Purpose: Outcome analysis of urachal cancer (UraC) is limited due to the scarcity of cases and different staging methods compared to urothelial bladder cancer (UroBC). We attempted to assess survival outcomes of UraC and compare to UroBC after stage-matched analyses. Materials and Methods: Total 203 UraC patients from a multicenter database and 373 UroBC patients in single institution from 2000 to 2018 were enrolled (median follow-up, 32 months). Sheldon stage conversion to corresponding TNM staging for UraC was conducted for head-to-head comparison to UroBC. Perioperative clinical variables and pathological results were recorded. Stage-matched analyses for survival by stage were conducted. Results: UraC patients were younger (mean age, 54 vs. 67 years; p < 0.001), with 163 patients (80.3%) receiving partial cystectomy and 23 patients (11.3%) radical cystectomy. UraC was more likely to harbor ≥ pT3a tumors (78.8% vs. 41.8%). While 5-year recurrence-free survival, cancer-specific survival (CSS) and overall survival were comparable between two groups (63.4%, 67%, and 62.1% in UraC and 61.5%, 75.9%, and 67.8% in UroBC, respectively), generally favorable prognosis for UraC in lower stages (pT1-2) but unfavorable outcomes in higher stages (pT4) compared to UroBC was observed, although only 5-year CSS in ≥ pT4 showed statistical significance (p=0.028). Body mass index (hazard ratio [HR], 0.929), diabetes mellitus (HR, 1.921), pathologic T category (HR, 3.846), and lymphovascular invasion (HR, 1.993) were predictors of CSS for all patients. Conclusion Despite differing histology, UraC has comparable prognosis to UroBC with relatively favorable outcome in low stages but worse prognosis in higher stages. The presented system may be useful for future grading and risk stratification of UraC.

Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus-2 not yet has established its treatment, but convalescent plasma has been expected to increase survival rates as in the case with other emerging viral infections. We describe two cases of COVID-19 treated with convalescent plasma infusion. Both patients presented severe pneumonia with acute respiratory distress syndrome and showed a favorable outcome after the use of convalescent plasma in addition to systemic corticosteroid. To our knowledge, this is the first report of the use of convalescent plasma therapy for COVID-19 in Korea.

PURPOSE: To report a case of recurrent intraocular hemorrhage due to type A hemophilia in a female patient without any previous medical history.CASE SUMMARY: A 51-year-old female patient without any previous medical history was referred to our clinic due to blurred vision in her left eye. Slit lamp microscopy of the anterior segment was nonspecific. Fundus examination revealed vitreous hemorrhage with retinal tear in her left eye. Vitrectomy and cataract surgery were performed. One day after surgery, hyphema and vitreous hemorrhage recurred. A coagulation disorder was suspected and further serological evaluation was conducted. Coagulation factor analyses showed that the activity of coagulation factors 8 and 12 decreased to 25% and 47%, respectively. Genetic sequence analyses were conducted, and a missense mutation of C6724G> A] was found in exon 25, and type A hemophilia was confirmed. CONCLUSIONS In patients who tend to show persistent bleeding even after proper treatment, hematological evaluation including coagulation factor assays, and the possibility of rare diseases such as hemophilia should be considered.

BACKGROUND/AIMS: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. METHODS: We analyzed 74 consecutive patients (3.8%) who survived out-of-hospital cardiac arrest among 1,986 patients who had angiographically proven coronary vasospasms. Electrical abnormalities were evaluated in serial follow-up electrocardiograms (ECGs) during and after the index event for a 3.9 years median follow-up. Major clinical events were defined as the composite of death and recurrent SCD events. RESULTS: Forty five patients (60.8%) displayed electrocardiographic abnormalities suggesting IPAS: Brugada type patterns in six (8.2%), arrhythmogenic right ventricular dysplasia patterns in three (4.1%), long QT syndrome pattern in one (2.2%), and early repolarization in 38 (51.4%). Patients having major clinical events showed more frequent Brugada type patterns, early repolarization, and more diffuse multivessel coronary vasospasms. Brugada type pattern ECGs (adjusted hazard ratio [HR], 4.22; 95% confidence interval [CI], 1.16 to 15.99; p = 0.034), and early repolarization (HR, 2.97; 95% CI, 1.09 to 8.10; p = 0.034) were ultimately associated with an increased risk of mortality. CONCLUSIONS: Even though a number of aborted SCD survivors have coronary vasospasms, some also have IPAS, which has the potential to cause SCD. Therefore, meticulous evaluations and follow-ups for IPAS are required in those patients.
Arrhythmias, Cardiac* ; Arrhythmogenic Right Ventricular Dysplasia ; Coronary Vasospasm* ; Death, Sudden, Cardiac* ; Electrocardiography ; Follow-Up Studies ; Heart Arrest ; Humans ; Long QT Syndrome ; Masks* ; Mortality ; Out-of-Hospital Cardiac Arrest ; Survivors

Thrombotic thrombocytopenic purpura (TTP) is a blood coagulation disorder that damages numerous organs, including the kidney, heart and brain. Features indicative of TTP include thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, kidney failure and fever. Infections, toxins, pregnancy and, rarely, autoimmune diseases are all known to be associated with TTP. We encountered a rare case of rheumatoid arthritis accompanied by TTP following tympanoplasty. The patient's confusion, thrombocytopenia and renal failure all improved after plasmapheresis and high-dose glucocorticoid therapy, but she eventually expired due to sepsis. We report on this case herein and also review the relevant literature.
Anemia, Hemolytic ; Arthritis, Rheumatoid* ; Autoimmune Diseases ; Blood Coagulation Disorders ; Brain ; Fever ; Heart ; Kidney ; Neurologic Manifestations ; Plasmapheresis ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic* ; Renal Insufficiency ; Sepsis ; Thrombocytopenia ; Tympanoplasty

Hyperextension injury in the thoracic spine is uncommon with only a few cases documented in the literature. The mechanism of these injuries is hyperextension combined with axial or shearing force. These types of injuries are associated with a high risk of dural tears and paraplegia. A 91-year-old female presented with acute back pain from a hyperextension injury in thoracic spine with no neurological deficit. Lumbar magnetic resonance imaging showed a intervertebral disc rupture. On day 20 of hospitalization, the herniated intervertebral disc compressed the spinal cord with incomplete paraplegia. Hyperextension injuries involving the three columns are very unstable and we recommend surgical treatment as soon as possible, not only because of the initial trauma, but a ruptured disc herniation can damage the spinal cord.
Back Pain ; Female ; Hospitalization ; Humans ; Intervertebral Disc ; Magnetic Resonance Imaging ; Paraplegia ; Rupture ; Spinal Cord ; Spine ; Thoracic Vertebrae

Metabolic syndrome is an important long term complication in chronic asymptomatic HIV-infected subjects under highly active antiretroviral therapy (HAART), because it can contribute to morbidity and mortality via cardiovascular disease (CVD). Therefore, a predictive marker for early detection of metabolic syndrome may be necessary to prevent CVD in HIV-infected subjects. Retinol-binding protein-4 (RBP-4) has been shown to be associated with metabolic syndrome in various non-HIV-infected populations. We performed a cross-sectional study to evaluate whether serum RBP-4 levels are correlated with metabolic syndrome in HIV-infected subjects receiving HAART. In total, 98 HIV-infected Koreans who had been receiving HAART for at least 6 months were prospectively enrolled. Metabolic syndrome was diagnosed according to the Adult Treatment Panel III criteria, and serum RBP-4 concentrations were measured using human RBP-4 sandwich enzyme-linked immunosorbent assay. Serum RBP-4 levels were significantly higher in HIV-infected subjects receiving HAART with metabolic syndrome (n=33, 33.9+/-7.7 microg/mL) than in those without it (n=65, 29.9+/-7.2 microg/mL) (p=0.012). In multivariate linear regression analysis, the number of components of metabolic syndrome presented and waist circumference were independently, significantly correlated with RBP-4 (p=0.018 and 0.030, respectively). In conclusion, we revealed a strong correlation between RBP-4 and the number of components of metabolic syndrome in HIV-infected subjects receiving HAART.
Adult ; *Antiretroviral Therapy, Highly Active ; Enzyme-Linked Immunosorbent Assay ; Female ; HIV Infections/*blood/*drug therapy ; Humans ; Male ; Metabolic Syndrome X/*blood/*drug therapy ; Middle Aged ; Retinol-Binding Proteins, Plasma/*metabolism

BACKGROUND: Small proportions of all the elderly stroke patients receive recombinant tissue plasminogen activator (r-tPA) therapy, although old age is not a proven contraindication to intravenous thrombolytic therapy for acute ischemic stroke. The purpose of this study was to identify reasons for exclusion from r-tPA therapy and factors associated with the decision of r-tPA use in elderly patients with acute ischemic stroke. METHODS: From the acute stroke registries of 22 domestic university hospitals taking the r-tPA therapy from January 2007 to May 2010, we extracted data of all acute ischemic stroke patients who were aged 80 or over and arrived within onset 3 hours. For all patients, we assessed the eligibility of r-tPA therapy using National Institute of Neurological Disorders and Stroke (NINDS) r-tPA trial criteria. For eligible patients, we compared all clinical variables between patients who were treated with r-tPA and those who were not, and analyzed potential factors related to the decision of r-tPA use. RESULTS: A total of 494 patients were included in this study. 255 patients (51.6%) were excluded by NINDS r-tPA trial criteria and the major reasons for exclusion were minor neurological deficit (53.7%) and clinical improvement (17.3%). Among 239 patients who were eligible for r-tPA, 162 (32.8%) patients received r-tPA and 77 (15.6%) did not. Multivariable analysis showed that younger age, shorter time-delay from onset to admission, non-smoker, no history of prior stroke, good pre-stroke functional status and severe initial neurological deficit were independently associated with the decision of r-tPA use in the elderly stroke patients predictors for r-tPA treatment. CONCLUSION: In very elderly patients, mild neurological deficit on arrival and rapid clinical improvement in neurological symptoms were the main reasons for exclusion from thrombolytic therapy.
Aged ; Hospitals, University ; Humans ; National Institute of Neurological Disorders and Stroke ; Registries ; Stroke ; Thrombolytic Therapy ; Tissue Plasminogen Activator

Achromobacter xylosoxidans is an uncommon cause of peritonitis in patients on maintenance continuous ambulatory peritoneal dialysis (CAPD). CAPD peritonitis caused by Achromobacter xylosoxidans carries high mortality and recurrence rates because of its virulence and resistance to most antimicrobial agents. We experienced a case of CAPD peritonitis caused by Achromobacter xylosoxidans in a patient with hypertension, diabetes mellitus, and end stage renal disease. The patient was treated with intravenous imipenem/cilastatin, and the CAPD catheter was removed. However, the patient died by aspiration pneumonia on the 34th day of hospitalization.
Achromobacter ; Achromobacter denitrificans ; Anti-Infective Agents ; Catheters ; Diabetes Mellitus ; Hospitalization ; Humans ; Hypertension ; Kidney Failure, Chronic ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis ; Pneumonia, Aspiration ; Recurrence

OBJECTIVES: Autism spectrum disorder (ASD) is a neurodevelopmental disorder that is characterized by abnormalities of social functioning, communication and behavior. The association of the 7q21-34 region with ASD has been reported. The DLX6 gene, which is located at the 7q22 region, is one of the positional and functional candidate genes for ASD. We found that there is no association between DLX6 polymorphisms and ASD in the Korean male population. METHODS: We selected three single nucleotide polymorphisms (SNPs) that might be implicated in the change of the DLX6 gene expression. The genomic DNA was collected from the venous blood of 147 male controls and 179 male patients with ASD. The genotypes of the selected SNPs were determined using the Illumina GoldenGate assay, and the statistical analyses were performed using HapAnalyzer software and SAS Enterprise. RESULTS: We found no association of the three SNPs in the DLX6 gene with ASD in the Korean male population. CONCLUSION: Our study suggests that the three SNPs in the DLX6 gene are not associated with ASD, and we need to analyze the previously reported regions for their associations with ASD.
Autistic Disorder ; Child ; Autism Spectrum Disorder ; DNA ; Gene Expression ; Genotype ; Humans ; Male ; Phenothiazines ; Polymorphism, Single Nucleotide