PURPOSE: Prepapillary vascular loops are blood vessels that project from the optic disc into the vitreous cavity and then return to the disc to continue their natural cource. Ninety percent are arterial in origin and usually congenital anomaly. We report four cases of prepapillary vascular loops associated with retinal and choroidal disorders. METHODS: Prepapillary vascular channel which showed twisted or ringlike appearance without leakage on fluorescein angiogram constituted the diagnosis. However, there may be a sector delay in filling of the optic disc or retina supplied by the loop due to the increased distance that blood must travel through the loop. RESULTS: These cases are not associated with vision-threatening diseases. The associated retinal diseases are diabetic retinopathy in one case, retinitis pigmentosa in one case, contralateral posterior uveitis in two cases. They are arterial loops. In two eyes were observed multiple prepapillary vascular loops. The vessels may appear as simple hairpin loops, spiral, or corkscrew configuration. In our cases, the presence of a cilioretinal artery and retinal artery obstruction could not be determined; other complication associated with prepapillary vascular loops were not observed. CONCLUSIONS: We assume that prepapillary vascular loops may occur acquiredly associated with other ocular diseases.
Arteries ; Blood Vessels ; Choroid ; Diabetic Retinopathy ; Diagnosis ; Fluorescein ; Retina ; Retinal Artery ; Retinal Diseases* ; Retinaldehyde* ; Retinitis Pigmentosa ; Uveitis, Posterior

PURPOSE: The objective of this study was to compare the complications and urodynamic parameters of the patients who underwent orthotopic bladder substitution with using ileocolic or ileal segments after radical cystectomy for treating invasive bladder cancer. MATERIALS AND METHODS: Between January 1990 and April 2006, 260 patients with invasive bladder cancer underwent radical cystectomy and construction of the urinary diversion; ileal conduit, indiana pouch, ileocolic neobladder, ileal neobladder were all done at St. Mary's Hospital. The mean age of the patient was 61.8 years (range: 46-86). The ratio of male and female was 88%/12%. Forty nine patients received an orthotopic ileocolic neobladder and 45 patients received an orthotopic ileal neobladder. The complications and urodynamic parameters were compared in both groups. RESULTS: The orthotopic ileocolic neobladder after radical cystectomy for treating invasive bladder cancer has been performed between 1990 and 1996 and the orthotopic ileal neobladder has been performed between 1996 and 2006. Ileocolic neobladder related complications developed in 10 patients; neobladder leakage in 1 (2%), neobladder rupture in 1 (2%), stricture of the ureteroenteric anastomosis site in 4 (8.2%), and stricture of the urethral anastomosis site in 4 (8.2%). Ileal neobladder related complications developed in 11 patients; ureteroenteric stricture in 7 (15.5%), stricture of the urethral anastomosis site in 3 (6.6%) and acute pyelonephritis in 1 (2.2%). The results of the mean maximal flow rate and mean postvoid residual volume were better in the ileal neobladder group than those in the ileocolic neobladder group. CONCLUSIONS: There were no significant differences in complications between ileocolic neobladder and ileal neobladder. The maximal uroflow and residual urine volume of the ileal neobladder were superior to those of the ileocolic neobladder on urodynamic study.
Colon ; Constriction, Pathologic ; Cystectomy* ; Female ; Humans ; Ileum ; Indiana ; Male ; Pyelonephritis ; Residual Volume ; Rupture ; Urinary Bladder Neoplasms ; Urinary Bladder* ; Urinary Diversion ; Urodynamics*

PURPOSE: To evaluate MRI findings of malignant ovarian tumors. MATERIALS AND METHODS: MRI findings were retrospectively reviewed in 25 patients with surgically confirmed 30 malignant ovarian tumors(common epithelial tumor;23, sex cord stromal tumor;2, endodermal sinus tumor ; 1, metastatic tumor ;4). The findings evaluated were the lesion size, solid and/or cystic component, wall thickness, septal thickness, necrosis, invasion of adjacent organ, ascites, and adenopathy. RESULTS: MRI findings of the malignant ovarian tumors were as follow:Size of lesion was 5-35cm(mean 14cm) ;solid component was present in 80%(24/30);wall thickness was more than 3ram in 90%(27/30);septal thickness was more than 3ram in 70%(21/30);tumor necrosis was present in 40%(12/30%) ;invasion of adjacent organ was present in 76%(19/25);ascites was present in 56%(14/25);lymphadenopathy was present in 24% (6/25). MRI findings of absence of solid component(6/6), even wall and septal thickness(7/7, 19/19) were found only in epithelial tumors. Uneven septal thickness more than 3mm(7/11) was a predominant MRI findings of non-epithelial tumors. Well-defined cystic lesion within solid component was seen in Krukenberg tumors. CONCLUSION: Evaluation of the lesion size, internal architecture, invasion of adjacent organ, ascites, and lymphadenopathy in MRI would enable diagnosis of malignant ovarian tumors and could lead to possible differential diagnosis of epithelial tumors from non-epithelial tumors.
Ascites ; Diagnosis ; Diagnosis, Differential ; Endodermal Sinus Tumor ; Humans ; Krukenberg Tumor ; Lymphatic Diseases ; Magnetic Resonance Imaging* ; Necrosis ; Retrospective Studies

The aim of this study was to evaluate the efficacy of intravenous immunoglobulin treatment for recurrent spontaneous abortion. Immunologic causes in either alloimmune or autoimmune type have been suggested for more than 80% of these patients. Various immunotherapy including paternal leukocyte transfusion has been used, but there is controversy on its efficacy and side-effects. The proposed immunomodulatory mechanism of intravenous immunoglobulin includes passive transfer of blocking or anti-idiotype antibody, blockade of Fc receptors, enhancement of supressor T-cell function, down regulation of B cell function. In this study, we used intravenous immunoglobulin for the prevention of spontaneous abortion. Five patients with a history of two or more spontaneous abortions were enrolled in this study. Other etiologic factors such as anatomical, chromosomal, hormonal factors were excluded. Three of them were positive for anti-cardiolipin antibody (ACA). When the pregnancy was diagnosed at about five weeks of gestation, 30 g intravenous immunoglobulin was administered and continued up to 28 weeks with three weeks. Ongoing pregnancy beyond 20 weeks was considered successful. Four among five patients (80%) was successful in maintaining pregnancy now ongoing 20, 31, 33, 39 weeks. One patient with ACA positive had abartion due to anembryonic pregnancy. No adverse reaction was observed during the treatment. From these preliminary data, it is suggested that intravenous immunoglobulin treatment may be effective in maintaining pregnancy in patients with unexplained recurrent spontaneous abortion, Further studies are needed to clarify the its immunomodulatory mechanism and establish a more simplified protocol limiting the use at certain critical period of time.
Abortion, Spontaneous* ; Critical Period (Psychology) ; Down-Regulation ; Female ; Humans ; Immunoglobulins* ; Immunotherapy ; Leukocyte Transfusion ; Pregnancy ; Receptors, Fc ; T-Lymphocytes

BACKGROUND: Automated external defibrillator(AED) represent a major breakdown to permit more widespread application of the principle of early defibrillation. Many recent efforts to improve emergency medical services(EMS) and increase survival rates are simply efforts to get defibrillation to patients as rapidly as possible. AED is major innovation for the prehospital care of ventricular fibrillation cardiac arrest patients. The purpose of this study is to evaluate the course of initial training to three different groups(nurses, firefighters, and EMT trainee) to use AED. METHOD: We studies the efficacy of education of AED to 33 nurses, 15 EMT trainee, and 16 firefighters. Training lasted 75 mins and included 45 mins an overview of defibrillation, protocols for using the AED, and operation of the AED(Laerdal Heartstart 3000),15 mins demonstraion.4 check list was used to grade the performance of cardiopulmonary resuscitation, operation of the AED, and the time required to deliver the first three defibrillations. RESULT: There were no statistically significant differences in performance and time required to deliver an electrical countershock among the groups(p=0.4). To the second test 92fo of all group completed all steps successfully. The step most often foiled was the preparing of the AED for defibrillation. CONCLUSION: In nurses, EMT trainee, and firefighters, it is both feasible and effective to train AED use irrespective of the degree of the trainee.
Cardiopulmonary Resuscitation ; Defibrillators* ; Education ; Emergencies* ; Firefighters* ; Heart Arrest ; Humans ; Survival Rate ; Ventricular Fibrillation

No abstract available.
Humans ; Polyamines*

OBJECTIVE: To investigate the relationship between serum creatine kinase (CK) level and pulmonary function in Duchenne muscular dystrophy (DMD). METHODS: A total of 202 patients with DMD admitted to the Department of Rehabilitation Medicine, Gangnam Severance Hospital were enrolled from January 1, 1999 to March 31, 2015. Seventeen patients were excluded. Data collected from the 185 patients included age, height, weight, body mass index, pulmonary function tests including forced vital capacity (FVC), peak cough flow, maximal expiratory pressure (MEP), and maximal inspiratory pressure (MIP), and laboratory measurements (serum level of CK, CK-MB, troponin-T, and B-type natriuretic peptide). FVC, MEP, and MIP were expressed as percentages of predicted normal values. RESULTS: Serum CK activities were elevated above normal levels, even in the oldest DMD group. Serum CK level was strongly correlated with pulmonary functions of sitting FVC (p<0.001), supine FVC (p<0.001), MIP (p=0.004), and MEP (p<0.001). CONCLUSION: Serum CK level is a reliable screening test even in patients with advanced DMD, and is a strong predictor of pulmonary functions.
Body Weight ; Cough ; Creatine Kinase* ; Creatine* ; Humans ; Mass Screening ; Muscular Dystrophy, Duchenne* ; Reference Values ; Rehabilitation ; Respiratory Function Tests ; Troponin T ; Vital Capacity

PURPOSE: To correlate the findings of perfusion-weighted imaging (PWI) with clinical outcomes in patients with acute lacunar infarction. MATERIALS AND METHODS: Eleven patients (7 males and 4 females) with acute lacunar infarction who were examined within 50 (mean, 29) hours of the onset of symptoms underwent conventional MRI, diffusion-weighted imaging (DWI) and PWI. Gadolinium (0.2 mmol/kg) was injected at a rate of 2 ml/sec, and PWI was performed using a gradient-echo EPI pulse sequence and the following parameters: TR/TE, 2000/60; flip angle, 90 degree; matrix size, 128X128. Relative cerebral blood volume (rCBV) maps were derived from gadolinium bolus perfusion-weighted images where rCBV ratios between infarcted areas were detected by DWI, and contralateral control areas were obtained. In each case, the resulting rCBV ratio at a lesion site was compared with the clinical outcome determined on the basis of the difference between National Institute Health Stroke Scale (NIHSS) scores at admission and discharge. RESULTS: With the aid of the time-intensity curve obtained at PWI, the rCBV maps revealed a hypoperfused area in 10 of 11 patients, and there was positive correlation (r=0.81) with clinical outcome. CONCLUSION: Although PWI has a lower detection rate than DWI, it may be a useful modality for helping determine prognosis in cases of acute lacunar infarction.
Blood Volume ; Diagnosis* ; Gadolinium ; Humans ; Magnetic Resonance Imaging* ; Male ; Perfusion* ; Prognosis ; Stroke ; Stroke, Lacunar*

Limb-girdle muscular dystrophy type 2B (LGMD2B), a subtype of autosomal recessive limb-girdle muscular dystrophy (ARLGMD), is characterized by a relatively late onset and slow progressive course. LGMD2B is known to be caused by the loss of the dysferlin protein at sarcolemma in muscle fibers. In this study, the clinical and pathological characteristics of Korean LGMD2B patients were investigated. Seventeen patients with ARLGMD underwent muscle biopsy and the histochemical examination was performed. For the immunocytochemistry, a set of antibodies against dystrophin, alpha, beta, gamma, delta-sarcoglycans, dysferlin, caveolin-3, and beta-dystroglycan was used. Four patients (24%) showed selective loss of immunoreactivity against dysferlin at the sarcolemma on the muscle specimens. Therefore, they were classified into the LGMD2B category. The age at the onset of disease ranged from 9 yr to 33 yr, and none of the patients was wheelchair bound at the neurological examination. The serum creatine kinase (CK) was high in all the patients (4010-5310 IU/L). The pathologic examination showed mild to moderate dystrophic features. These are the first Korean LGMD2B cases with a dysferlin deficiency confirmed by immunocytochemistry. The clinical, pathological, and immunocytochemical findings of the patients with LGMD2B in this study were in accordance with those of other previous reports.
Adolescent ; Adult ; Age of Onset ; Child ; Creatine Kinase/blood ; Disease Progression ; Female ; Human ; Immunohistochemistry ; Korea ; Male ; Membrane Proteins/biosynthesis ; Muscle Proteins/biosynthesis ; Muscles/pathology ; Muscular Dystrophies/*diagnosis/*metabolism ; Support, Non-U.S. Gov't ; Time Factors

Congenital myopathies are clinical and genetic heterogeneous disorders characterized by skeletal muscle weakness and specific structural changes in muscle fiber. Congenital myopathy with fiber type disproportion (CFTD) is an established disorder of congenital myopathy. CFTD is characterized by non-progressive childhood neuromuscular disorders with a relatively good prognosis and type 1 fiber predominance and smallness. Congenital myopathy with type 1 fiber predominance (CMT1P) is also a distinct entity of congenital myopathy characterized by non-progressive childhood neuromuscular disorders and type 1 fiber predominance without smallness. Little is known about CMT1P. Clinical characteristics, including dysmorphic features such as hip dislocation, kyphoscoliosis, contracture, and high arch palate, were analyzed along with laboratory and muscle pathologies in six patients with CMT1P and three patients with CFTD. The clinical manifestations of CFTD and CMT1P were similar. However, the frequency of dysmorphic features is less in CMT1P than in CFTD. Long term observational studies of CMT1P are needed to determine if it will change to another form of congenital myopathy or if CMT1P is a distinct clinical entity.
Myopathies, Structural, Congenital/*diagnosis ; Muscular Diseases/*pathology ; Muscles/pathology ; Male ; Infant ; Humans ; Female ; Child, Preschool ; Child ; Biopsy ; Adult