Congenital porto-systemic shunts are increasingly recognized in several settings and at any age. The following are some of the most common presentations: prenatal ultrasound, neonatal cholestasis, incidental finding on abdominal imaging, or systemic complications such as unexplained cardiopulmonary or neurological disease, or the presence of liver nodules in a non-cirrhotic liver. The purpose of the present review is to summarize clinical presentation and current recommendations for management, and highlight areas of future research. Illustrative examples from the veterinary literature complement our current lack of knowledge of this rare malformation often masquerading as a multisystem disease. Address correspondence and reprint requests to Valérie A. McLin, MD, Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology and Nutrition Unit, University Hospitals Geneva and Geneva University, Geneva, Switzerland (e-mail: valerie.mclin@hcuge.ch). Received 12 July, 2018 Accepted 12 December, 2018 The authors report no conflicts of interest. © 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,
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