BACKGROUND/AIMS: The aim of this study is to describe the outcome of the national survey and to determine the endoscopic retrograde cholangiopancreatography (ERCP) quality in Korea by comparing with the quality indicators. METHODS: We used the database of Health Insurance Review & Assessment Service and then performed anonymous national survey. RESULTS: Completed questionnaires were returned by 129 of 157 ERCP endoscopists. In Korea, annual ERCP rates have been consistently increased over years. Individual ERCP volume was high (>200 per year) in about half of ERCP endoscopists. Most ERCP endoscopists performed all of level I procedures. However, manometry, cholangiopancreatoscopy, and pancreatic procedures were performed mostly in institutions with high hospital volume. The rate of overall success was more than 90% in most ERCP endoscopists. However, the rate of precut sphincterotomy was high in more than a fourth of ERCP endoscopists. Twelve ERCP endoscopists experienced post-ERCP mortality within recent 1 year. ERCP training and radiation protection during ERCP did not meet the standard of quality indicators especially in institutions with low or moderate hospital volume. CONCLUSIONS: Technical issues during ERCP procedures in Korea fulfill the standard of quality indicators. However, a great effort is needed to improve issues about ERCP training and radiation protection.
Anonyms and Pseudonyms ; Cholangiopancreatography, Endoscopic Retrograde ; Insurance, Health ; Korea ; Manometry ; Mortality ; Radiation Protection ; Republic of Korea ; Surveys and Questionnaires

BACKGROUND/AIMS: Proton pump inhibitor-responsive esophageal eosinophilia (PPI-REE) is a newly recognized form of eosinophilic esophagitis (EoE) that responds to PPI therapy. It remains unclear whether PPI-REE represents a subphenotype of gastroesophageal reflux disease, a subphenotype of EoE, or its own distinct entity. The aim was to evaluate the clinicopathologic features of PPI-REE. METHODS: Six patients were diagnosed with PPI-REE based on symptoms, endoscopic abnormalities, esophageal eosinophilia with > or =15 eosinophils/high-power field, and a response to PPI treatment. Symptoms and endoscopic and pathological findings were evaluated. RESULTS: The median follow-up duration was 12 months. Presenting symptoms included dysphagia, heartburn, chest pain, foreign body sensation, acid reflux, and sore throat. All patients had typical endoscopic findings of EoE such as esophageal rings, linear furrows, nodularity, and whitish plaques. Three patients had a concomitant allergic disorder, and one had reflux esophagitis. Four patients exhibited elevated serum IgE, and five had positive skin prick tests. All patients experienced symptomatic resolution within 4 weeks and histologic resolution within 8 weeks after starting PPI therapy. There was no symptomatic recurrence. CONCLUSIONS: PPI therapy induced rapid resolution of symptoms and eosinophil counts in patients with PPI-REE. Large-scale studies with long-term follow-up are warranted.
Adult ; Asian Continental Ancestry Group ; Chest Pain/etiology ; Deglutition Disorders/etiology ; Diagnosis, Differential ; Eosinophilic Esophagitis/complications/*drug therapy/*pathology ; Esophagus/pathology ; Female ; Follow-Up Studies ; Gastroesophageal Reflux/etiology ; Heartburn/etiology ; Humans ; Male ; Middle Aged ; Pharyngitis/etiology ; Phenotype ; Proton Pump Inhibitors/*therapeutic use ; Republic of Korea ; Retrospective Studies ; Sensation Disorders/etiology ; Treatment Outcome ; Young Adult

A 75-year-old woman with hypertension presented with acute suppurative cholangitis. Chest radiography revealed severe kyphosis. Abdominal computed tomography revealed a large stone impacted in the common bile duct (CBD). The patient underwent emergent endoscopic retrograde cholangiopancreatography, and cholangiography revealed a large stone (7×3 cm) in the CBD that could not be captured using a large basket. We could not use the percutaneous approach for stone fragmentation by using a cholangioscope because of severe degenerative kyphosis. Finally, we performed holmium laser lithotripsy under peroral cholangioscopy by using an ultraslim endoscope, and the large stone in the CBD was successfully fragmented and removed without complications.
Aged ; Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Common Bile Duct* ; Endoscopes ; Female ; Gallstones ; Humans ; Hypertension ; Kyphosis* ; Lasers, Solid-State ; Lithotripsy ; Lithotripsy, Laser* ; Radiography ; Thorax

A 24-year-old man was admitted due to an incidentally detected mass in his left testis, which showed radiopaque calcification on plain X-ray film. Left orchiectomy was performed, and the resected testis contained a well-demarcated, hard mass measuring 1.1 cm. Histological analysis revealed that the tumor was composed of neoplastic cells, fibrotic stroma, and laminated or irregularly shaped calcific bodies. The individual cells had abundant eosinophilic or clear cytoplasm with round nuclei, each of which contained one or two conspicuous nucleoli. They were arranged in cords, trabeculae, clusters, and diffuse sheets. There were several foci of intra-tubular growth patterns, with thickening of the basal lamina. Immunohistochemically, the neoplastic cells were positive for S-100 protein and vimentin, focally positive for inhibin alpha, and negative for cytokeratin, CD10, and Melan-A. In addition to reporting this rare case, we also review the relevant literature regarding large cell calcifying Sertoli cell tumors.
Basement Membrane ; Cytoplasm ; Eosinophils ; Humans ; Immunohistochemistry ; Inhibins ; Keratins ; MART-1 Antigen ; Orchiectomy ; S100 Proteins ; Sertoli Cell Tumor* ; Testis* ; Vimentin ; X-Ray Film ; Young Adult

BACKGROUND/AIMS: Antiphospholipid antibodies (aPL) have been detected in various proportions of patients with primary immune thrombocytopenia (ITP), but the clinical significance of this is debatable. The present study aimed to determine the frequency and clinical implications of elevated aPL in adult patients with ITP. METHODS: We prospectively studied newly diagnosed adult patients with ITP who were enrolled between January 2003 and December 2008 at Chungnam National University Hospital. They were evaluated for the presence of lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) at diagnosis and were followed for the development of thrombosis. RESULTS: Seventy consecutive patients with ITP (median age, 48 years; range, 18 to 79) were enrolled. Twenty patients (28.5%) were positive for aPL at the time of diagnosis: aCL alone in 15 (75%), aCL and LA in two (10%), and LA alone in three (15%). Patients who had platelet counts < 50,000/microL were administered oral prednisolone with or without intravenous immune globulin. No difference was found between the aPL-positive and -negative groups regarding gender, initial platelet count, and response to the therapy. After a median follow-up of 20 months (range, 2 to 68), two of 20 patients who were aPL-positive (10%) developed thrombosis, whereas no thrombotic event was found among those who were aPL-negative. CONCLUSIONS: Our data suggest that aPL levels should be determined at the initial presentation of ITP and that patients found to be aPL-positive should receive closer follow-up for thrombotic events.
Adolescent ; Adult ; Aged ; Antibodies, Anticardiolipin/*blood ; Antibodies, Antiphospholipid/blood ; Chi-Square Distribution ; Female ; Glucocorticoids/therapeutic use ; Humans ; Lupus Coagulation Inhibitor/*blood ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Prospective Studies ; Purpura, Thrombocytopenic, Idiopathic/*blood/drug therapy ; Thrombocythemia, Essential/*blood/drug therapy ; Thrombosis ; Young Adult

Multiple solitary plasmacytoma is a very rare disease entity, which occurs in up to 5% of patients with solitary plasmacytomas. We report an atypical case of multiple solitary plasmacytoma that recurred in multiple visceral organs without any evidence of bone marrow involvement. A 68-year-old male presented with voiding difficulty. Twenty months earlier, he had been placed on local radiotherapy for solitary plasmacytomas in the right 6th rib and right iliac bone. Recurrences were noted 14 and 12 months later in several ribs and the 5th cervical vertebra, respectively. These were well controlled with local radiotherapy and conventional systemic chemotherapy. He had multiple soft tissue masses in the stomach, pancreas, pelvic cavity, and right buttock. An endoscopic biopsy of the gastric mass confirmed the diagnosis of plasmacytoma. Local radiotherapy to the pelvic mass and systemic therapy consisting of bortezomib and dexamethasone were given, and he has been well for 8 months.
Aged ; Biopsy ; Bone Marrow ; Boronic Acids ; Buttocks ; Dexamethasone ; Humans ; Male ; Pancreas ; Plasmacytoma ; Pyrazines ; Rare Diseases ; Recurrence ; Ribs ; Spine ; Stomach ; Viscera ; Bortezomib

BACKGROUND: Hypocellularity of bone marrow (BM), not associated with significant dyshematopoiesis, is often found in patients with isolated thrombocytopenia, but its clinical implications have not been studied. We prospectively studied the clinical features and natural history of these patients. METHODS: Adults with isolated thrombocytopenia (platelet counts <100x10(9)/L) in the absence of dyshematopoiesis, cytogenetic abnormalities, or megakaryocytic hyperplasia and who had BM hypocellularity (below 30% in patients aged less than 60 years; below 20% in patients aged 60 years or more) were enrolled at Chungnam National University Hospital between January 2002 and December 2006. They were monitored regularly for changes in platelet counts or development of additional cytopenia. RESULTS: Twenty patients (17 men and 3 women) were enrolled in the study. The median age was 29 years (range, 18-70 years). At initial presentation, the platelet counts ranged from 12x10(9)/L to 99x10(9)/L (median, 63x10(9)/L) and were >50x10(9)/L in 16 patients (80%). BM cellularity ranged from 5% to 25% (median, 15%) and was < or =10% in 6 patients (30%). During the median 48-month follow-up (range, 12-90 months), platelet counts of 3 of the 20 patients recovered to normal levels (>150x10(9)/L) after 12, 56 and 66 months. Three patients developed pancytopenia after 11, 70 and 90 months. Two patients were consistent with moderate aplastic anemia, and 1 was confirmed as having refractory cytopenia with multilineage dysplasia. In the remainder of the patients, platelet counts remained unchanged. CONCLUSION: Isolated thrombocytopenia accompanied by hypocellular marrow encompasses a group of heterogeneous conditions.
Adult ; Aged ; Anemia, Aplastic ; Bone Marrow ; Chromosome Aberrations ; Follow-Up Studies ; Humans ; Hyperplasia ; Male ; Myelodysplastic Syndromes ; Natural History ; Pancytopenia ; Platelet Count ; Prospective Studies ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia

Cyclic vomiting syndrome (CVS) is a disorder characterized by recurrent episodes of incapacitating nausea and vomiting interspersed with symptom free periods. Common triggers of cyclic vomiting include noxious stress, excitement, fatigue and menstrual period. Here, we report a case of cyclic vomiting syndrome in adult patient characterized by stereotypical vomiting attack, occurring in every menstruation period. Recurrent vomiting episodes began 6 years ago and we treated this patient with subcutaneous injection of goserelin, a gonadotropin-releasing hormone analogue (GnRHa) and oral estrogen. After 4 months of therapy, she was symptom free for the following 5 years, even with the resumed normal menstruation. Recurrence of vom - iting attack with same pattern occurred 1 month before readmission. Treatment with intravenous lorazepam aborted vomiting, but could not prevent recurrences of vomiting and epigastric pain. We treated the patient with GnRHa and oral estradiol again which effectively prevented recurrence of the symptoms.
Adult ; Estradiol ; Estrogens ; Fatigue ; Female ; Gonadotropin-Releasing Hormone ; Goserelin ; Humans ; Injections, Subcutaneous ; Lorazepam ; Menstruation ; Nausea ; Recurrence ; Vomiting

Central nervous system (CNS) myelomatosis, which is the presence of monoclonal plasma cells in the cerebrospinal fluid (CSF), is extremely rare. We report a case of CNS myelomatosis developed in a 45-year-old woman with multiple myeloma in complete response, which was achieved by allogeneic peripheral blood stem cell transplantation using a reduced-intensity conditioning regimen consisting of melphalan, fludarabine, and antithymocyte globulin. Two months after the transplant, she developed a moderate motor and sensory weakness in both lower extremities. Atypical plasma cells were found in the CSF, and immunofixation revealed monoclonal light chain in the CSF. She was given three courses of weekly intra-thecal chemotherapy consisting of methotrexate, cytarabine, and dexamethasone, which cleared the CSF. This case indicates that the allogeneic transplantation could not control CNS myelomatosis, despite successfully treating the bone marrow myeloma.
Antilymphocyte Serum ; Bone Marrow ; Central Nervous System ; Cytarabine ; Dexamethasone ; Female ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Light ; Lower Extremity ; Melphalan ; Methotrexate ; Middle Aged ; Multiple Myeloma ; Nervous System ; Peripheral Blood Stem Cell Transplantation ; Plasma Cells ; Transplantation, Homologous ; Transplants ; Vidarabine

BACKGROUND: Bone marrow (BM) mesenchymal stem cells (MSCs) can be expanded over 20~30 cell doublings in vitro even in the absence of any growth factors. However, the mechanisms that govern MSC proliferation are not well understood. METHODS: We investigated the role of signaling of the pertussis toxin (PTX)-sensitive G protein-coupled receptor in the proliferation of BM MSCs. RESULTS: PTX inhibited the proliferation of human BM MSCs and murine BM stromal MS-5 cells in a dose-dependent manner. Among the chemokines produced by the BM stromal cells, stromal cell-derived factor-1 (SDF-1) enhanced the proliferation of BM MSCs, while MIP-1alpha, MCP-3 or RANTES did not. PTX also inhibited the proliferation of some fibroblasts, such as MRC-5 and NIH-3T3, but did not affect the proliferation of HeLa and HSF cells. HSF cells did not express CXCR4 mRNA, but did produce SDF-1. In contrast, HeLa cells expressed CXCR4 strongly on the cell surface, but did not produce SDF-1. BM MSCs, MS-5, MRC-5, and NIH-3T3 cells all expressed CXCR4 minimally on the cell surface. These cells, however, had abundant CXCR4 protein in their cytoplasm, which was demonstrated by flow cytometric analysis performed after permeabilization of the cells. In addition, an ELISA performed on the culture supernatants of the cells revealed that these cells constitutively produce and secrete SDF-1. CONCLUSION: These results indicate that the signaling through the PTX-sensitive G protein-coupled receptor, which is induced by autocrine factors, plays an important role in the proliferation of BM MSCs and in some fibroblasts, and that SDF-1 is the most probable candidate for the autocrine growth factor.
Bone Marrow* ; Cell Proliferation ; Chemokine CCL3 ; Chemokine CCL5 ; Chemokines ; Cytoplasm ; Enzyme-Linked Immunosorbent Assay ; Fibroblasts ; HeLa Cells ; Humans ; Intercellular Signaling Peptides and Proteins ; Mesenchymal Stromal Cells* ; NIH 3T3 Cells ; Pertussis Toxin ; RNA, Messenger ; Stromal Cells ; Whooping Cough*