ABSTRACT
Background and Objectives: Prion diseases are fatal neurodegenerative disorders caused by the abnormal proteinase K-resistant prion protein (PrPSc). Since variant Creutzfeldt-Jakob disease (CJD) was first reported in the United Kingdom (UK) in 1996, the occurrence of variant CJD has been reported in over 10 countries. To date, variant CJD has not been reported in Korea. However, the E211K somatic mutation in the prion protein gene (PRNP), which is related to bovine spongiform encephalopathy (BSE), was reported in Korean Holstein cattle, and atypical BSE, which is supposed to be sporadic BSE, has been occurring in many countries, including Japan and the USA. These results suggest that BSE may occur naturally in Korea. Thus, we performed a preemptive PrPSc test in appendix specimens to diagnose variant CJD in a Korean population. Materials and Methods: In the present study, we investigated CJD-related mutations and polymorphisms of the PRNP gene and carried out an examination on PrPSc in appendix specimens of Korean patients after appendectomy. Results: In all Korean appendix specimens tested, PrPSc bands were not detected. Conclusion: To the best of our knowledge, this was the first evaluation of PrPSc in Korean appendix specimens.
Subject(s)
Appendix , Creutzfeldt-Jakob Syndrome , Encephalopathy, Bovine Spongiform , Prion Diseases , Prions , Animals , Appendix/metabolism , Cattle , Creutzfeldt-Jakob Syndrome/genetics , Creutzfeldt-Jakob Syndrome/metabolism , Encephalopathy, Bovine Spongiform/metabolism , Endopeptidase K , Prion Diseases/genetics , Prion Proteins/genetics , Prions/genetics , Prions/metabolismABSTRACT
A 23-year-old Korean female presented epigastric pain of two-months' duration. She had a laparoscopic ovarian cyst excision 8 months previously. Clinical examination was normal. An abdominal computed tomogram (CT) demonstrated a 10-cm solid mass in the distal pancreas, with signs of splenic artery and vein occlusion, gastric and transverse colon invasion. Operative findings showed a mass involving distal pancreas, invasive to the posterior wall of the antrum of the stomach and transverse colon and 4th portion of the duodenum without lymph node involvement. The surgery consisted of a distal pancreatectomy, splenectomy and combined partial resection of the stomach, transverse colon and 4th portion of the duodenum. The immunohistochemistry and histopathological features were consistent with a confirmed diagnosis of intra-abdominal desmoid type fibromatosis (DTF). The prognosis of pancreatic DTF is not known and she showed no recurrence or distant metastasis during a 3 year follow-up. Herein we report a rare case with an isolated, sporadic, and non-trauma-related DTF, located at the pancreatic body and tail.
ABSTRACT
BACKGROUNDS/AIMS: Pancreatic leakage is a major cause of postoperative death and morbidity after pancreaticoduodenectomy (PD). A recent study introduced Blumgart anastomosis (BA), which minimizes severe complications after PD. This study compares BA with conventional anastomosis (CA) for pancreaticojejunostomy (PJ) after PD at a single institution. METHODS: A total of 87 patients who underwent PD at our hospital between January 2003 and October 2015 were enrolled in this study. The patients were divided into two groups according to the anastomosis type. Of them, 44 patients underwent anastomosis using CA (group A, conventional duct-to-mucosa anastomosis) and 43 underwent anastomosis using BA (group B, Blumgart anastomosis). RESULTS: There was a significant difference in duration of the operation between groups A and B (473.1±102.0 versus 386.4±58.5 min, p<0.001) and intraoperative transfusion (2.2±2.7 versus 0.7±1.5 units, p<0.001). There was no significant difference between groups A and B in incidence of postoperative pancreatic fistula (POPF) (43.2% versus 27.9%, p=0.137) ,postoperative hemorrhage (PPH) (13.7% versus 7.0%, p=0.209), delayed gastric emptying (DGE) (29.5% versus 9.3%, p=0.063), surgical and non-surgical complications (60.5% versus 59.1%, p=0.896), length of ICU stay (9.0±6.3 versus 7.4±7.2 days, p=0.099), or length of postoperative hospital stay (37.7±16.7 versus 41.6±15.1 days, p=0.118). CONCLUSIONS: The results of this study suggest that BA-type PJ is not inferior to CA-type PJ in terms of postoperative complications.
