We present the first case report of fragile X-associated tremor ataxia syndrome (FXTAS) in the Republic of Korea. A 75-year-old male developed progressive gait ataxia, parkinsonism, and a mood disorder. Magnetic resonance imaging revealed T2 high signal intensity within the middle cerebellar peduncles. Analysis of the fragile X mental retardation 1 gene revealed a CGG trinucleotide repeat number of 136. FXTAS should be considered when a patient has atypical parkinsonism, cerebellar ataxia, and specific MRI abnormalities.

Thirty-one distal femoral and nine proximal tibial primary bone tumor patients who had prosthetic replacements were reviewed retrospectively. Average follow-up was thirty-eight months(range: 12- 109 months). There were thirty-two(osteosarcoma: 29, chondrosarcoma: 3) stage IIB lesions and eight 1B lesions(giant cell tumor). Twenty-nine patients were surviving at final follow-up. Overall prosthetic survival was 81%, 27% at 2 and 5 year respectively. Eleven prostheses were revised. The causes of revisions were infection(7), metal failure(2), fracture of host bone(1), and loosening(1). Eight revisions were successful, one was fair, and two patients needed an amputation. Prognostic factors which were analyzed for survival of prostheses were age, sex, location of primary lesion, percent of bone resected, and stage. Only the location of primary lesion showed statistical significance and diabolic pattern of survival curve was noted between two groups in resection length(<40% versus >40%). Average functional score was 26. The tibial side had worse prosthetic survival and a major threat to this procedure was due to infection.
Amputation ; Chondrosarcoma ; Follow-Up Studies ; Humans ; Knee Joint* ; Knee* ; Prostheses and Implants ; Prosthesis Failure ; Retrospective Studies ; Sarcoma

No abstract available.
Adult ; Fingers ; Humans

Background: Shoulder arthroplasty is technically demanding and relies heavily on the precision of surgical techniques. Proper glenoid component sizing plays a crucial role in successful shoulder arthroplasty. We measured the size and penetrative depth of the glenoid for peg or screw fixation in nonarthritic and degenerative arthritic shoulders by using three-dimensional computed tomography to determine the reference dimensions of the glenoid in nonarthritic and degenerative arthritic shoulders. Methods: From January 2010 to January 2011, data on two groups of patients were collected and reviewed. Group 1 comprised 38 patients who underwent surgical treatment due to fracture of the proximal humerus and who had no evidence of a pathological glenoid. Group 2 comprised 14 patients who underwent surgical treatment due to osteoarthritis of the glenohumeral joint. The height (maximal superoinferior diameter) of the glenoid was measured, and the width (anteroposterior [AP] diameter) of the glenoid was measured at five different levels (H1–H5). Axial images were taken at H1–H5 levels, the AP glenoid diameter of each was divided into eight areas, and division points were labeled as W1–W7. The penetrative depths between the near cortex and far cortex of the glenoid (thickness) at each point (W1–W7) were measured. Results: The overall mean glenoid height was 37.67 ± 4.09 mm in nonarthritic glenoids and 39.42 ± 3.54 mm in degenerative arthritic glenoids. The nonarthritic glenoid was significantly thicker than the degenerative arthritic glenoid at the H1W3, H1W4, H1W5, H2W7, H3W1, H3W6, H3W7, H4W5, H4W6, H4W7, H5W4, H5W5, H5W6, and H5W7 points. The posteroinferior quadrant had the smallest penetrative depth in both nonarthritic and degenerative arthritic glenoids. Also, the degenerative arthritic glenoids were significantly thinner than the nonarthritic glenoids along the posterior and inferior parts of the glenoid. Conclusions The posterior and inferior parts of the degenerative arthritic glenoid appears thinner than the nonarthritic glenoid. Thus, caution has to be taken when drilling the screw hole or inserting screws into the posteroinferior parts, where the glenoid is thinner than 15 mm on average, to avoid penetration of the far cortex.

We developed the 166Ho-chitosan, the new radiation synovectomy agent. Holmium is the more practical isotope based on its longer half-life. And chitosan, is ideal and suitable particles based on its soluble and biodegradable characteristics. We investigated the biocompatibility of the 166Ho-chitosan complex to evaluate the suitability as a radiation synovectomy agent. In vitro stability test, the 166Ho-chitosan complex suspension in saline was stored at room temperture and 37degrees C for 25 days and decay rate was of determined by ITLC(Instant Thin Layer Chromatography). In vivo stability test, the 166Ho-chitosan complex was injected into rabbit joints and followed by gamma camera imaging to quantify extra-articular leakage. Biodistribution study, the 166Ho-chitosan complex was injected into rabbit joints. After 48 hours heart, liver, urinary bladder, spleen, lung, brain, kidney, blood were extracted and radioactivities were measured. In vitro stability test, there was no significant change of radioactivity and no leakage problem indicating the prepared 166Ho-chitosan complex is sufficiently stable. In vivo stability tests revealed that more than 98% of the 166Ho-chitosan remained in joint over a 2 days period. The mean retention percentage of 166Ho-chitosan in knee were 99.9%, 99.9%, 99.8%, 99.7% at 2 h, 6 h, 1 day and 2 days, respectively. A biodistribution study of the rabbits revealed that leakage to heart, liver, urinary bladder, spleen, lung, brain, kidney, blood is 0.71, 1.5, 0.50, 1.5, 0.25, 0.26, 0.81, 0.065(% Injected Dose x 10-3/gram), respectively. The 166Ho-chitosan complex shows less leakge than any other radiation synovectomy agents. Our results indicate that 166Ho-chitosan have the biocompatibility and the suitability as a radiation synovectomy agent.
Brain ; Chitosan* ; Half-Life ; Heart ; Holmium ; Joints ; Kidney ; Knee ; Liver ; Lung ; Rabbits* ; Radioactivity ; Radionuclide Imaging ; Spleen ; Urinary Bladder

BACKGROUND: Early reperfusion therapy with thrombolytic agents or primary PTCA is most important to salvage ischemic myocardium in acute myocardial infarction(AMI). Timely reperfusion of jeopardized myocardium clearly improves hemodynamics, decreases infarct size and improves survival. The extent of protection appears to be directly related to the rapidity of reperfusion after onset of coronary occlusion. Although the intravenous thrombolysis is a feasible therapy in the patients with evolving AMI, the benifit of thrombolytic therapy decreases because of the time delay after onset of symptom. This study was perfomed to analyze the factors time delay between onset of symptom and the thrombolytic therapy with retrospective and prospective questionaire in the patients with AMI. METHOD: Eighty one patients with AMI were included in this study who came to the emergency room(ER) of Chungnam National University Hospital(CNUH) from Feburary 1995 to October 1996. Delay between door and thrombolytic therapy was defined as hospital time delay. RESULTS: Thrombolytic therapy(rt-PA or urokinase iv) was done in 60 patients(74.1%) and mean prehopital time delay was significantly decreased in the patients with thrombolytic therpapy when compared with those without thormbolytic threapy(462+/-90 vs 1375+/-473 minutes, p=0.005). There were no singificant factors for prehospital time delay such as age, sex, redsidence, ER near residence, transfer time to ER near residence, family status, family history of AMI, severity of chest pain, presence of risk factors of cardiovascular disease(CVD), previous CVD, degree of education, history of other disease and routine check, transfer methods. The only 8 patients(9.8%) knew about AMI and 7 patients among these patient came to ER earlier and received thrombolytic therapy. From 57 referred patients, 40 patients(70.2%) received reperfusion therapy and only 30 patients(52.6%) had recored EKG in the referred hospital. In the analysis of hospital delay from patient's arrival to the thrombolytic therapy, the arrival time at weekdays and weekend had no differences, but hospital delay were significantly prolonged when patients arrived at ER in the night. CONCLUSION: Since prehospital time delay is a most important factor of time delay for the effective thrombolytic therapy in AMI, the pubic education program and effective transport system are needed. And routine record of EKG in patient with chest pain in the local hospital is very helpful to start effective thromolytic therapy at ER. The well designed prospective study with more patinets in our local region is essential to get more accurate information about transport system and to improve survival rate in patients with AMI.
Chest Pain ; Chungcheongnam-do ; Coronary Occlusion ; Education ; Electrocardiography ; Emergencies ; Fibrinolytic Agents ; Hemodynamics ; Humans ; Myocardial Infarction* ; Myocardium ; Prospective Studies ; Reperfusion ; Retrospective Studies ; Risk Factors ; Survival Rate ; Thrombolytic Therapy* ; Urokinase-Type Plasminogen Activator

BACKGROUND: The aging of the patient population is one of rhe most important factirs influencing health care delivery. Currently 5% of the Korean population is elderly, defined as older than 65years of age, with this group projected to increase to 13.1% by the year 2021. Cardiovascular disease is the leading cause of death and of disability in the elderly age group. mong them, coronary heart disease is the most importane. METHOD: Study population composed of 216 patients who were admittied to the hospital with first acute myocardial infarction and they were divided into two groups according to the age(older than 65 years of age vs younger). Clonical features, risk factors of coronary heart disease, in-hospital outcome and complication were compared in elderly patients and others group. Results : 1) The risk factors of coronary heart disease is similar to younger patients but pattern of chest pain is less typical than younger patients. 2) Clinical presentation of elderly patients is similar to younger patients except Killip class on admission.(1.66vs 1.91,P=0.04) 3) In-hospital mortality of elderly patients in higher than younger patients. In addition to an increased incidence of death, recurrent ischemia, stroke, AV block, ventricular arrythmia, pulmonary edema occured more frequently with advanced age. 4) Especially in the thrombolytic therapy group, in-hospital death, reinfarction and recurrent ischemia is higher than primary PTCA group in elderly patients. CONCLUSIONS: Diagnosis of acute chest pain is difficult in elderly patients and in-hospital mortality and morbidity is higher than in younger patients. Thus more accurate diagnosis and discriminative therapeutic modality is needed.
Aged ; Aging ; Arrhythmias, Cardiac ; Atrioventricular Block ; Cardiovascular Diseases ; Cause of Death ; Chest Pain ; Coronary Disease ; Delivery of Health Care ; Diagnosis ; Hospital Mortality ; Humans ; Incidence ; Ischemia ; Myocardial Infarction* ; Pulmonary Edema ; Risk Factors ; Stroke ; Thrombolytic Therapy

For the treatment of coarction of aorta, surgical intervention has been known as a standard therapy.During last decade balloon angioplasty for coarctation of the aorta has been reported as a successful and safe procedure in about 300 cases. This angioplasty was done mainly in infants and children, and little cases in adults and adolescents. A 22 year-old adult with coarctation of aorta have recieved balloon angioplasty. He visited to emergency room due to severe headache and the blood presure of arm was 240/130mmHg at emergency room. The blood pressure at ward was 168/92mmHg in upper extremities, 104/82mmHg in lower extrimities. His aortogram showed coarctation of thoracic aorta below left subclavian artery. The pressure gradient beween ascending aorta and right femoral artery was decreased from 60mmHg to 0mmHg after balloon dilatation (2 times, balloon diameter 18mm). There were no significant complications. The follow-up magnetic resonance image in 4 month after balloon angioplasty showed no evidence of restenosis or saccular aneurysm. Initial hypertension turned to normal blood pressure in 4 months after balloon angioplasty. This adult case of successful balloon angioplasty for coarctation of aorta is the first case reported in Korea.
Adolescent ; Adult* ; Aneurysm ; Angioplasty ; Angioplasty, Balloon* ; Aorta ; Aorta, Thoracic ; Aortic Coarctation* ; Arm ; Blood Pressure ; Child ; Dilatation ; Emergency Service, Hospital ; Femoral Artery ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Infant ; Korea ; Subclavian Artery ; Upper Extremity ; Young Adult

BACKGROUND: We compared clinical properties and patient satisfaction between spinal anesthesia and popliteal sciatic nerve block (PSNB) for hallux valgus surgery. METHODS: Forty patients undergoing hallux valgus surgery were divided into spinal group (spinal anesthesia with 2.5 ml of 0.5% bupivacaine [n = 20]) and PSNB group (PSNB with 30 ml of 0.75% ropivacaine mixed with 10 ml of normal saline solution using a nerve stimulator [n = 20]). The PSNB group used a patient-controlled-analgesia (PCA) pump for postoperative pain control. The quality and side effects were compared between the two groups. A questionnaire was used to evaluate patient satisfaction with the use of anesthetic techniques and postoperative pain control in the PSNB group. This study was assessed 3 days postoperatively by a blinded observer. RESULTS: Procedure time and time from anesthesia until start of sugery were significantly shorter in the spinal group than those in the PSNB group (P < 0.01). Anesthesia-related complications such as hypotension, bradycardia, shivering, nausea/vomitting, post-dural puncture headache (PDPH) and urinary retension were observed in 15%, 10%, 5%, 5%, 10%, and 20% of patients in the spinal group, respectively. PSNB was not associated with these complications. Patient satisfaction was slightly higher for PSNB than for spinal anesthesia. In the PSNB group, patient satisfaction with postoperative pain-control was 95% above ordinary satisfaction. CONCLUSIONS: Despite the long duration of the procedure, PSNB is relatively safe, provides an adequate level of anesthesia, effectively controls postoperative pain and reduces side effects. Therefore, PSNB could be a potential anesthetic technique for hallux valgus surgery.
Amides ; Anesthesia ; Anesthesia, Spinal ; Bradycardia ; Bupivacaine ; Hallux ; Hallux Valgus ; Humans ; Hypotension ; Nerve Block ; Pain, Postoperative ; Patient Satisfaction ; Post-Dural Puncture Headache ; Surveys and Questionnaires ; Sciatic Nerve ; Shivering ; Sodium Chloride

PURPOSE: Although survival of osteosarcoma patient has markedly improved, cases of non-responders to chemotherapy and late-relapsers are still perplexing. Our strategy was to analyze the impact of chemotherapy and surgery on the survival for each stage, and to evaluate long-term survival and find prognostic factors within the same stage. MATERIALS AND METHODS: From May 1985 to Feb. 1999, 461 osteosarcomas were enlisted at our department and among them 348 cases were evaluable. There were 1 IB, 4 IIA, 302 IIB, and 41 IIIB. Two hundred and fifty-five (IIA/IIB:4/251) out of 348 cases followed our protocol of chemotherapy and surgery. Two hundred and ten cases had neoadjuvant chemotherapy and 45 had adjuvant only. RESULTS: Eleven year event free survival of the neoadjuvant group was 56.7%. On univariate analysis for 210 neoadjuvant cases, age (<13yrs), type of surgery (amputation), type of chemotherapy (methotrexate, adriamycin, cisplatin), pathologic response (>90%), local recurrence, pathologic fracture, location and size were statistically significant. But multivariate one revealed age, type of surgery, local recurrence and pathologic response as useful factors. There were 12 local recurrences (5.7%) and 100 metastasis among 255 stage II and their average onset from treatment was 17.8 month. Survival after metastasis was 5.6% at 55 months and the aggressively treated group made gains in survival (P<0.0001). Survival of 41 stage III was 0% at 64 months and this group also had an advantage in survival through intensive chemotherapy and surgery for primary and metastatic lesions (p=0.04). CONCLUSION: Long-term survival of the treated 255 stage II group was 55% at 14 years. For stage II: age, local recurrence and pathologic response were meaningful prognostic factors. Aggressive surgery and chemotherapy were necessary to improve the survival of stage III and stage II with late metastasis group.
Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Fractures, Spontaneous ; Humans ; Korea* ; Neoplasm Metastasis ; Osteosarcoma* ; Recurrence ; Survival Rate*