Noncirrhotic hyperammonemia as a cause of acute confusion remains diagnostic challenge. Deficiency of ornithine transcarbamylase (OTC) is the urea cycle disorder, inborn errors caused by a defect of the enzymes in the urea cycle, leading to an accumulation of ammonia mainly in newborn. There were very few cases, in which OTC deficiency result in hyperammonemia in adulthood. Herein, we report a young adult woman of hyperammonemic encephalopathy with OTC deficiency, diagnosed by high blood ammonia, glutamine and low plasma levels of citrulline. Next generation sequencing showed the c.386+5G>A mutation of the OTC gene.

Background/Aims: The prognostic significance of 18F-fluorodeoxyglucose (FDG)-positron emission tomography-computed tomography (PET/CT) in peripheral T-cell lymphomas (PTCLs) are controversial. We explored the prognostic impact of sequential 18F-FDG PET/CT during frontline chemotherapy of patients with PTCLs. Methods: In total, 143 patients with newly diagnosed PTCLs were included. Sequential 18F-FDG PET/CTs were performed at the time of diagnosis, during chemotherapy, and at the end of chemotherapy. The baseline total metabolic tumor volume (TMTV) was calculated using the the standard uptake value with a threshold method of 2.5. Results: A baseline TMTV of 457.0 cm3 was used to categorize patients into high and low TMTV groups. Patients with a requirehigh TMTV had shorter progression-free survival (PFS) and overall survival (OS) than those with a low TMTV (PFS, 9.8 vs. 26.5 mo, p = 0.043; OS, 18.9 vs. 71.2 mo, p = 0.004). The interim 18F-FDG PET/CT response score was recorded as 1, 2–3, and 4–5 according to the Deauville criteria. The PFS and OS showed significant differences according to the interim 18F-FDG PET/CT response score (PFS, 120.7 vs. 34.1 vs. 5.1 mo, p < 0.001; OS, not reached vs. 61.1 mo vs. 12.1 mo, p < 0.001). Conclusions The interim PET/CT response based on visual assessment predicts disease progression and survival outcome in PTCLs. A high baseline TMTV is associated with a poor response to anthracycline-based chemotherapy in PTCLs. However, TMTV was not an independent predictor for PFS in the multivariate analysis.

Purpose: Cysteinyl leukotrienes (CysLTs) have been recognized as key mediators associated with type 2 inflammation in the airways of asthmatic patients. CysLTs are associated with airway constriction, eosinophil recruitment/activation, and airway remodeling. The study aimed to understand the role of CysLTs in adult asthmatics in a real-world clinical setting. Methods: One hundred five adult asthmatics who had maintained antiasthmatic medications were enrolled. Asthmatic subjects were classified into 2 groups according to urinary leukotriene E4 (uLTE4) levels, and their clinical parameters and inflammatory mediators, including forced expiratory volume in 1 second (FEV1) %, fractional exhaled nitric oxide (FeNO), blood eosinophil count, serum periostin (sPON), and urinary eosinophil derived neurotoxin (uEDN) were compared between the high-uLTE4 and low-uLTE4 groups. Results: The prevalence of chronic rhinosinusitis (CRS), severe asthma, and aspirin-exacerbated respiratory disease (AERD) were significantly higher in the high-uLTE4 group than in the low-uLTE4 group. The high-uLTE4 group had lower FEV1% and maximal midexpiratory flow %, but higher FeNO levels than the low-uLTE4 group. In addition, blood eosinophil count, sPON, and uEDN levels were significantly higher in the high-uLTE4 group than in the low-uLTE4 group. The presence of AERD and levels of FeNO, sPON, and uEDN were significantly associated with higher uLTE4 levels in asthmatics. Conclusion CysLTs are associated with type 2 inflammation in the airways of asthmatic patients, contributing to the development of AERD, CRS, and asthma severity. The stratification of clinical phenotypes according to the uLTE4 level could support optimizing anti-inflammatory therapy for better control of asthma.

Background/Aims: Administrative databases provide valuable information for large-cohort studies. This study aimed to evaluate the diagnostic accuracy of an administrative database for resected gastric adenomas. Methods: Data of patients who underwent endoscopic resection for benign gastric lesions were collected from three hospitals. Gastric adenoma cases were identified in the hospital database using International Classification of Diseases (ICD) 10-codes. The non-adenoma group included patients without gastric adenoma codes. The diagnostic accuracy for gastric adenoma was analyzed based on the pathological reports of the resected specimen. Results: Among 5,095 endoscopic resections with codes for benign gastric lesions, 3,909 patients were included in the analysis. Among them, 2,831 and 1,078 patients were allocated to the adenoma and non-adenoma groups, respectively. Regarding the overall diagnosis of gastric adenoma with ICD-10 codes, the sensitivity, specificity, positive predictive value, and negative predictive value were 98.7%, 88.5%, 95.2%, and 96.8%, respectively. There were no significant differences in these parameters between the tertiary and secondary centers. Conclusions Administrative codes of gastric adenoma, according to ICD-10 codes, showed good accuracy and can serve as a useful tool to study prognosis of these patients in real-world data studies in the future.

Background: The optimal level of glycosylated hemoglobin (HbA1c) to prevent adverse clinical outcomes is unknown in patients with chronic kidney disease (CKD) and type 2 diabetes mellitus (T2DM). Methods: We analyzed 707 patients with CKD G1-G5 without kidney replacement therapy and T2DM from the KoreaN Cohort Study for Outcome in Patients With Chronic Kidney Disease (KNOW-CKD), a nationwide prospective cohort study. The main predictor was time-varying HbA1c level at each visit. The primary outcome was a composite of development of major adverse cardiovascular events (MACEs) or all-cause mortality. Secondary outcomes included the individual endpoint of MACEs, all-cause mortality, and CKD progression. CKD progression was defined as a ≥50% decline in the estimated glomerular filtration rate from baseline or the onset of end-stage kidney disease. Results: During a median follow-up of 4.8 years, the primary outcome occurred in 129 (18.2%) patients. In time-varying Cox model, the adjusted hazard ratios (aHRs) for the primary outcome were 1.59 (95% confidence interval [CI], 1.01 to 2.49) and 1.99 (95% CI, 1.24 to 3.19) for HbA1c levels of 7.0%–7.9% and ≥8.0%, respectively, compared with <7.0%. Additional analysis of baseline HbA1c levels yielded a similar graded association. In secondary outcome analyses, the aHRs for the corresponding HbA1c categories were 2.17 (95% CI, 1.20 to 3.95) and 2.26 (95% CI, 1.17 to 4.37) for MACE, and 1.36 (95% CI, 0.68 to 2.72) and 2.08 (95% CI, 1.06 to 4.05) for all-cause mortality. However, the risk of CKD progression did not differ between the three groups. Conclusion This study showed that higher HbA1c levels were associated with an increased risk of MACE and mortality in patients with CKD and T2DM.

Background: It has become important to identify and manage risk factors for subclinical atrial fibrillation (AF) with an increase in its detection rate. Thus, this research aimed to investigate whether alcohol consumption contrib‑ utes to the development of subclinical AF. Methods: This prospective study enrolled 467 patients without AF from a multicenter pacemaker registry. The incidence of subclinical AF (episodes of atrial rate > 220 beats per minute without symptoms) was compared between alcohol-drinking and non-drinking groups. Results: During followup (median 18 months), the incidence and risk of long-duration atrial high-rate episodes (AHRE) ≥ 24 h were increased in the alcohol group compared to the non-alcohol group [5.47 vs. 2.10 per 100 personyears, adjusted hazard ratio (HR), 2.83; 95% confidence interval (CI), 1.14–7.04; P = 0.03]. After propensity score match‑ ing, the incidence and risk of long-duration AHRE were higher in the alcohol group (6.97 vs. 1.27 per 100 personyears, adjusted HR, 7.84; 95% CI, 1.21–50.93; P = 0.03). The mean burden of long-duration subclinical AF was higher in the alcohol group than in the non-alcohol group (0.18 vs. 1.61% during follow-up, P = 0.08). Conclusion Alcohol consumption was associated with an increased risk of subclinical AF. Long-duration AHRE inci‑ dence and AHRE burden were higher in alcohol drinkers than in non-drinkers.

Educational evaluation involves data collection and the analysis of various education-related factors to make decisions that improve educational quality. Systematic educational evaluation is essential for enhancing the quality of education. This study reports a case of student-conducted process evaluation of a medical school’s student support system and the procedure for devising improvement plans. Sixteen Inje University College of Medicine students participated in the Education Evaluation Committee (IUCM-EEC) to understand the educational improvement process as learners and actively achieve improvement. The Quality Improvement Committee of the Inje University College of Medicine (IUCM-QIC) decided to reform its student support system based on a previous educational evaluation in 2019. The evaluation of the student support system was conducted for 10 months in 2021 by the student subcommittee, under the guidance of the IUCM-EEC. The CIPP (context-input-process-product) evaluation model was used for a systematic evaluation. Accordingly, the subcommittee developed evaluation criteria and indicators, and analyzed relevant data collected from surveys and the previous literature. For further recommendations and revision ideas, the student subcommittee members interviewed faculty members from six other medical schools and also conducted a focus group interview with the dean and vice deans of IUCM. Finally, the student subcommittee submitted a report to the IUCM-QIC. Communication with various stakeholders is essential for a successful evaluation process. In this case, students, as key stakeholders in education, evaluated the student support system. Their active participation helped improve their understanding of the evaluation process.

Background: Denosumab (DMB) is a bone antiresorptive agent used to treat osteoporosis or metastatic cancer of the bones. However, denosumab-associated osteonecrosis of the jaw (DRONJ) has become a common complication in cancer patients. The prevalence of osteonecrosis of the jaw (ONJ) in cancer patients is estimated to be similar for both bisphosphonate-related cases (1.1 to 1.4%) and denosumab-related cases (0.8 to 2%), with the addition of adjunctive therapy with anti-angiogenic agents reportedly increasing its prevalence to 3%. (Spec Care Dentist 36(4):231–236, 2016). The aim of this study is to report on DRONJ in cancer patients treated with DMB (Xgeva ® , 120mg).Case presentation In this study, we identified four cases of ONJ among 74 patients receiving DMB therapy for meta‑ static cancer. Of the four patients, three had prostate cancer and one had breast cancer. Preceding tooth extraction within 2 months of the last DMB injection was found to be a risk factor for DRONJ. Pathological examination revealed that three patients had acute and chronic inflammation, including actinomycosis colonies. Among the four patients with DRONJ referred to us, three were successfully treated without complications and had no recurrence following surgical treatment, while one did not follow up. After healing, one patient experienced a recurrence at a different site.Sequestrectomy in conjunction with antibiotic therapy and cessation of DMB use proved to be effective in managing the condition, and the ONJ site healed after an average 5-month follow-up period. Conclusion Conservative surgery, along with antibiotic therapy and discontinuation of DMB, was found to be effec‑ tive in managing the condition. Additional studies are needed to investigate the contribution of steroids and antican‑ cer drugs to jaw bone necrosis, the prevalence of multicenter cases, and whether there is any drug interaction with DMB.

In this study, the Search Your Mind (S.Y.M., 心) project aimed to collect prospective digital phenotypic data centered on mood and anxiety symptoms across psychiatric disorders through a smartphone application (app) platform while using both centralized and decentralized research designs: the centralized research design is a hybrid of a general prospective observational study and a digital platform-based study, and it includes face-to-face research such as informed written consent, clinical evaluation, and blood sampling. It also includes digital phenotypic assessment through an application-based platform using wearable devices. Meanwhile, the decentralized research design is a non-face-to-face study in which anonymous participants agree to electronic informed consent forms on the app. It also exclusively uses an application-based platform to acquire individualized digital phenotypic data. We expect to collect clinical, biological, and digital phenotypic data centered on mood and anxiety symptoms, and we propose a possible model of centralized and decentralized research design.

Despite the coronavirus disease 2019 (COVID-19) vaccination roll-out, variant-related outbreaks have occurred repeatedly in Korea. Although public hospitals played a major role in COVID-19 patients’ care, difficulty incorporating evolving COVID-19 treatment guidelines called for a clinical pathway (CP). Eighteen public hospitals volunteered, and a professional review board was created. CPs were formulated containing inclusion/exclusion criteria, application flow charts, and standardized order sets. After CP roll-out, key parameters improved, such as increased patient/staff five-point satisfaction scores (0.41/0.57) and decreased hospital stays (1.78 days)/medical expenses (17.5%). The CPs were updated consistently after roll-out as new therapeutics drugs were introduced and quarantine policies changed.