), Valencia (2000-2016) y Sevilla (2004-2016). Resultados: Se recogieron 35 casos (el 80% varones), con una media de edad de 29+/-5 años. La incidencia fue de 0,09/100.000 habitantes/año; 18 sujetos tenían algún factor de riesgo y 29, 1 o más factores de riesgo o hallazgos post mortem asociados con DAT: cardiopatía congénita (n=16), sospecha de DAT familiar (n=11), consumo de cocaína (n=6) e hipertensión arterial (n=5). En 24 casos, 12 con algún factor de riesgo, ya habían presentado síntomas previamente; 16 acudieron al médico, pero nunca hubo sospecha de DAT. El dolor torácico (n=12) fue el síntoma más frecuente. Los hallazgos autópsicos principales fueron: degeneración quística de la media (n=27), dilatación de la aorta ascendente (n=21), cardiomegalia (n=20) y válvula aórtica bicúspide (n=14). Conclusiones: La incidencia de muerte súbita por DAT fue muy baja. Los factores de riesgo más frecuentes fueron las cardiopatías congénitas, seguidas de la sospecha de DAT familiar y el consumo de cocaína. Se debería incluir la DAT en el diagnóstico diferencial del dolor torácico en jóvenes, principalmente varones, con factores de riesgo asociados
Introduction and objectives: Thoracic aortic dissection (TAD) is infrequent in young people and its characteristics differ from those in the adult population. This study aimed to analyze the clinical and pathological characteristics of sudden death due to TAD in people aged 1 to 35 years. Methods: Multicenter population-based study based on forensic autopsies conducted in the provinces of Biscay (1991-2016), Valencia (2000-2016), and Seville (2004-2016). Results: We identified 35 individuals with sudden death due to TAD (80% males), with a mean age of 29+/-5 years. The incidence was 0.09/100 000 inhabitants/y. Eighteen persons had at least 1 risk factor for TAD, and this figure increased to 29 when postmortem findings were included: congenital heart disease (n=16), suspicion of familial TAD (n=11), cocaine use (n=6), and hypertension (n=5). Twenty-four individuals, 12 with at least 1 risk factor, had prodromal symptoms, and 16 of them visited their physician, but TAD was not suspected in any of them. The most frequent symptom was chest pain (n=12). The main autopsy findings were cystic degeneration of the media (n=27), dilatation of the ascending aorta (n=21), cardiac hypertrophy (n=20), and bicuspid aortic valve (n=14). Conclusions: The incidence of sudden death due to TAD in young people was very low. The most frequent risk factors were congenital heart disease followed by suspicion of familial TAD and cocaine use. TAD should be included in the differential diagnosis of chest pain in young people, mainly male patients with at least 1 risk factor
Subject(s)
Humans , Male , Female , Young Adult , Adult , Aortic Dissection/mortality , Death, Sudden, Cardiac/epidemiology , Aortic Aneurysm/mortality , Autopsy/statistics & numerical data , Diagnosis, Differential , Chest Pain/etiology , Forensic Pathology/methodsABSTRACT
INTRODUCTION AND OBJECTIVES: Thoracic aortic dissection (TAD) is infrequent in young people and its characteristics differ from those in the adult population. This study aimed to analyze the clinical and pathological characteristics of sudden death due to TAD in people aged 1 to 35 years. METHODS: Multicenter population-based study based on forensic autopsies conducted in the provinces of Biscay (1991-2016), Valencia (2000-2016), and Seville (2004-2016). RESULTS: We identified 35 individuals with sudden death due to TAD (80% males), with a mean age of 29±5 years. The incidence was 0.09/100 000 inhabitants/y. Eighteen persons had at least 1 risk factor for TAD, and this figure increased to 29 when postmortem findings were included: congenital heart disease (n=16), suspicion of familial TAD (n=11), cocaine use (n=6), and hypertension (n=5). Twenty-four individuals, 12 with at least 1 risk factor, had prodromal symptoms, and 16 of them visited their physician, but TAD was not suspected in any of them. The most frequent symptom was chest pain (n=12). The main autopsy findings were cystic degeneration of the media (n=27), dilatation of the ascending aorta (n=21), cardiac hypertrophy (n=20), and bicuspid aortic valve (n=14). CONCLUSIONS: The incidence of sudden death due to TAD in young people was very low. The most frequent risk factors were congenital heart disease followed by suspicion of familial TAD and cocaine use. TAD should be included in the differential diagnosis of chest pain in young people, mainly male patients with at least 1 risk factor.
Subject(s)
Aorta/pathology , Aortic Aneurysm, Thoracic/complications , Aortic Dissection/complications , Death, Sudden/epidemiology , Adolescent , Adult , Aortic Dissection/diagnosis , Aortic Aneurysm, Thoracic/diagnosis , Autopsy , Child , Child, Preschool , Death, Sudden/etiology , Death, Sudden/pathology , Female , Humans , Incidence , Infant , Male , Spain , Young AdultABSTRACT
Peripheral inflammation contributes to minimal hepatic encephalopathy in chronic liver diseases, which could be mediated by neuroinflammation. Neuroinflammation in cerebellum of patients with chronic liver diseases has not been studied in detail. Our aim was to analyze in cerebellum of patients with different grades of liver disease, from mild steatohepatitis to cirrhosis and hepatic encephalopathy: (a) neuronal density in Purkinje and granular layers; (b) microglial activation; (c) astrocyte activation; (d) peripheral lymphocytes infiltration; (e) subtypes of lymphocytes infiltrated. Steatohepatitis was classified as SH1, SH2 and SH3. Patients with SH1 show Th17 and Tfh lymphocytes infiltration in the meninges, microglia activation in the molecular layer and loss of 16 ± 4% of Purkinje and 19 ± 2% of granular neurons. White matter remains unaffected. With the progression of liver disease to worse stages (SH2, SH3, cirrhosis) activation of microglia and astrocytes extends to white matter, Bergman glia is damaged in the molecular layer and there is a further loss of Purkinje neurons. The results reported show that neuroinflammation in cerebellum occurs at early stages of liver disease, even before reaching cirrhosis. Neuroinflammation occurs earlier in the molecular layer than in white matter, and is associated with infiltration of peripheral Th17 and Tfh lymphocytes.
