Liver transplantation in patients with type IIIa glycogen storage disease, cirrhosis and hepatocellular carcinoma.

Type III glycogen storage disease (GSD-III) is an autosomal recessive disorder due to the deficiency of the glycogen debrancher enzyme. 80% of the patients have hepatic and muscular involvement (IIIa), compared to 15% with only liver involvement (IIIb). As the life expectancy improves in these patients, the possible liver complications are better understood.

Giant intra-abdominal liposarcoma.

We report the case of a 67-year-old male with epigastric pain and weight loss during the last nine months. Physical examination revealed a hard palpable mass in the epi-mesogastrium. An abdominal ultrasound identified a large, heterogeneous and hypovascular mass, which compressed the left hepatic lobe and the pancreas.

Hepatic encephalopathy secondary to a splenorenal shunt that manifested a long time after a liver transplantation.

Splenorenal shunts are a rare cause of hyperammonemia and hepatic encephalopathy in the absence of cirrhosis. We report the case of a woman, who presented hepatic encephalopathy, with a normal functioning graft, after 14 years of liver transplantation, confirmed by liver biopsy.

Spontaneous retroperitoneal hematoma in a patient under anticoagulant agents presenting as upper gastrointestinal bleeding.

We present the case of a 44-year-old woman with past history of repeated miscarriage and Budd-Chiari syndrome secondary to primary myelofibrosis. Because of this she was under treatment with oral anticoagulant agents. The patient was admitted in hospital as she presented with gastrointestinal bleeding (melena), asthenia and progressive anemia. In an initial upper endoscopy an extrinsic duodenal compression associated with an ulcer on the posterior face of the first portion of duodenum and upper duodenal knee was observed. In the following days a huge spontaneous retroperitoneal hematoma due to anticoagulation was diagnosed by computed tomography. This was treated with a percutaneous drainage and withdrawal of the antithrombotic drugs. The evolution of the patient was initially satisfactory but she suffered subclavian and jugular vein thrombosis, and reintroduction of anticoagulant agents at the lowest therapeutic doses was required.