This report describes a case of Ebstein anomaly in a fetus with cardiomegaly, severe tricuspid regurgitation, pulmonary regurgitation, and retrograde ductal flow that showed a marked increase in the size of the right atrium with advancing gestational age. Elective preterm delivery was performed at 35 weeks gestation. The prostaglandin E1 infusion resulted in more pronounced systemic hypotension and acidosis secondary to circular shunt across the patent ductus arteriosus as well as pulmonary regurgitation and tricuspid regurgitation. Emergency surgical intervention consisting of main pulmonary artery ligation, ductus arteriosus ligation, central shunt creation, and plication of the right atrium without cardiopulmonary bypass was performed 4 h after birth. At the age of 16 days, the Starnes procedure was performed. The infant's postoperative course was uneventful. A fetus that has Ebstein anomaly associated with pulmonary regurgitation is at risk for circular shunt across the patent ductus arteriosus after delivery. Planned delivery and surgical intervention without delay after birth are useful for the treatment of such cases.
An 11-year-old boy with severe chronic active Epstein-Barr virus infection (CAEBV) underwent successful cord blood transplantation (CBT) after consecutive failure of peripheral blood and bone marrow transplantation from his HLA-mismatched mother. CB cells from an unrelated donor were infused after conditioning with total body irradiation (12 Gy), melphalan (120 mg/m 2 ), and etoposide (600 mg/m 2 ). Complete remission without circulating EBV-DNA has continued for 15 months after a delayed hematologic recovery. This is the first successful report of CBT for CAEBV. CB may therefore be an alternate source of stem cells for the curative treatment of CAEBV, despite the absence of EBV-specific cytotoxic T lymphocytes. Am.
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