An 82-year-old man diagnosed with supraglottic cancer sought a consultation for percutaneous endoscopic gastrostomy (PEG) placement. Preoperative chest radiography (posterior-anterior [PA] view) revealed no abnormalities, and PEG tube placement was performed using the “pull” method. Chest radiography (PA view) performed 3 days postoperatively showed free air that was not observed immediately after PEG tube placement; therefore, the patient was diagnosed with pneumoperitoneum. Abdominal computed tomography confirmed that the PEG tube was appropriately positioned within the stomach; however, the colon was observed between the abdominal wall and stomach, which indicated that the PEG tube had passed through the colon. Review of preoperative chest radiographs (PA views) confirmed that the colon was visualized in the area wherein usually stomach gas should have been observed. The patient was diagnosed with a gastro-colo-cutaneous fistula that occurred postoperatively, following a procedure that was performed without confirmation of anatomical variations. The PEG tube was removed surgically, and we performed percutaneous gastrostomy.

OBJECTIVE: Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by infiltration of T lymphocytes, monocytes, and macrophages in muscle tissues. Interleukin-17 (IL-17), a Th17 cytokine, has potent pro-inflammatory actions and plays a role in autoimmune diseases. We investigated the expression of IL-17 in muscle tissues of patients with IIMs. METHODS: We measured the IL-17 mRNA level of muscle tissues from 14 patients with IIMs (9 patients with dermatomyositis and 5 patients with polymyositis) by real-time RT-PCR and compared with controls. We also performed an immunohistochemical stain to detect IL-17 expression. RESULTS: The expressions of IL-17 were significantly enhanced in IIMs than controls. In immunohistochemistry, IL-17 was expressed in perimysial, endomysial and perivascular infiltrating inflammatory cells. CONCLUSION: These results suggest that IL-17 plays a role in the immunopathogenesis of IIMs.

A 32-yr-old male patient with recurrent pneumothorax associated with bronchial atresia of the subsegmental branch of the posterior segmental bronchus of the right upper lobe was successfully treated with right upper lobectomy. Before surgery, the bronchial atresia with pneumothorax was suspected on the chest radiograph and CT scans, which showed the findings of bronchocele with localized hyperinflation of the right upper lobe. The examination of surgical specimen from the resected right upper lobe suggests that the cause of the recurrent pneumothorax was the rupture of the subpleural bullae in the hyperinflated lung segment distal to the atretic bronchus.
Adult ; Bronchi/*abnormalities/pathology ; Bronchial Diseases/*diagnosis ; Human ; Male ; Pneumothorax/*diagnosis ; Respiratory System Abnormalities/*pathology ; Tomography, X-Ray Computed

PURPOSE: The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease. METHODS: The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed. RESULTS: The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more physical stigmata. Chromosome breakage tests were positive in 9 out of 11 available patients. The median follow-up duration was 69.5 months. The Kaplan-Meier (KM) survival of all patients was 83.3% at 10 years and 34.7% at 20 years, respectively. Seven patients underwent 9 stem cell transplantations (SCTs). Among them, 5 were alive by the end of the study. Ten-year KM survival after SCT was 71.4% with a median follow-up of 3.4 years. All 5 patients treated with supportive treatment alone died of infection or progression at the median age of 13.5 years, except for one with short follow-up duration. Acute leukemia developed in 2 patients at 15.4 and 18.1 years of age. Among 6 patients who are still alive, 3 had short stature and 1 developed insulin-dependent diabetes mellitus. CONCLUSION: We provide information on the long-term outcomes of FA patients in Korea. A nation-wide FA registry that includes information of the genotypes of Korean patients is required to further characterize ethnic differences and provide the best standard of care for FA patients.
Bone Marrow ; Christianity ; Chromosome Breakage ; Diabetes Mellitus, Type 1 ; Diagnosis ; Fanconi Anemia* ; Follow-Up Studies* ; Genotype ; Humans ; Jeollanam-do ; Korea ; Leukemia ; Medical Records ; Retrospective Studies ; Standard of Care ; Stem Cell Transplantation ; Treatment Outcome*

Primary duodenal carcinoma is rare. Duodenal mucinous adenocarcinoma (DMA) is even rarer, and its associated manifestations and typical endoscopic or imaging findings are not well characterized. Herein, we report a case of primary DMA in an asymptomatic 58-year-old man who visited our hospital for a regular health screening. Upper endoscopy revealed an approximately 4-cm lesion in the second portion of the duodenum, but the mass was not visualized on computed tomography. Biopsies revealed a tubular adenoma that was subsequently resected. Frozen biopsies demonstrated DMA with a background of low-grade tubular adenoma for which we performed Roux-en-Y duodenojejunostomy and jejunojejunostomy. To our knowledge, this is the first report of a patient with DMA in Korea.
Adenocarcinoma ; Adenocarcinoma, Mucinous* ; Adenoma ; Biopsy ; Duodenal Neoplasms ; Duodenum ; Endoscopy ; Humans ; Korea ; Mass Screening ; Middle Aged

Botulinum neurotoxin type A (BoNT/A) is a metalloprotease that cleaves SNAP-25 (synaptosome-associated protein of 25 kDa), a specific cellular protein essential for neurotransmitter release. As well as mouse bioassay to detect BoNT/A, various assay methods based on its endopeptidase activity have been developed. In this study, we tried to develop a BoNT/A assay system using recombinant SNAP-25 with glutathione S-transferase (GST) tags at both termini as substrate. The recombinant GST-SNAP-25-GST with 70 kDa was expressed and purified in E. coli and synthesized N-terminal 50 kDa and C-terminal 25 kDa fragment after cleavage at the Gln(197)-Arg(198) bond by BoNT/A. To detect both fragments, we obtained rabbit antisera against peptides corresponding to the cleaved ends of each fragment. In the western blotting, the N-terminal fragment was detected by the antibody specifically recognizing the newly exposed C-terminus (corresponding to amino acid residue 191-197). This assay system was able to detect until 3.125 ng of BoNT/A, which corresponded to about 90 fold LD50 in mice. These results suggest that the in vitro endopeptidase assay developed in this study would replace others to detect BoNT/A.
Animals ; Biological Assay ; Blotting, Western ; Glutathione Transferase ; Immune Sera ; Lethal Dose 50 ; Mice ; Neurotransmitter Agents ; Peptides

Botulinum neurotoxin type A (BoNT/A) is a metalloprotease that cleaves SNAP-25 (synaptosome-associated protein of 25 kDa), a specific cellular protein essential for neurotransmitter release. As well as mouse bioassay to detect BoNT/A, various assay methods based on its endopeptidase activity have been developed. In this study, we tried to develop a BoNT/A assay system using recombinant SNAP-25 with glutathione S-transferase (GST) tags at both termini as substrate. The recombinant GST-SNAP-25-GST with 70 kDa was expressed and purified in E. coli and synthesized N-terminal 50 kDa and C-terminal 25 kDa fragment after cleavage at the Gln(197)-Arg(198) bond by BoNT/A. To detect both fragments, we obtained rabbit antisera against peptides corresponding to the cleaved ends of each fragment. In the western blotting, the N-terminal fragment was detected by the antibody specifically recognizing the newly exposed C-terminus (corresponding to amino acid residue 191-197). This assay system was able to detect until 3.125 ng of BoNT/A, which corresponded to about 90 fold LD50 in mice. These results suggest that the in vitro endopeptidase assay developed in this study would replace others to detect BoNT/A.
Animals ; Biological Assay ; Blotting, Western ; Glutathione Transferase ; Immune Sera ; Lethal Dose 50 ; Mice ; Neurotransmitter Agents ; Peptides

Lymphangioma is a benign vascular lesion with characteristics of subepithelial tumor which can proliferate in the lymphatic system. Lymphangioma of the small-bowel mesentery is rare, having been reported in less than 2% of all lymphangiomas. Lymphangioma does not require any specific treatment because it is absolutely a benign tumor. However, surgical exploration is rarely required for cases with disease-related symptoms or complications, or for those misdiagnosed as a malignant lesion. We recently experienced a case of mesenteric cavernous lymphangomas in a 53-year-old female who was misdiagnosed as having a liposarcoma. The final diagnosis was confirmed by a pathologic examination of the specimen that was obtained via laparoscopic exploration. Herein, we report a very rare case of mesenteric cavernous lymphangioma along with a brief review of relevant literature.
Female ; Humans ; Intestine, Small/pathology ; Laparoscopy ; Lymphangioma/*diagnosis/pathology/surgery ; Mesentery/pathology ; Middle Aged ; Peritoneal Neoplasms/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed

Objective: To investigate the expression of toll-like receptor (TLR)-2, 4 and 9 in idiopathic inflammatory myopathies (IIMs). Methods: The expression of TLR-2, 4 and 9 was measured by real-time RT-PCR and immunohistochemical stain (IHS) from muscle tissues in patients with IIMs and controls. Results: The expression levels of TLR-2, 4 and 9 in IIMs were significantly higher than controls. TLR-2, 4 and 9 were mainly expressed on sarcolemma of muscle fibers, perimysial vascular endothelium and infiltrating inflammatory cells in dermatomyositis, whereas, they were mainly expressed on sarcolemma of muscle fibers, destructed muscle fibers, and enodmysial infiltrating inflammatory cells in polymyositis. Conclusion: TLR-2, 4 and 9 were highly expressed in muscle tissue of IIMs. These results suggest that TLR-2, 4 and 9 play a role in pathogenesis of IIMs.
Dermatomyositis ; Endothelium, Vascular ; Humans ; Myositis* ; Polymyositis ; Sarcolemma ; Toll-Like Receptors*

BACKGROUND: Dilution of blood cardioplegia is not needed in IAWBC as it is in cold blood cardioplegia because it does not aggregate red blood cells on normal body temperature and does not compromise micro coronary circulation. This study was designed to evaluate the safety and efficacy of undiluted potassium solution in IAWBC. MATERIAL AND METHOD: Thirty patients who underwent CABG with IAWBC were grouped into dilutedplegia (n=14) and microplegia (n=16). Potassium was delivered conventionally with 4:1 delivery kit in the dilutedplegia group. The undiluted potassium was directly connected on the blood of oxygenator in the microplegia group. RESULT: There were no differences in sex, age, left ventricular ejection fraction, number of grafts, aortic cross clamping time, and the value of perioperative myocardial enzyme between the two groups. There were no perioperative myocardial infarction and hospital mortality. The amount of crystalloid cardioplegia was 1346+/-597 mL in dilutedplegia (mean+/-standard deviation, and 28+/-9 mL in microplegia (p<0.0001). The hematocrit during cardiopulmonary bypass was 21+/-4% in dilutedplegia and 24+/-3% in microplegia (p>0.05). 11 patients in dilultedplegia received blood transfusion, but 4 patients in microplegia received blood transfusion (p<0.05). The amount of urine and hemofiltration during the operation were more in dilutedplegia (1250+/-810 mL, 1689+/-548 mL) than in microplegia (959+/-410 mL, 1481+/-784 mL; p<0.05). CONCLUSION: The undiluted potassium of IAWBC in CABG operation is a safe, effective technique for myocardial protection to prevent fluid overload, and blood transfusion. There is no need to use the delivery kit.
Blood Transfusion ; Body Temperature ; Cardiopulmonary Bypass ; Constriction ; Coronary Circulation ; Erythrocytes ; Heart Arrest, Induced* ; Hematocrit ; Hemofiltration ; Hospital Mortality ; Humans ; Myocardial Infarction ; Oxygen ; Oxygenators ; Potassium* ; Stroke Volume ; Transplants