Distal myopathy with rimmed vacuoles is a rare muscle disease and, so far as we know, it has not been reported in Korea as yet. This disorder is known to be inherited as autosomal recessive trait and to have no specific treatment. Hereby, we report 3 patients of distal myopathy with rimmed vacuoles in a family. The clinical characteristics of these patients were slowly progressive symmetrical muscle weakness and wasting of all 4 extremities, worse in distal legs. The pretibial muscles were involved more markedly than the calf muscles. Serum muscle enzymes were increased. The prominent EMG findings were myopathic changes, but reduced recruitment was occasionally found in some distal muscles The muscle biopsies of right biceps brachii muscle were performed in two patients, which showed the characteristic rimmed vacuoles by light microscope. Membranous whorls and randomly oriented intracytoplasmic filaments were found by electron microscope. The severity of pathological abnormalities were related to the clinical status of the patient. One had been treated with steroid(prednisolone) for several years but with no improvement.
Biopsy ; Distal Myopathies* ; Extremities ; Humans ; Korea ; Leg ; Muscle Weakness ; Muscles ; Vacuoles*

PURPOSE: We wanted to evaluate the short term clinical and radiological results and the complications of primary total hip arthroplasty with using a Accolade TMZF femoral stem. MATERIALS AND METHODS: 66 cases among 80 cases underwent total hip arthroplasty by one surgeon with using an Accolade TMZF femur stem from January 2002 to August 2006. The minimum follow-up was 30 months and we retrospectively analyzed these 66 patients. RESULTS: The mean Harris Hip Score improved from 54 to 92 at the last follow up. On the last follow-up X-ray, all the femoral stems (100%) showed stable fixation and there was no osteolysis or loosening, but there were 34 cases of stress shields and 20 cases of cortical hypertrophy. The acetabular component revealed stable fixation in 65 cases (98.5%) and loosening in one case (1.5%). There was no acetabular osteolysis, but 22 cases of radiolucency were observed in the Dee Lee and Charnley zone II. As complications, there were 5 cases of heterotrophic ossification, 1 case of greater trochanteric bursitis, 2 cases of thigh pain and 1 case with a squeaking sound. CONCLUSION: Primary total hip arthroplasty using a cementless Accolade TMZF femoral stem showed good results in the short term, but long term follow up is needed.
Arthroplasty ; Bursitis ; Femur ; Follow-Up Studies ; Hip ; Hypertrophy ; Osteolysis ; Retrospective Studies ; Thigh

PURPOSE: This study evaluated the shortening and rotational deformity after closed intramedullary nailing of femur shaft fracture according to Winquist-Hansen classification type. MATERIALS AND METHODS: This study was based on 98 cases who received cloased intramedullary fixation about their femur shaft fractures between January 2000 and October 2005 with minimum 12 months follow up. The rotational deformity was analysed by Yang's method (45 cases) preoperatively and postoperatively, and the shortening by orthoradiogram (55 cases). Furthermore we analysed other complications, for example nonunion, infection, and metal failure. RESULTS: We found more than 15 degrees anteversion difference of both femurs in 10 cases. Among them, 9 cases were classified to type 3, 4. According to Winquist-Hansen classification, rotational deformity ranged from 3.7° (Type 1) to 8.9° (Type 4). More than 2 cm leg length discrepancy (LLD) was found in 9 cases, all of them were classified as Winquist-Hansen classification type 3, 4. In the type 1, LLD was checked as 3.2 mm and type 4, 14.2 mm. CONCLUSION: To prevent the shortening and rotational deformity after intramedullary fixation of Winquist-Hansen classification type 3, 4 femur shaft fracture, intraoperatively the exact contralateral femoral anteversion and length should be checked.
Classification* ; Congenital Abnormalities* ; Femur* ; Follow-Up Studies ; Fracture Fixation, Intramedullary* ; Leg ; Methods

Idiopathic restrictive cardiomyopathy is characterized by clinical and hemodynamic findings of restrictive cardiomyopathy in the absence of morphologic cause. The differential diagnosis between idiopathic restrictive cardiomyopathy and noncalcified constrictive pericarditis is difficult but the distinction is crucial because of the therapeutic implication. The diagnosis of idiopathic restrictive cardiomyopathy is aided by echocardiography and cardiac catheterization, cardiac magnetic resonance imaging. The patients should be approached systemically to differentiate from noncalcified constritive pericarditis.
Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Diagnosis ; Diagnosis, Differential ; Echocardiography ; Hemodynamics ; Humans ; Magnetic Resonance Imaging ; Pericarditis ; Pericarditis, Constrictive

PURPOSE: This study estimated the prevalence of lower urinary tract symptoms (LUTS) in Korean men and the conditions for being diagnosed with or treated for LUTS/benign prostatic hyperplasia (BPH). METHODS: We analyzed cross-sectional data collected from 69,851 Korean men who were 40 years of age or older and had participated in the Korean Community Health Survey performed in 2011. Interviewers performed face-to-face surveys that included sociodemographic questions, the International Prostate Symptom Score (IPSS), and questions regarding whether the subjects had been diagnosed with or treated for LUTS/BPH. We estimated the prevalence of LUTS and assessed whether the subjects had been diagnosed with or treated for LUTS/BPH. RESULTS: Moderate to severe LUTS, storage symptoms, and voiding symptoms increased with age. The IPSS quality of life score was 1.5±0.004 in the mild LUTS group (n=57,701), 3.3±0.01 in the moderate LUTS group (n=9,203), and 4.3±0.02 in the severe LUTS group (n=2,947) (P<0.0001). The prevalence of moderate to severe LUTS in those who had not been diagnosed with LUTS/BPH was 64.5% (7,847 of 12,150), and the prevalence of moderate to severe LUTS in those who had been diagnosed with LUTS/BPH but had not been treated was 23.5% (2,853 of 12,150). CONCLUSIONS: The severity of LUTS in Korean men increased with age, and the IPSS quality of life score increased with the severity of LUTS. Many Korean men with moderate to severe LUTS had not been diagnosed or treated for LUTS/BPH. Socioeconomic conditions played an important role in health-seeking behavior among patients with LUTS/BPH.
Health Surveys* ; Humans ; Lower Urinary Tract Symptoms* ; Male ; Prevalence ; Prostate ; Prostatic Hyperplasia ; Quality of Life

In 17 myotonic cases (16 of myotonic muscular dystrophy and 1 of congenital myotonia), 13 cases showed significant decremental responses on RNS study. The decremental responses had the tendency of more prominence on faster stimulating rate, and in the proximal limb muscles. This result suggests that the RNS test is useful but not specific for the disorder of neuromuscular junction.
Extremities ; Humans ; Muscles ; Muscular Dystrophies ; Neuromuscular Junction

PURPOSE: A retrospective study was undertaken to determine the role of conventional radiotherapy with or without surgery for treating a supraglottic carcinoma in terms of the local control and survival. MATERIALS AND METHODS: From Jan. 1986 to Oct. 1996, a total of 134 patients were treated for a supraglottic carcinoma by radiotherapy with or without surgery. Of them, 117 patients who had completed the radiotherapy formed the base of this study. The patients were redistributed according to the revised AJCC staging system (1997). The number of patients of stage I, II, III, IVA, IVB were 6 (5%), 16 (14%), 53 (45 %), 32 (27%), 10 (9%), respectively. Eighty patients were treated by radical radiotherapy in the range of 61.2~79.2 Gy (mean : 69.2 Gy) to the primary tumor and 45.0~93.6 Gy (mean : 54.0 Gy) to regional lymphatics. All patients with stage I and IVB were treated by radiotherapy alone. Thirty-seven patients underwent surgery plus postoperative radiotherapy in the range of 45.0~68.4 Gy (mean : 56.1 Gy) to the primary tumor bed and 45.0~59.4 Gy (mean : 47.2 Gy) to the regional lymphatics. Of them, 33 patients received a total laryngectomy (+-lymph node dissection), three had a supraglottic horizontal laryngectomy (+-lymph node dissection), and one had a primary excision alone. RESULTS: The 5-year survival rate (5YSR) of all patients was 43%. The 5YSRs of the patients with stage I+I, III+V were 49.9%, 41.2%, respectively (p=0.27). However, the disease-specific survival rate of the patients with stage I (n=6) was 100%. The 5YSRs of patients who underwent surgery plus radiotherapy (S+T) vs radiotherapy alone (RT) in stage II, III, IVA were 100% vs 43% (p=0.17), 62% vs 52% (p=0.32), 58% vs 6% (P<0.001), respectively. The 5-year actuarial locoregional control rate (5YLCR) of all the patients was 57%. The 5YLCR of the patients with stage I, II, III, IVA, IVB was 100%, 74%, 60%, 44%, 30%, respectively (p=0.008). The 5YLCR of the patients with S+T vs RT in stage II, III, IVA was 100% vs 68% (p=0.29), 67% vs 55% (p=0.23), 81% vs 20% (P<0.001), respectively. In the radiotherapy alone group, the 5YLCR of the patients with a complete, partial, and minimal response were 76%, 20%, 0%, respectively (P<0.001). In all patients, multivariate analysis showed that the N-stage, surgery or not, and age were significant factors affecting the survival rate and that the N-stage, surgery or not, and the ECOG performance index were significant factors affecting the locoregional control. In the radiotherapy alone group, multivariate analysis showed that the radiation response and N-stage were significant factors affecting the overall survival rate as well as locoregional control. CONCLUSION: In early stage supraglottic carcinoma, conventional radiotherapy alone is an equally effective modality compared to surgery plus radiotherapy and could preserve the laryngeal function. However, in the advanced stages, radiotherapy combined with concurrent chemotherapy for laryngeal preservation or surgery should be considered. In bulky neck disease, all the possible planned neck dissections after induction chemotherapy or before radiotherapy should be attempted.
Drug Therapy ; Humans ; Induction Chemotherapy ; Laryngectomy ; Multivariate Analysis ; Neck ; Neck Dissection ; Radiotherapy* ; Retrospective Studies ; Survival Rate

Sensory neuropathy is characterized by pure sensory dysfunction, ultimately evolving into areflexia and severe sensory ataxia in spite of the preservation of the other functions in the nervous system such as motor function. This disorder is known to be commonly found in cancer patients as a paraneoplastic syndrome, but nowadays many reports are made that sensory neuropathy is not merely related to malignancy but also with autoimmune disorders such as Sjogren's syndrome or polyclonal gammopathy, etc. Hence we report two patients who have been diagnosed as chronic idiopathic ataxic sensory neuropathy through electrophysiological and histopathological studies without any evidence of cancer or any other known cause.
Ataxia ; Humans ; Nervous System ; Paraneoplastic Syndromes ; Sjogren's Syndrome

Exposure to intense noise has been identified as a potential risk in the development of hearing impairment. Social concern about excessive noise is increasing and this also extends to the operating room. A noise level study was performed in the operating room during mastoidectomy with a sound level meter and it was analyzed by a sound-analyzing program. The drilling instruments used included the Stryker, Midas, M.P.S. and Med-Next. The operator was exposed to sound levels from 69 to 83 dBA. The loudest drilling instrument was the Midas and it produced an average sound level of 83 dBA to the operator. The mean exposure time was 41 minutes during mastoidectomy. This is below the occupational noise-level regulations in Korea. However, considering that individual susceptibility to noise varies and that the otologic surgeon is repeatedly exposed to prolonged drilling noise, ear protection is recommended for the operators of high-speed drilling instruments.
Chronic Disease ; Human ; Mastoid/surgery* ; Noise*/adverse effects ; Otitis Media/surgery ; Otolaryngology/instrumentation*

PURPOSE: To compare and analyze length change of patella tendon after intramedullary nailing of tibial shaft fracture using transtendinous approach. MATERIALS AND METHODS: Thirty-two cases were analyzed from December, 1999 to December, 2005. Insall Salvati ratios were estimated. Severity of initial trauma, duration of nail retension, knee function and pain on change of length of patellar tendon was evaluated. RESULTS: Mean duration of nail retention was twenty-two months. The shortening of patella tendon was observed in 25 cases (p<0.001). The effect of AO type and the duration of nail retension on the decrease of Insall Salvati ratio was not significant (p>0.05, p=0.778). Lysholom score decrease to 89.5. There was no significant difference between the shortening of patellar tendon length and knee pain (p=0.058). CONCLUSION: After intramedullary nailing for closed tibia fracture, shortening of patellar tendon length is observed. That is irrelevant to the fracture type and the duration of nail retension. The shortening of patella tendon length may contribute to decreasing of knee function, but it was no significance of knee pain after intramedullary nailing.
Fracture Fixation, Intramedullary ; Knee ; Nails ; Patella ; Patellar Ligament ; Retention (Psychology) ; Tibia ; Tibial Fractures