no abstract available.

A printing error was identified in the figure 3.

Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease. In cases of PSC, liver transplantation is the only effective treatment that can delay the disease's natural course. We report a case of rapidly progressive PSC requiring liver transplantation. A 52-year-old woman visited our hospital with abdominal pain. There was no evidence of PSC, as there was no elevation in cholestatic liver enzymes at her first visit. Although her total bilirubin was in a normal range at the initial visit, liver dysfunction progressed rapidly. Despite endoscopic procedures and ursodeoxycholic acid intake, total bilirubin levels rose to 18.9 mg/dL, and liver transplantation was performed 17 months after her first visit. PSC was pathologically confirmed after liver transplantation.
Abdominal Pain ; Bilirubin ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Cholangitis, Sclerosing ; Female ; Humans ; Liver ; Liver Diseases ; Liver Transplantation ; Reference Values ; Ursodeoxycholic Acid

Malignant hilar biliary obstruction (MHBO) frequently accompanies cholestasis and cholangitis, and requires biliary stent placement. To prevent stent occlusion and prolong survival, local ablation therapy can be considered adjunctive to stent placement. Intraductal radiofrequency ablation (ID-RFA) is a recently developed local therapy for malignant biliary obstruction that can be easily performed employing endoscopic retrograde cholangiography. The use of ID-RFA to treat MHBO (as distinct from distal biliary obstruction) was suggested to be associated with severe adverse events. However, recent comparative studies have shown that ID-RFA is feasible and safe, and acceptably efficacious, in patients with advanced MHBO; newer temperature-controlled ID-RFA devices may enhance safety further. Regularly repeated ID-RFA with stent exchange affords better survival than stenting alone. However, the optimal ID-RFA strategy for MHBO remains inconclusive given the lack of data. Further large-scale clinical trials are needed.

Nesidioblastosis is a term used to describe pathologic overgrowth of pancreatic islet cells. It also means maldistribution of islet cells within the ductules of exocrine pancreas. Generally, nesidioblastosis occurs in beta-cell and causes neonatal hyperinsulinemic hypoglycemia or adult noninsulinoma pancreatogenous hypoglycemia syndrome. Alpha-cell nesidioblastosis and hyperplasia is an extremely rare disorder. It often accompanies glucagon-producing marco- and mircoadenoma without typical glucagonoma syndrome. A 35-year-old female was referred to our hospital with recurrent acute pancreatitis. On radiologic studies, 1.5 cm sized mass was noted in pancreas tail. Cytological evaluation with EUS-fine-needle aspiration suggested serous cystadenoma. She received distal pancreatectomy. The histologic examination revealed a 1.7 cm sized neuroendocrine tumor positive for immunohistochemical staining with glucagon antibody. Multiple glucagon-producing micro endocrine cell tumors were scattered next to the main tumor. Additionally, diffuse hyperplasia of pancreatic islets and ectopic proliferation of islet cells in centroacinar area, findings compatible to nesidioblastosis, were seen. These hyperplasia and almost all nesidioblastic cells were positive for glucagon immunochemistry. Even though serum glucagon level still remained higher than the reference value, she has been followed-up without any evidence of recurrence or hormone related symptoms. Herein, we report a case of alpha-cell nesidioblastosis and hyperplasia combined with glucagon-producing neuroendocrine tumor with literature review.
Adult ; Chromogranin A/blood ; Female ; Glucagon/*metabolism ; Glucagon-Secreting Cells/metabolism ; Humans ; Hyperplasia/complications/*diagnosis ; Islets of Langerhans/metabolism/ultrasonography ; Nesidioblastosis/complications/*diagnosis ; Neuroendocrine Tumors/complications/*diagnosis/pathology ; Pancreas/*pathology ; Tomography, X-Ray Computed

Hereditary spherocytosis is a disease caused by deficiency of erythrocyte lipid membrane protein. Hereditary spherocytosis shows hemolysis of erythrocyte, and it leads to anemia, jaundice by elevation of indirect bilirubin. Almost of patients are diagnosed in their infancy, and can be cured by splenectomy about their age 6–7. Herein, we report a rare case of 33-year-old male was suffered from gallbladder stone and cholangitis those are thought to be the late complications of hereditary spherocytosis. We performed endoscopic retrograde cholangiopancreaticography to remove common bile duct stones. After he got cholecystectomy and splenectomy, there was no recurrence of choledocholithiasis. This is the first case in Korea who didn’t undergo splenectomy until grown up, shows cholangitis as a late disease manifestation of hereditary spherocytosis.