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.

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.

Sepsis is one of the significant causes of morbidity and mortality. The burden caused by sepsis has continued to increase in recent years in the Korea, highlighting the urgent need for the implementation of strategies to improve sepsis treatment outcomes. We therefore designed a web-based sepsis registry system (“Korean Registry for Improving Sepsis Survival” [KISS]) protocol to be used in hospitals in the Korea for evaluation of the epidemiology and clinical characteristics of patients with sepsis, via an analysis of outcome predictors. The inclusion criteria of this registry are as follows: adult patients ≥18 years admitted to the participating hospitals who are diagnosed with sepsis or septic shock. Demographic and clinical information data of the patients will be collected from hospital medical records and will be recorded in a case report form, which will be entered into a web-based data management system. The analysis of the collected data will be performed as follows: (1) epidemiological and clinical characteristics of sepsis and septic shock, (2) application of sepsis bundles and antibiotic stewardship, and (3) audit and feedback. In conclusion, we aim to build the comprehensive web-based sepsis registry in the Korea through a nation-wide network of participating hospitals. Information collected and analyzed through the KISS can be used for further improvements in the clinical management of sepsis. Furthermore, the KISS will facilitate research leading to the formulation of public health policies regarding sepsis bundle and antibiotic stewardship strategies in the Korea.

Kommerell diverticulum is a rare congenital anomaly of the aortic arch characterized by dilation at the proximal descending aorta, which gives rise to an aberrant subclavian artery. Kommerell diverticulum is usually asymptomatic, but can also be associated with symptoms due to compression of the esophagus or trachea, and can rarely be fatal due to dissection or rupture of the diverticulum. Here, we report a rare case of dysphagia caused by compression of the esophagus by Kommerell diverticulum originating from the right-sided aortic arch.

Demineralized dentin matrix (DDM) has been used as a recombinant human bone morphogenetic protein-2 (rhBMP-2) carrier in many clinical trials. To optimize the clinical safety and efficacy of rhBMP-2 with DDM, efforts have been made to improve the delivery of rhBMP-2 by 1) lowering the administered dose, 2) localizing the protein, and 3) prolonging its retention time at the action site as well as the bone forming capacity of the carrier itself. The release profile of rhBMP-2 that is associated with endogenous BMP in dentin has been postulated according to the type of incorporation, which is attributed to the loosened interfibrillar space and nanoporous dentinal tubule pores. Physically adsorbed and modified, physically entrapped rhBMP-2 is sequentially released from the DDM surface during the early stage of implantation. As DDM degradation progresses, the loosened interfibrillar space and enlarged dentinal tubules release the entrapped rhBMP-2. Finally, the endogenous BMP in dentin is released with osteoclastic dentin resorption. According to the postulated release profile, DDM can therefore be used in a controlled manner as a sequential delivery scaffold for rhBMP-2, thus sustaining the rhBMP-2 concentration for a prolonged period due to localization. In addition, we attempted to determine how to lower the rhBMP-2 concentration to 0.2 mg/mL, which is lower than the approved 1.5 mg/mL.
Bone Morphogenetic Proteins ; Collagen ; Dentin ; Humans ; Osteoclasts

Demineralized dentin matrix (DDM) has been used as a recombinant human bone morphogenetic protein-2 (rhBMP-2) carrier in many clinical trials. To optimize the clinical safety and efficacy of rhBMP-2 with DDM, efforts have been made to improve the delivery of rhBMP-2 by 1) lowering the administered dose, 2) localizing the protein, and 3) prolonging its retention time at the action site as well as the bone forming capacity of the carrier itself. The release profile of rhBMP-2 that is associated with endogenous BMP in dentin has been postulated according to the type of incorporation, which is attributed to the loosened interfibrillar space and nanoporous dentinal tubule pores. Physically adsorbed and modified, physically entrapped rhBMP-2 is sequentially released from the DDM surface during the early stage of implantation. As DDM degradation progresses, the loosened interfibrillar space and enlarged dentinal tubules release the entrapped rhBMP-2. Finally, the endogenous BMP in dentin is released with osteoclastic dentin resorption. According to the postulated release profile, DDM can therefore be used in a controlled manner as a sequential delivery scaffold for rhBMP-2, thus sustaining the rhBMP-2 concentration for a prolonged period due to localization. In addition, we attempted to determine how to lower the rhBMP-2 concentration to 0.2 mg/mL, which is lower than the approved 1.5 mg/mL.

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

BACKGROUND: The verbal fluency test consists of two separate tests of semantic fluency and phonemic fluency. The performance patterns of these tests differ with the type of dementia. We studied the patterns of verbal fluency between Alzheimer's disease (AD) and subcortical vascular dementia (SVaD), and assessed the clinical utility of these tests. METHODS: The 1,475 selected participants comprised 73 normal control subjects, 673 patients with amnestic mild cognitive impairment (aMCI), 535 AD patients, 42 patients with subcortical vascular mild cognitive impairment (svMCI), and 152 SVaD patients. We analyzed the z-score for the total number of animal items as a semantic fluency index and the z-score of the phonemic total score as a phonemic fluency index. RESULTS: The performance of semantic fluency was lower than that of phonemic fluency in all groups. The SVaD group showed the worst scores and abnormal performances on both tests, while the AD group presented abnormal performance only for semantic fluency. Dividing the patients with dementia according to severity revealed a different pattern between AD and SVaD for the clinical dementia rating (CDR) stage of 0.5. The performance of the AD group declined gradually with CDR. However, the SVaD group performed very poorly in both tests even for very mild dementia (CDR stage of 0.5). The aMCI and svMCI groups exhibited similar performance patterns. CONCLUSIONS: The pattern of semantic and phonemic fluency was not clinically useful at the MCI stage, but it could be helpful in differentiating AD and SVaD in the early stage of dementia.
Alzheimer Disease* ; Animals ; Dementia* ; Dementia, Vascular* ; Humans ; Mild Cognitive Impairment ; Semantics ; Task Performance and Analysis*

Desmopressin diacetate arginine vasopressin (DDAVP) is a synthetic analogue of the arginine vasopressin that is widely used in the treatment of diabetes insipidus, nocturnal enuresis, and polyuria. Although it is generally well-tolerated, DDAVP can cause hyponatremia, especially in elderly patients. There are many reports of DDAVP-induced hyponatremia, but there has been only one case report in which sinus node dysfunction was caused by severe hyponatremia. Here we report a case of sick sinus syndrome that occurred during an episode of severe hyponatremia induced by chronic use of desmopressin in a 91-year-old man who had nocturnal enuresis.
Aged ; Arginine Vasopressin ; Deamino Arginine Vasopressin* ; Diabetes Insipidus ; Humans ; Hyponatremia* ; Nocturnal Enuresis ; Polyuria ; Sick Sinus Syndrome*