São Paulo medical journal = Revista paulista de medicina
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Review Case Reports
Primary aortoenteric fistula related to septic aortitis.
Primary aortoenteric fistulas usually result from erosion of the bowel wall due to an associated abdominal aortic aneurysm. A few patients have been described with other etiologies such as pseudoaneurysm originating from septic aortitis caused by Salmonella. ⋯ A 65-year-old woman was admitted with Salmonella bacteremia that evolved to septic aortitis. An aortic pseudoaneurysm secondary to the aortitis had eroded the transition between duodenum and jejunum, and an aortoenteric fistula was formed. In the operating room, the affected aorta and intestinal area were excised and an intestine-to-intestine anastomosis was performed. The aorta was sutured and an axillofemoral bypass was carried out. In the intensive care unit, the patient had a cardiac arrest that evolved to death.
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Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. ⋯ The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.