Inflammatory pseudotumor is a benign disease entity, which is histologically composed of inflammatory cells and fibrotic stroma. It is mainly found in the respiratory tract, but it has also been reported in the liver, central nervous system, soft tissues, urological system, and gastrointestinal tract. It is extremely rare in the spleen and there have been no cases of splenic inflammatory pseudotumor associated with autoimmune thyroiditis in Korea. The pathogenesis or etiology is unknown, but hypotheses include infection, autoimmunity, and parenchymal necrosis with hemorrhage. Because inflammatory pseudotumor may mimic malignant tumors on radiologic findings, pathologic confirmation is necessary to distinguish it from lymphoproliferative disorders of the spleen and other malignant tumors. We report a case of inflammatory pseudotumor of the spleen associated with autoimmune thyroiditis in a patient who complained of periumbilical pain.
Autoimmunity ; Central Nervous System ; Gastrointestinal Tract ; Granuloma, Plasma Cell* ; Hemorrhage ; Humans ; Korea ; Liver ; Lymphoproliferative Disorders ; Necrosis ; Respiratory System ; Spleen* ; Thyroiditis, Autoimmune*

Acute extensive ischemic gastritis is an extremely rare disease because the stomach has an abundant submucosal vascular plexus with a dual blood supply from the pancreaticoduodenal and gastroduodenal arteries. Smoking, hypertension, and atherosclerotic vascular diseases can be major risk factors for ischemic gastritis. Acute gastric ischemia presents as an acute abdomen with diarrhea or hematemesis that rapidly progresses to acute peritonitis, irreversible septic shock, and death if untreated. We report a case of acute extensive ischemic gastritis combined with tetraplegia due to cervical myelopathy and extensive atherosclerotic changes of the celiac trunk and abdominal aorta.
Abdomen, Acute ; Aorta, Abdominal ; Arteries ; Diarrhea ; Gastritis* ; Hematemesis ; Hypertension ; Ischemia ; Peritonitis ; Quadriplegia ; Rare Diseases ; Risk Factors ; Shock, Septic ; Smoke ; Smoking ; Spinal Cord Diseases ; Stomach ; Vascular Diseases

Brunner's gland adenoma is a rare benign proliferative lesion developing most commonly in the posterior wall of the duodenum. It is usually small in size and asymptomatic. Depending on its size or location, however, the clinical manifestations of this tumor may be variable from nonspecific symptoms to gastrointestinal bleeding or obstruction. Brunner's gland adenoma in the proximal jejunum is extremely rare. We report a very rare case of giant Brunner's gland adenoma developing in the proximal jejunum which presented as iron deficiency anemia and mimicked intussusceptions on radiologic studies.
Adenoma ; Anemia, Iron-Deficiency ; Duodenum ; Hemorrhage ; Intussusception ; Iron ; Jejunum

Caroli's disease is defined as a communication between congenital cystic dilatation of the bile duct and the biliary system. Caroli's disease accompanied with autosomal dominant polycystic kidney disease is a very rare finding and the differential diagnosis between Caroli's disease with polycystic kidney disease and a polycystic liver is very important. A 67-year-old male patient was admitted to our hospital for hematochezia, and he underwent hemodialysis for chronic renal failure due to his autosomal dominant polycystic kidney disease. On colonoscopy, ulcero-infiltrative tumor was observed in the sigmoid colon. It was diagnosed as adenocarcinoma on tissue biopsy. On abdominal computerized tomography and magnetic resonance cholangiopancreatography, a number of simple cysts was observed in both kidneys and we were able to identify the communication between the dilated intrahepatic ducts and the intrahepatic cystic lesions, and so we diagnosed this patient as having Caroli's disease.
Adenocarcinoma ; Aged ; Bile Ducts ; Biliary Tract ; Biopsy ; Caroli Disease* ; Cholangiopancreatography, Magnetic Resonance ; Colon* ; Colon, Sigmoid ; Colonic Neoplasms* ; Colonoscopy ; Diagnosis, Differential ; Dilatation ; Gastrointestinal Hemorrhage ; Humans ; Kidney ; Kidney Failure, Chronic ; Liver ; Male ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant ; Renal Dialysis

Miller-Dieker syndrome is a multiple malformation syndrome characterized by severe lissencephaly and characteristic facial abnormalities at birth. It is associated with visible or submicroscopic deletions within chromosome 17p13.3 including PAFAH1B1 (LIS1) gene. We report a six-month-old boy who presented with spasm and generalized myoclonic seizures. The patient was born at 40 weeks' gestation to a 36-year-old woman and showed developmental delay without microcephaly or prominent facial abnormality. Magnetic resonance imaging of the brain showed a few gyrus (lissencephaly). High resolution cytogenetic analysis from peripheral blood showed a normal karyotype. However, fluorescence in situ hybridization (FISH) of the metaphase chromosome using Miller-Dieker/ILS probe (Oncor, Gaithersburg, Maryland, USA) revealed only one signal of probe, indicating a microdeletion of 17pl3.3 region including PAFAH1B1 (LIS1) gene. We suggest that FISH 17p13.3 studies should be performed in addition to a standard metaphase analysis in patients with lissencephaly even if facial anomaly is not noted. A confirmatory diagnosis using FISH would be helpful in terms of leading to allow genetic counseling and availability prenatal diagnosis to the family.
Adult ; Brain ; Classical Lissencephalies and Subcortical Band Heterotopias* ; Cytogenetic Analysis ; Diagnosis ; Female ; Fluorescence ; Genetic Counseling ; Humans ; In Situ Hybridization ; Karyotype ; Lissencephaly ; Magnetic Resonance Imaging ; Male ; Maryland ; Metaphase ; Microcephaly ; Parturition ; Pregnancy ; Prenatal Diagnosis ; Seizures ; Spasm

The purpose of this study is to present an actual example for procedures for developing a PBL package based on philosophical backgrounds derived from Problem-based learning. To perform a systemic study on the operations of an intergrated curricula under multi-disciplines, a research team made up of several professors with different academic backgrounds was formed. Among the four situations for the patients with perception-adjustment disorder, especially a procedure for the development of PBL package which can be used in the emergency room situation has been proposed. The little(2000)'s PBL package model has been applied for this study. Tha package includes course objectives, learning objectives, concept map, situation scenario, tutor guide, and evaluation method. It is believed that learning objectives achievement procedures designed as a part of a problem-based learning package development procedures for the nursing of patients with perception-adjustment can be achieved at the same level as the learning objectives for the science of nursing founded by the Korean Nurses Association.
Curriculum ; Emergency Service, Hospital ; Humans ; Learning ; Nursing ; Problem-Based Learning*

Pacemaker-mediated tachycardia (PMT) is a well-known complication of dual-chamber pacemaker with atrioventricular sensing and pacing (DDD) or atrial synchronous, ventricular demand pacemaker (VDD). PMT usually starts with sensing a retrograde P waves linked to ventricular extrasystole with retrograde ventriculoatrial conduction and forms reentrant or circus movement tachycardia with the pacemaker itself as an antegrade limb and the conducting tissue of the heart as a retrograde limb. Recently, a number of pacemaker manufactures have incorporated in their devices a variety of relatively complex algorithms to prevent PMT. Despite these measures, PMT may still occur because of inappropriate programming or unpredictable variations of ventri-culoatrial conduction. We report two cases of PMT in 78 year-old man and 60 year-old man who received DDD type pacemakers due to sick sinus syndrome, and a case of PMT in 69 year-old man who had suffered complete heart block and received a VDD type pacemaker. In these cases, we investigate symptoms, mechanism of PMT, programed parameters of pacemaker and treatments of PMT.
Aged ; Dichlorodiphenyldichloroethane ; Extremities ; Heart ; Heart Block ; Humans ; Middle Aged ; Sick Sinus Syndrome ; Tachycardia* ; Ventricular Premature Complexes

The enterobiliary fistulas are mostly spontaneous and are caused by biliary disease associated with calculi. Review of many series shows that the most common variety of enterobiliary fistulas is a cholecystoduodenal fistula followed by cholecystocolic, cholecystogastric and choledochoduodenal fistula in that order. But very few cases of choledochocolonic fistulae have been reported. We report a case of choledocholithiasis combined with the absence of gallbladder leading to a choledochocolonic fistula, which was confirmed by endoscopic retrograde cholangiography and abdominal exploration in a 63- year-old male patient with complaint of right upper abdominal pain.
Abdominal Pain ; Calculi ; Cholangiography ; Choledocholithiasis ; Fistula* ; Gallbladder* ; Humans ; Intestinal Fistula ; Male

Recently it has been discovered that anomalous union of pancreaticobiliary duct (AUPBD) may play an important role in the pathogenesis of bile duct cancer. Similar to this fact, there were a few reports on the clinical significance of the low junction of the cystic duct (LJCD). LJCD refers to the situation whereby the cystic duct enters the common duct at a low position between the upper margin of the pancreas and the duodenal opening of the bile duct. The pathogenetic mechanism in LJCD is similar to AUPBD in that the pancreatic juice refluxes to the bile duct, mixes with biles, and then generates the mutagens, which act as inflammatory substances and carcinogens on the epithelium of the bile duct. A 51-year-old male who experienced dyspepsia was hospitalized due to his abnormally functioning liver and dilated common bile duct. We confirmed primary cholangiocarcinoma on the common hepatic duct and gallbladder carcinoma associated with LJCD by ERCP and pathologic review of surgical specimen. We report a case of synchronous type double primary cancer associated with LJCD with a brief review of the literatures.
Bile ; Bile Duct Neoplasms ; Bile Ducts ; Carcinogens ; Cholangiocarcinoma ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct ; Cystic Duct* ; Dyspepsia ; Epithelium ; Gallbladder ; Hepatic Duct, Common ; Humans ; Liver ; Male ; Middle Aged ; Mutagens ; Pancreas ; Pancreatic Juice