Purpose: Studies on intestinal Behçet’s disease (BD) complicated by myelodysplastic syndrome (MDS) are rare, and no established therapeutic guidelines exist. This study aimed to evaluate the clinical presentation and outcomes of patients with intestinal BD complicated by MDS (intestinal BD–MDS) and suggest a treatment strategy. Materials and Methods: Data from patients with intestinal BD–MDS from four referral centers in Korea who were diagnosed between December 2000 and December 2022 were retrospectively analyzed. Clinical features and prognosis of intestinal BD–MDS compared with age-, sex-matched intestinal BD without MDS were investigated. Results: Thirty-five patients with intestinal BD–MDS were included, and 24 (70.6%) had trisomy 8. Among the 35 patients, 23 (65.7%) were female, and the median age at diagnosis for intestinal BD was 46.0 years (range, 37.0–56.0 years). Medical treatments only benefited eight of the 32 patients, and half of the patients underwent surgery due to complications. Compared to 70 matched patients with intestinal BD alone, patients with intestinal BD–MDS underwent surgery more frequently (51.4% vs. 24.3%; p=0.010), showed a poorer response to medical and/or surgical treatment (75.0% vs. 11.4%; p<0.001), and had a higher mortality (28.6% vs. 0%; p<0.001). Seven out of 35 patients with intestinal BD–MDS underwent hematopoietic stem cell transplantation (HSCT), and four out of the seven patients had a poor response to medical treatment prior to HSCT, resulting in complete remission of both diseases. Conclusion Patients with intestinal BD–MDS frequently have refractory diseases with high mortalities. HSCT can be an effective treatment modality for medically refractory patients with intestinal BD–MDS.

Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. Although most PPGLs are benign, approximately 10% have metastatic potential. Approximately 40% cases are reported as harboring germline mutations. Therefore, timely and accurate diagnosis of PPGLs is crucial. For more than 130 years, clinical, molecular, biochemical, radiological, and pathological investigations have been rapidly advanced in the field of PPGLs. However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. Furthermore, great progress on genetics has shifted the paradigm of genetic testing of PPGLs. The Korean PPGL task force team consisting of the Korean Endocrine Society, the Korean Surgical Society, the Korean Society of Nuclear Medicine, the Korean Society of Pathologists, and the Korean Society of Laboratory Medicine has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs.

Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. Although most PPGLs are benign, approximately 10% have metastatic potential. Approximately 40% cases are reported as harboring germline mutations. Therefore, timely and accurate diagnosis of PPGLs is crucial. For more than 130 years, clinical, molecular, biochemical, radiological, and pathological investigations have been rapidly advanced in the field of PPGLs. However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. Furthermore, great progress on genetics has shifted the paradigm of genetic testing of PPGLs. The Korean PPGL task force team consisting of the Korean Endocrine Society, the Korean Surgical Society, the Korean Society of Nuclear Medicine, the Korean Society of Pathologists, and the Korean Society of Laboratory Medicine has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs.

PURPOSE: To evaluate the perioperative, functional, and oncological outcomes of renal cryoablation (RC) of small renal masses (SRMs) performed in Korea University Hospital. MATERIALS AND METHODS: We reviewed an Institutional Review Board-approved database of 70 patients who underwent RC and were followed up for a minimum of 3 months by a single surgeon in Korea University Hospital from August 2007 to May 2014. Among these patients, 68 patients (79 renal masses) were enrolled in our research. We evaluated perioperative, functional, and oncologic outcomes of RC. RESULTS: A total of 68 patients (79 renal masses) underwent RC in our institution. The mean age of the patients was 62.0 years. The mean tumor size was 2.25 cm. Among the 59 patients who underwent laparoscopic surgery, only 1 patient (1.47%) was converted to open surgery. No other perioperative complications occurred. The mean preoperative and 1-month postoperative estimated glomerular filtration ratio (eGFR) were 71.8 and 68.3 mL/min/1.73 m2, respectively (p=0.19). The mean 1-year postoperative eGFR was 65.0 mL/min/1.73 m2 (p=0.25). The mean follow-up period was 59.76 months (range, 3-119 months). Local tumor recurrence occurred in eight tumors (15.4%; a total of 52 renal cell carcinomas). Concerning treatment in the patients with recurrence, five patients underwent re-treatment and three patients are under active surveillance. None of the eight patients who experienced local recurrence had additional recurrence or tumor progression during the follow-up period. In our study, the recurrence-free rate was 83.0% and the cancer-specific survival rate was 100%. Moreover, the 5- and 10-year overall survival rates were both 100%. CONCLUSIONS: Long-term experience with RC in our institution demonstrates that RC is a safe and effective treatment for patients with SRMs.
Adult ; Aged ; Aged, 80 and over ; Biopsy ; Carcinoma, Renal Cell/pathology/radiography/*surgery ; Cryosurgery/adverse effects/*methods ; Follow-Up Studies ; Glomerular Filtration Rate ; Humans ; Kidney Neoplasms/pathology/radiography/*surgery ; Laparoscopy/adverse effects/methods ; Middle Aged ; Neoplasm Recurrence, Local ; Tomography, X-Ray Computed ; Treatment Outcome ; Young Adult

PURPOSE: We previously described a new procedure specific module (Tube 3) to allow the practice of vesicourethral anastomosis after robot-assisted radical prostatectomy. Herein, we report a predetermined proficiency level of Tube 3 and preliminary validation to explore whether this new module can lead to performance improvement in the da Vinci system. MATERIALS AND METHODS: Eight urology residents and three urology fellows performed the Tube 3 module 1 hour daily for 7 days. The learning curve was depicted through a scatterplot and the stable point was identified through the cumulative sum chart. Concurrent and predictive validations were performed with the da Vinci system. The mean time to complete the task and end product rating score between Tube 3 training group and no Tube 3 training group were compared. RESULTS: Concerning the learning curve, about 41 repetitions comprising about 5 hours were needed to achieve this stable point when the mean time to complete Tube of 384 seconds was set as a target. With regarding to the concurrent and predictive validation, there significant differences were evident in the mean time to complete 16 needle passages and the vesicourethral anastomosis and the end product rating score. CONCLUSIONS: The virtual reality (VR) simulator can yield sufficient improvement in technical performance in Tube 3 within 5 hours. The acquired proficiency can be transferable to the vesicourethral anastomosis using the da Vinci system.
Anastomosis, Surgical/methods ; Clinical Competence ; Computer Simulation ; Education, Medical, Graduate/*methods ; Humans ; Learning Curve ; Male ; Prostatectomy/*education/methods ; Robotic Surgical Procedures/*education/methods ; Simulation Training/methods ; Urethra/surgery ; Urinary Bladder/surgery ; User-Computer Interface

OBJECTIVE: To evaluate the prevalence and risk factors of upper extremity musculoskeletal diseases (MSDs) among Korean farmers. METHODS: The study was carried out from June 2013 to August 2015 on 850 farmers and 203 non-farmers (controls) in Gyeongnam Province. Physical examinations were performed by rheumatologists, orthopedists, and rehabilitation specialists. Plain radiography, a nerve conduction examination, and magnetic resonance imaging were performed, and the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire was used to assess upper extremity function. RESULTS: Thirty-four different types of upper extremity MSDs were detected in the 1,053 study subjects. The prevalence of any MSD in farmers was 8.96-fold higher than in control (p<0.001). The most obvious difference in prevalence between farmers and non-farmers was hand osteoarthritis (48.2% vs. 4.9%). Mean total DASH score was higher for farmers than non-farmers (14.29+/-13.66 vs. 10.03+/-10.85, p<0.001). Among farmers, myofascial pain syndrome, rotator cuff tear, and epicondylitis were more prevalent among overhead workers (growing persimmons, pears, and grapes) than in non-overhead workers (growing rice and upland crops). The following factors were associated with a rotator cuff tear; older age, overhead work, high waist circumference, and lower level of education. Hand osteoarthritis was found to be associated with older age, a female gender, high waist circumference, and longer total work time. CONCLUSION: The prevalence of upper extremity MSDs is much higher in farmers than non-farmers and greater still for farmers doing overhead work. Various factors contribute to the occurrence of upper extremity MSDs, and thus, the authors suggest an efficient preventive strategy, which involves consideration of type of work and risk factors, be established for farmers to reduce upper extremity MSDs.
Arm ; Diospyros ; Education ; Female ; Hand ; Humans ; Magnetic Resonance Imaging ; Musculoskeletal Diseases* ; Myofascial Pain Syndromes ; Neural Conduction ; Osteoarthritis ; Physical Examination ; Prevalence* ; Pyrus ; Radiography ; Rehabilitation ; Risk Factors* ; Rotator Cuff ; Shoulder ; Specialization ; Tears ; Upper Extremity* ; Waist Circumference

PURPOSE: To analyze the complications after robot-assisted radical cystectomy (RARC) by use of a standardized reporting methodology by a single surgeon. MATERIALS AND METHODS: We prospectively reviewed a maintained institutional database of 52 patients who underwent RARC to manage bladder cancer and were followed up in 3 months by a single surgeon at Korea University Medical Center from 2007 through 2014. All complications within 90 days of surgery were defined and categorized into 5 grades according to the Clavien-Dindo classification. Logistic regression analysis was used to identify predictors of complications. RESULTS: Fifty percent of patients (26 of 52) experienced a complication of any grade <90 days after surgery, and 11 patients (21.2%) experienced a major complication. Complications were grouped in systems-based categories. Fifty complications occurred in 52 patients and hematologic complication (transfusion) was the most common (13 of 52). Wound dehiscence, anastomotic leakage, urinary tract obstruction, mechanical obstruction, and thromboembolism occurred as major complications. Mean estimated blood loss (EBL) was 247 mL and mean total operative time was 496 minutes. The mean number of lymph nodes harvested was 24.6, with 30.5 for extended dissection. EBL (over 300 mL), operative time, and method of urinary diversion were significant negative predictors of minor complications, whereas EBL (over 300 mL) was a significant negative predictor of major complications (p<0.05). CONCLUSIONS: The present results show that the complication rate reported by use of a standardized methodology after robotic radical cystectomy is still considerable although comparable to that of contemporary robot series. EBL, operative time, and diversion methods were predictors of complications.
Adult ; Aged ; Anastomotic Leak ; Blood Loss, Surgical ; Cystectomy/*adverse effects ; Female ; Hospitals, University ; Humans ; Logistic Models ; Lymph Node Excision ; Male ; Middle Aged ; Operative Time ; *Postoperative Complications ; Prospective Studies ; Republic of Korea ; Risk Factors ; *Robotic Surgical Procedures ; Surgical Wound Dehiscence ; Thromboembolism ; Treatment Outcome ; Urinary Bladder/pathology ; Urinary Bladder Neoplasms/*surgery ; Urinary Diversion/*adverse effects

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.
Arteries ; Arterioles ; Biopsy ; Delayed Diagnosis* ; Diagnosis ; Kidney ; Lung ; Microscopic Polyangiitis ; Purpura, Schoenlein-Henoch* ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Venules ; Wegener Granulomatosis*

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-Schonlein purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-Schonlein purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.
Arteries ; Arterioles ; Biopsy ; Delayed Diagnosis* ; Diagnosis ; Kidney ; Lung ; Microscopic Polyangiitis ; Purpura, Schoenlein-Henoch* ; Respiratory System ; Systemic Vasculitis ; Vasculitis ; Venules ; Wegener Granulomatosis*

PURPOSE: To determine whether radiofrequency thermal ablation can be used to treat benign cystic lesions in a porcine gallbladder model. MATERIALS AND METHODS: This experimental study of radiofrequency thermal ablation involved the use of 15 exvivo porcine gallbladders and 15-G expandable needle electrodes. To investigate optimal temperature parame-ters,three groups of five were designated according to target temperature: Group A: 70 degrees C; Group B: 80 degrees C; Group C: 90 degrees C. After the target temperature was reached, ablation lasted for one minute. Gallbladder width, height and length were measured before and after ablation, and the estimated volume reduction ratios of the three groups were compared. Whether adjacent liver parenchyma around the gallbladder fossa was ablated by heat conducted from hot bile was also determined, and the thickness of the ablated area of the liver was measured. RESULTS: The volume reduction ratio in Group A, B and C was 42.7%, 41.7% and 42.9%, respectively (p>.05). In all 15 cases, gallbladder walls lost their transparency and elasticity at about 70 degrees C. In nine of ten cases in Groups B and C, the hepatic capsule around the gallbladder fossa was retracted at about 80 degrees C. The mean thickness of liver parenchymal damage adjacent to the gallbladder was 5.4 mm in Group B and 9.8 mm in Group C. In Group A livers, only one case showed minimal gradual parenchymal change. Microscopically, all three groups showed complete coagulation necrosis of the wall. CONCLUSION: On the basis of this feasibility study, radiofrequency thermal ablation is potentially suitable for the ultrasound-guided treatment of symptomatic cystic lesions including benign hepatic or renal cyst.
Bile ; Catheter Ablation ; Elasticity ; Electrodes ; Feasibility Studies ; Gallbladder* ; Hot Temperature ; Liver ; Necrosis ; Needles ; Pilot Projects*