Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation

Ralph J. Fuchs, Jay Levin, Meghan Tadel, William Merritt – 24 April 2007 – Afibrinogenemia is a rare hereditary coagulation disorder characterized by a propensity toward bleeding. A 21‐year‐old Hispanic woman with afibrinogenemia developed ascites, a distended abdomen, an enlarged liver, scleral icterus, and umbilical vein and abdominal wall vein distension. Computed tomography (CT) scan showed occlusion of the hepatic veins and the infrahepatic vena cava consistent with Budd‐Chiari syndrome.

Nonanastomotic biliary strictures after liver transplantation, part 2: Management, outcome, and risk factors for disease progression

Robert C. Verdonk, Carlijn I. Buis, Eric J. van der Jagt, Annette S. H. Gouw, Abraham J. Limburg, Maarten J. H. Slooff, Jan H. Kleibeuker, Robert J. Porte, Elizabeth B. Haagsma – 24 April 2007 – Nonanastomotic biliary strictures (NAS) after orthotopic liver transplantation (OLT) are associated with high retransplant rates. The aim of the present study was to describe the treatment of and identify risk factors for radiological progression of bile duct abnormalities, recurrent cholangitis, biliary cirrhosis, and retransplantation in patients with NAS.

Nonanastomotic biliary strictures after liver transplantation, part 1: Radiological features and risk factors for early vs. Late presentation

Carlijn I. Buis, Robert C. Verdonk, Eric J. Van der Jagt, Christian S. van der Hilst, Maarten J. H. Slooff, Elizabeth B. Haagsma, Robert J. Porte – 24 April 2007 – Nonanastomotic biliary strictures (NAS) are a serious complication after orthotopic liver transplantation (OLT). The exact pathogenesis is unclear. Purpose of this study was to identify risk factors for the development of NAS after OLT. A total of 487 adult liver transplants with a median follow‐up of 7.9 years were studied. All imaging studies of the biliary tree were reviewed.

Pediatric hepatopulmonary syndrome is seen with polysplenia/interrupted inferior vena cava and without cirrhosis

Nitika Arora Gupta, Carlos Abramowsky, Todd Pillen, Douglas Redd, Carlos Fasola, Thomas Heffron, Rene Romero – 24 April 2007 – Hepatopulmonary syndrome (HPS) is a triad of liver dysfunction, hypoxemia, and intrapulmonary vascular dilatation. We describe the prevalence and clinical features of HPS at a pediatric liver transplant center. Patients referred to Children's Healthcare of Atlanta/Emory University transplant program from February 1999 to May 2005 were reviewed. Oxygen saturation in room air was screened by percutaneous pulse oximetry.

Combined liver‐kidney transplantation in glycogen storage disease Ia: A case beyond the guidelines

Mirco Belingheri, Luciana Ghio, Ambra Sala, Francesca Menni, Laura Trespidi, Mariano Ferraresso, Luisa Berardinelli, Giorgio Rossi, Alberto Edefonti, Rossella Parini – 24 April 2007 – Glycogen storage disease type Ia (GSD Ia) is a rare metabolic disorder due to hepatic glucose‐6‐phosphatase deficiency. Although great progress has been made in managing affected patients, severe hypoglycemia, lactic acidosis, hyperlipidemia, hepatic cytolysis, and impaired kidney function are frequent.

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