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.

Historical analysis of experience with small bowel capsule endoscopy in a spanish tertiary hospital. / Análisis histórico de la experiencia en cápsula endoscópica de intestino delgado en un hospital terciario español.

INTRODUCTION: Capsule endoscopy was approved by the FDA in 2001. Gastrointestinal bleeding and inflammatory bowel disease are the main indications. It has been available in our hospital since 2004. METHODS: We retrospectively analysed data from patients who underwent small bowel capsule endoscopy in our hospital from October 2004 to April 2015. Indications were divided into: Obscure gastrointestinal bleeding (occult and overt), inflammatory bowel disease, and other indications. Findings were divided into: Vascular lesions, inflammatory lesions, other lesions, normal studies, and inconclusive studies. RESULTS: A total of 1027 out of 1291 small bowel studies were included. Mean patient age was 56.45 years; 471 were men and 556 women. The most common lesion observed was angiectasia, as an isolated finding or associated with other lesions. Findings were significant in up to 80% of studies when the indication was gastrointestinal bleeding, but in only 50% of studies in inflammatory bowel disease. Diagnostic yield was low in the group «other indications¼. No major complications were reported. DISCUSSION: Small bowel capsule endoscopy has high diagnostic yield in patients with gastrointestinal bleeding, but yield is lower in patients with inflammatory bowel disease. CONCLUSIONS: Our experience shows that capsule endoscopy is a safe and useful tool for the diagnosis of small bowel disease. The diagnostic yield of the technique in inflammatory bowel disease must be improved.

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.