No abstract available.
Lipomatosis* ; Neoplasm Metastasis* ; Small Cell Lung Carcinoma*

Camurati-Engelmann disease (CED) is an autosomal dominant progressive diaphyseal dysplasia caused by mutations in the transforming growth factor-beta1 (TGFB1) gene. We report the first Korean family with an affected mother and son who were diagnosed with CED. The proband is a 19-yr-old male with a history of abnormal gait since the age of 2. He also suffered from proximal muscle weakness, pain in the extremities, and easy fatigability. Skeletal radiographs of the long bones revealed cortical, periosteal, and endosteal thickenings, predominantly affecting the diaphyses of the upper and lower extremities. No other bony abnormalities were noted in the skull and spine and no remarkable findings were seen on laboratory tests. The patient's mother had a long-standing history of mild limb pain. Under the impression of CED on radiographic studies, we performed mutation analysis. A heterozygous G to A transition at cDNA position +653 in exon 4 of the TGFB1 gene (R218H) was detected in the patient and his mother.
Adult ; Amino Acid Substitution ; Camurati-Engelmann Syndrome/*diagnosis/radiography ; DNA Mutational Analysis ; Diaphyses/radiography ; Heterozygote ; Humans ; Korea ; Male ; Muscle Weakness/radiography ; Pedigree ; Transforming Growth Factor beta1/*genetics

PURPOSE: To evaluate the long-term results of treatment of epiphyseal fractures of the distal radius in children. MATERIALS AND METHODS: 23 cases of distal radial epiphyseal fracture, treated by two methods: group 1, closed reduction (CR) plus cast (6 cases); group 2, CR and K-wire fixation (under anesthesia due to marked translation of the distal fragment and swelling) plus cast (17 cases), were selected for this study. All patients were followed up for more than 1 year (average: 3.2 years). Postoperatively, epiphyseal displacement and epiphyseal angulation were measured on anteroposterior and lateral radiographs. At follow-up, the affected and normal sides were compared. Final results were classified by radiologic (radial inclination, volar tilting and radial shortening) and clinical (limitation of ROM, wrist pain, grip strength and wrist deformity) criteria. RESULTS: Group 1 had 5 good, 1 fair result; group 2 had 14 good, 2 fair and 1 poor - there was no statistically significant difference between two groups. All cases where the epiphyseal displacement was less than 30% had good results. A poor case showed a radial shortening, wrist deformity and pain due to premature epiphyseal closure. Premature epiphyseal closure was treated by bar resection and free fat, along with corrective osteotomy when necessary and lengthening of radius with or without epiphysiodesis of the ulna. CONCLUSION: Remodeling can be expected in epiphyseal fractures of the distal radius. Repeated forceful attempts to achieve accurate reduction should be avoided to prevent secondary physeal injury.
Anesthesia ; Child ; Congenital Abnormalities ; Displacement (Psychology) ; Follow-Up Studies ; Hand Strength ; Humans ; Osteotomy ; Radius ; Wrist

PURPOSE: This study aimed to test whether rotavirus-associated necrotizing enterocolitis (RV+NEC) produced diffe rent clinical findings or outcomes from those of non-rotavirus necrotizing enterocolitis (RV-NEC). METHODS: Eight patients from the RV+NEC group and 22 patients from the RV-NEC group diagnosed with modified Bell stage II or higher NEC were selected for this study. Fecal specimens from all infants were tested for rotavirus infection using a monoclonal antibody-based enzyme immunoassay (EIA). Clinical, radiographic, and clinical outcome data were analyzed retrospectively. RESULTS: RV+NEC infants had a significantly higher birth weight and were born at a significantly higher gestational age (33.5+/-3.3 weeks vs. 29.3+/-4.4 weeks; P=0.01). There were no differe nces in the occurrence of thrombocytopenia, mural gas, and pneumoperitoneum between the 2 groups. However, portal vein gas was more common in the RV+NEC group (88% vs. 9%; P<0.01). Neither the incidence of Bell stage III (or higher) NEC nor surgical inte rvention differed between the two groups. The number of complications and mortality rates were also similar. CONCLUSION: Rotavirus-associated NEC occurs in infants with a higher birth weight and those born at a greater gestational age. However, the severity of the condition and the resulting outcomes did not differ from those for infants affected by non-rotavirus NEC.
Birth Weight ; Enterocolitis ; Enterocolitis, Necrotizing ; Gestational Age ; Humans ; Immunoenzyme Techniques ; Incidence ; Infant ; Infant, Newborn ; Pneumoperitoneum ; Portal Vein ; Retrospective Studies ; Rotavirus Infections ; Thrombocytopenia

We report a case of 51-year-old woman with pure unroofed coronary sinus without persistent left superior vena cava and other cardiac anomaly. She presented with dyspnea on exertion during several years. Her chest film showed prominent cardiomegaly and dilated hilar vessels. Cardiac rhythm was atrial fibrillation. Transthoracic echocardiography demonstrated the enlarged coronary sinus with defect toward left atrium on parasternal long axis view and significant flow from coronary sinus into right atrium on subxyphoid view, and its other findings were dilated right ventricle and right atrium, paradoxical septal motion, moderate tricuspid regurgitation and mild mitral regurgitation, which were mimicking of large secundum atrial septal defect. Radionuclide cardioangiography and cardiac catheterization showed the existence of significant shunt. There was no evidence of persistent left superior vena cava on chest CT. Closure of Coronary sinus opening was done. Thereafter her symptoms of congestive heart failure were much improved. We think that careful examination of 2-D echocardiography can be valuable tool for diagnosis of unroofed coronary sinus in adult patient.
Adult ; Atrial Fibrillation ; Axis, Cervical Vertebra ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomegaly ; Coronary Sinus* ; Diagnosis ; Dyspnea ; Echocardiography ; Female ; Heart Atria ; Heart Failure ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Middle Aged ; Mitral Valve Insufficiency ; Thorax ; Tomography, X-Ray Computed ; Tricuspid Valve Insufficiency ; Vena Cava, Superior*

A 66-year-old female who had undergone surgery for a herniated disc at the L5-S1 level at another hospital 3 months earlier was admitted with persistent back and leg pain. She was diagnosed with spondylodiscitis at the L5-S1 level by magnetic resonance imaging. A biopsy was performed, and Candida parapsilosis and Enterococcus faecium were isolated from the excised material. We report herein a case of successful treatment of polymicrobial spondylodiscitis in accordance with accurate microbiological diagnosis. Based on this case, we hope to encourage physicians to perform biopsies more aggressively or repeatedly to improve the diagnostic yield.
Aged ; Biopsy ; Candida ; Coinfection ; Diagnosis ; Discitis* ; Enterococcus faecium ; Female ; Hope ; Humans ; Intervertebral Disc Displacement ; Leg ; Magnetic Resonance Imaging

East Asian surgeons generally report lower morbidity and mortality rates for gastrectomy with D2 lymphadenectomy than do surgeons in Western countries; however, the disparity remains unexplained. The aim of this article was to determine the feasibility and safety regarding cases in which East Asian surgeons perform such procedures in Caucasian patients (CPs). Twelve CPs underwent gastrectomy with lymphadenectomy for gastric cancer at Yonsei University Severance Hospital, Seoul, Korea between June 2011 and April 2014. Procedures performed included total gastrectomy (7 of 12, 58%), distal gastrectomy (4 of 12, 33%), and completion total gastrectomy (1 of 12, 8%). Nine patients (75%) underwent D2 lymphadenectomy, and D1+ lymphadenectomy was performed in three others (25%). In four patients (33%), combined resections were carried out. The median values of surgical parameters were as follows: operative time, 266.5 min (range, 120-586 min); estimated blood loss, 90 mL (range, 37-350 mL); retrieved lymph node count, 37.5 (range, 22-63); and postoperative hospital stay, 13.7 days (range, 5-63 days). No mortality was encountered, although two patients (17%) experienced complications (both Clavien-Dindo classification grade IIIa anastomotic leakages), which were successfully managed by conservative treatment. In the hands of East Asian surgeons, mortality and short-term morbidity appears to be acceptably low in CPs subjected to gastrectomy with lymphadenectomy for gastric cancer.
Adult ; Aged ; *European Continental Ancestry Group ; Female ; *Gastrectomy/adverse effects ; Humans ; Length of Stay ; *Lymph Node Excision/adverse effects ; Male ; Middle Aged ; Operative Time ; *Patients ; Pilot Projects ; Republic of Korea ; Safety ; Stomach Neoplasms/pathology/*surgery ; *Surgeons

BACKGROUND: We report a case of a spray painter who developed malignant fibrous histiocytoma (MFH) of the maxillary sinus following long-term exposure to chromium, nickel, and formaldehyde, implying that these agents are probable causal agents of MFH. CASE REPORT: The patient developed right-sided prosopalgia that began twenty months ago. The symptom persisted despite medical treatment. After two months, he was diagnosed with MFH through imaging studies, surgery, and pathological microscopic findings at a university hospital in Seoul. His social, medical, and family history was unremarkable. The patient had worked for about 18 years at an automobile repair shop as a spray painter. During this period, he had been exposed to various occupational agents, such as hexavalent chromium, nickel, and formaldehyde, without appropriate personal protective equipment. He painted 6 days a week and worked for about 8 hours a day. Investigation of the patient's work environment detected hexavalent chromium, chromate, nickel, and formaldehyde. CONCLUSIONS: The study revealed that the patient had been exposed to hexavalent chromium, formaldehyde, and nickel compounds through sanding and spray painting. The association between paranasal cancer and exposure to the aforementioned occupational human carcinogens has been established. We suggest, in this case, the possibility that the paint spraying acted as a causal agent for paranasal cancer.
Automobiles* ; Carcinogens ; Chromium ; Formaldehyde ; Histiocytoma, Malignant Fibrous* ; Humans ; Maxillary Sinus* ; Nickel ; Occupational Exposure ; Paint ; Paintings ; Seoul ; Silicon Dioxide

PURPOSE: Recently established in 1997, Sungkyunkwan University School of Medicine was the first medical school in Korea to adopt problem-based learning (PBL) as a core curriculum from the very beginning. The purpose of this study was to evaluate the PBL curriculum from the viewpoint of the medical students. METHODS: This study used a qualitative approach to understand students' perceptions of the positive aspects of PBL. A total of 22 second-year medical students at Sungkynkwan medical school were surveyed. Student perceptions were obtained from questionnaires and face-to-face interviews. The interview was designed to evoke more personal and in-depth responses. RESULTS: Students' perceptions of the effects of PBL were found to be very favorable. Most students showed positive responses on the issues of more active learning attitude, self-directed learning, motivation to study, improved problem solving, and integrated learning. CONCLUSION: The results of this study demonstrated that the students had a good understanding of PBL and experienced its positive aspects of PBL approach. It might be concluded that PBL was successfully implemented into the curriculum and specific and expected effects of PBL accomplished.
Curriculum* ; Evaluation Studies as Topic* ; Humans ; Korea ; Learning ; Motivation ; Problem Solving ; Problem-Based Learning* ; Schools, Medical ; Students, Medical ; Surveys and Questionnaires

High mobility group box 1 (HMGB1) is a pivotal mediator of sepsis progression. Remifentanil, an opioid agonist, has demonstrated anti-inflammatory effects in septic mice. However, it is not yet known whether remifentanil affects the expression of HMGB1. We investigated the effects of remifentanil on HMGB1 expression and the underlying mechanism in septic rats. Forty-eight male Sprague-Dawley rats were randomly divided into 3 groups; a sham group, a cecal ligation and puncture (CLP) group, and a CLP with remifentanil treatment (Remi) group. The rat model of CLP was used to examine plasma concentrations of proinflammatory cytokines, tissue HMGB1 mRNA and the activity of nuclear factor (NF)-κB in the liver, lungs, kidneys, and ileum. Pathologic changes and immunohistochemical staining of NF-κB in the liver, lungs, and kidneys tissue were observed. We found that remifentanil treatment suppressed the level of serum interleukin (IL)-6 and tumor necrosis factor (TNF)-α 6 hours after CLP, and serum HMGB1 24 hours after CLP. HMGB1 mRNA levels and the activity of NF-κB in multiple organs decreased by remifentanil treatment 24 hours after CLP. Remifentanil treatment also attenuated nuclear expression of NF-κB in immunohistochemical staining and mitigated pathologic changes in multiple organs. Altogether, these results suggested that remifentanil inhibited expression of HMGB1 in vital organs and release of HMGB1 into plasma. The mechanism was related to the inhibitory effect of remifentanil on the release of proinflammatory cytokines and activation of NF-κB.
Animals ; Cytokines ; HMGB1 Protein ; Humans ; Ileum ; Inflammation ; Interleukins ; Kidney ; Ligation ; Liver ; Lung ; Male ; Mice ; Models, Animal ; Plasma ; Punctures ; Rats* ; Rats, Sprague-Dawley ; RNA, Messenger ; Sepsis ; Tumor Necrosis Factor-alpha