Background: Factors related to the development and severity of polycystic liver disease (PLD) have not been well established. We aimed to evaluate the genetic and epidemiologic risk factors of PLD in patients with autosomal dominant polycystic kidney disease (ADPKD). Methods: Adult patients with inherited cystic kidney disease were enrolled from May 2019 to May 2021. Demographic, clinical, and laboratory data were collected at the initial study visit. The severity of PLD was graded based on the height-adjusted total liver volume: < 1,000 mL/m (Gr1), 1,000–1,800 mL/m (Gr2), and > 1,800 mL/m (Gr3). Targeted exome sequencing was done by a gene panel including 89 ciliopathy-related genes. We searched out the relative factors to the presence and the severity of PLD using logistic regression analysis. Results: Of 602 patients with typical ADPKD, 461 (76.6%) patients had PLD. The patients with PLD showed female predominance and a higher frequency of other ADPKD-related complications. The genetic variants with truncating mutation of PKD1 (PKD1-proteintruncating [PT]) or PKD2 commonly affected the development and severity of PLD. An older age, female sex, and higher kidney volume with Mayo classification 1C-1E was significantly associated with the development of PLD, but not with the severity of PLD. On the other hand, higher body mass index, lower hemoglobin, and higher alkaline phosphatase (ALP) were the significant risk factors of severe PLD (≥ Gr2). Conclusion Hepatic involvement in ADPKD could be related to kidney manifestations and genetic variants including PKD1-PT or PKD2. Monitoring hemoglobin and ALP and evaluating the genetic variants might help predict severe PLD.

Identifying genetic mutations in individuals with inherited cystic kidney disease is necessary for precise treatment. We aimed to elucidate the genetic characteristics of cystic kidney disease in the Korean population. Methods: We conducted a 3-year prospective, multicenter cohort study at eight hospitals from May 2019 to May 2022. Patients with more than three renal cysts were enrolled and classified into two categories, typical autosomal dominant polycystic kidney disease (ADPKD) and atypical PKD. We identified the clinical characteristics and performed a genetic analysis using a targeted gene panel. Results: A total of 725 adult patients were included in the study, of which 560 (77.2%) were diagnosed with typical ADPKD and 165 (22.8%) had atypical PKD. Among the typical ADPKD cases, the Mayo imaging classification was as follows: 1A (55, 9.9%), 1B (149, 26.6%), 1C (198, 35.8%), 1D (90, 16.3%), and 1E (61, 11.0%). The atypical PKD cases were classified as bilateral cystic with bilateral atrophic (31, 37.3%), lopsided (27, 32.5%), unilateral (nine, 10.8%), segmental (eight, 9.6%), bilateral cystic with unilateral atrophic (seven, 8.4%), and asymmetric (one, 1.2%). Pathogenic variants were found in 64.3% of the patients using the ciliopathy-related targeted gene panel. The typical ADPKD group demonstrated a higher discovery rate (62.3%) than the atypical PKD group (41.8%). Conclusion: We present a nationwide genetic cohort’s baseline clinical and genetic characteristics for Korean cystic kidney disease.

Previous studies have shown that tricuspid regurgitation (TR) can be developed in patients with atrial fibrillation (AF) due to annular dilatation. This study aimed to investigate the incidence and predictors of the progression of TR in patients with persistent AF. A total of 397 patients (66.9±11.4 years, 247 men; 62.2%) with persistent AF were enrolled between 2006 and 2016 in a tertiary hospital, and 287 eligible patients with follow-up echocardiography were analyzed. They were divided into two groups according to TR progression (progression group [n=68, 70.1±10.7 years, 48.5% men] vs. non-progression group [n=219, 66.0±11.3 years, 64.8% men]). Among 287 patients in the analysis, 68 had worsening TR severity (23.7%). Patients in the TR progression group were older and more likely to be female. Patients with left ventricular ejection fraction ＜50% were less frequent in the progression group than those in the non-progression group (7.4% vs. 19.6%, p=0.018). Patients with mitral valve disease were more frequent in the progression group. Multivariate analysis with COX regression demonstrated independent predictors of TR progression, including left atrial (LA) diameter ＞54 mm (HR 4.85, 95%CI 2.23-10.57, p＜0.001), E/e’ (HR 1.05, 95%CI 1.01-1.10, p=0.027), and no use of antiarrhythmic agents (HR 2.20, 95%CI 1.03-4.72, p=0.041). In patients with persistent AF, worsening TR was not uncommon. The independent predictors of TR progression turned out to be greater LA diameter, higher E/e’, and no use of antiarrhythmic agents.

Objective: This study aimed to investigate the perception of emergency medical service (EMS) providers and medical directors toward the field skill proficiency of EMS providers. We further examined differences in perception according to the certification and hospital career of individuals. Methods: This survey was conducted enrolling all active EMS providers in Busan, Ulsan, and Gyeongnam, as well as emergency physicians who participated in direct medical direction. Pre-developed questionnaires were sent as text messages to individual EMS providers and emergency physicians using an internet-based survey tool (Google Forms).Questionnaires were composed of 25 items in 7 categories: “airway management”, “ventilatory support”, “circulatory support”, “field assessment and management of trauma patients”, “field assessment and management of patients with chest pain”, “field assessment and management of patients with neurologic symptoms”, and “other items”. The response was based on a five-point Likert scale, where 0 score indicated no experience at all. Results: The questionnaire was distributed to 1,781 EMS providers and 52 medical directors; of these, 1,314 (73.7%) EMS providers and 34 (65.3%) medical directors completed the survey. EMS providers rated themselves as above average (3 points) for most of the questions. However, the majority responded that they had no experience or low proficiency in endotracheal intubation and prehospital delivery (median 2; interquartile range [IQR], 0-3). Conversely, medical directors assessed the EMS provider’s proficiency as above average in use of I-gel, recognition of hypoglycemia, field management of trauma patients, use of oropharyngeal and nasopharyngeal airway, use of laryngeal mask airway, and optimal oxygen supply (median, 4; IQR, 3-4), but responded with low scores for most other questions. Based on the EMS provider certification, nurses scored themselves more proficient than level-1 emergency medical technicians (EMTs) for intravenous access (P<0.001), whereas level-1 EMTs recognized themselves more proficient than nurses for endotracheal intubation (P<0.001), use of Magill forceps (P=0.004), and pediatric cardiopulmonary resuscitation (P<0.001). Conclusion This study recognized the discrepancies in the perception of EMS provider’s field skill proficiency, as perceived by EMS providers and medical directors, and between level-1 EMTs and nurses. We propose that regional EMS authorities need to make persistent efforts to narrow these perception gaps through effective educational programs for EMS providers and medical directors.

Background: Although the consumption of vitamin beverages has increased because of the recent interest in health and beauty, guidelines addressing appropriate consumption habits are lacking. Thus, the aim of this study was to investigate the erosive potential of several vitamin beverages and to propose guidelines for the appropriate intake of these drinks. Methods: Five vitamin beverages were selected after a pre-investigation of the current beverage market. Coca-Cola and mineral water were selected as the control beverages. The pH of the beverages was measured with a calibrated pH meter, and the titratable acidity (TA) was determined by using 1 M sodium hydroxide to reach pH 5.5 (TA5.5) and 7.0 (TA7.0). The screening method suggested by the International Organization for Standardization was used to measure pH variation (pH) by using an under-saturated hydroxyapatite solution to determine the difference between the initial and final pH of the screening solution. All measurements were performed in triplicate. Results: All vitamin beverages tested in this study exhibited a low pH (2.53∼2.99), similar to Coca-Cola, which is known to be a highly acidic beverage. The highest TA5.5 and TA7.0 values of the vitamin beverages were 7.03 ml and 8.81 ml, respectively. The largest change in pH determined by using the screening solution was found in Bacchus D (pH 1.44±0.05). The mean pH of the vitamin beverages was 1.12±0.29, which was higher than that of Coca-Cola (positive control, pH 0.58±0.05). Conclusion Vitamin beverages exhibited an erosive potential capable of damaging enamel surfaces. Therefore, the frequency of vitamin beverage intake should be limited, and individuals consuming these drinks should try to restore normal oral pH as quickly as possible.

Aged ; Caregivers ; Dementia ; Depression ; Empathy ; Epidemiologic Studies ; Humans ; Korea ; Pilot Projects

PURPOSE: The aim of this study was to evaluate the quality of the trauma care system of our hospital, in which emergency physicians care for major trauma patients in the emergency intensive care unit (ICU) in consultation with intervention radiologists and surgeons. METHODS: This was a retrospective observational study conducted in an emergency ICU of a tertiary referral hospital. We enrolled consecutive patients who had been admitted to our emergency ICU with major trauma from March 2007 to September 2010. We collected data with respect to demographic findings, mechanisms of injury, the trauma and injury severity score (TRISS), emergency surgery, angiographic intervention, and 6-month mortality. Then, we compared the observed and predicted survivals of the patients. The Hosmer-Lemeshow test and calibration plots by using 10 groups, one for each decile, of predicted mortality were used to evaluate the fitness of TRISS. P-values of greater than 0.05 represent a fair calibration. RESULTS: Among 116 patients, 12 (10.34%) were dead within 6 months after admission to the ICU, and 29 (25.00%) and 38 (32.80%) patients received emergency surgery and angiographic intervention, respectively. The mean injury severity score and revised trauma score were 36.97+/-17.73 and 7.84+/-6.75, respectively. The observed survival and the predicted survival of the TRISS were 89.66% (95% confidence interval [CI]: 84.03~95.28%) and 69.85% (95% CI: 63.80~75.91%), respectively. The calibration plots showed that the observed survival of our patients was consistently higher than the predicted survival of the TRISS (p<0.001). CONCLUSION: The observed survival for the trauma care system of our hospital, in which emergency physicians care for major trauma patients in the emergency ICU in consultation with intervention radiologists and surgeons, was higher than the predicted survival of the TRISS.
Calibration ; Critical Care ; Emergencies ; Emergency Medicine ; Humans ; Injury Severity Score ; Intensive Care Units ; Radiology, Interventional ; Retrospective Studies ; Tertiary Care Centers

PURPOSE: The purpose of this study was to examine the effects of a stress-management program on stress coping methods, interpersonal relations and quality of life in patients with chronic mental illness. METHODS: A nonequivalent control group pre-posttest design was used for this quasi-experimental study. The study was conducted from May 1 to December 30, 2010. The 41 participants in this study were selected from patients with chronic mental illness (20 for the experimental group and 21 for the control group). Datas were analyzed chi2-test, t-test, paired t-test, and one-way ANCOVA with the SPSS/WIN 15.0 program. RESULTS: There were significant changes in stress coping methods scores, interpersonal relations scores and quality of life scores in the experimental group before and after treatment, which were significantly different from those in the control group. CONCLUSION: The results of the study indicate that the stress-management program resulted in significant improvement in stress coping methods, interpersonal relations and quality of life for patients with chronic mental illness. Therefore, this study shows stress-management programs are useful in clinical practice as effective nursing interventions in patients with chronic mental illness.
Humans ; Interpersonal Relations* ; Nursing ; Quality of Life*

PURPOSE: To report a case of presumed intraocular natural killer/T-cell lymphoma (NKTL). CASE SUMMARY: A 61-year-old male presented with visual disturbance of the left eye for duration of 3 years. He had been treated with systemic chemotherapy and radiotherapy for nasal NKTL 1 year prior. Inflammatory reaction in the anterior chamber and vitreous opacity were observed in the left eye. The patient was diagnosed with uveitis in the left eye at a local clinic 3 years prior to visiting us. Because the patient did not respond to anti-inflammatory therapy, we performed diagnostic and therapeutic vitrectomy. Intraoperatively, vitreous opacity was thin sheet like in appearance. The vitreous specimen contained few lymphoid cells and was positive for Epstein Barr virus-encoded RNA (EBER). Systemic workups showed no metastasis to other organs. The patient was treated with systemic methotrexate chemotherapy and intravitreal methotrexate injected 3 times (once per week). During the 12-month follow-up period after the last intravitreal injection, the recurrence of lymphoma and related uveitis was not observed.
Anterior Chamber ; Eye ; Follow-Up Studies ; Humans ; Intravitreal Injections ; Lymphocytes ; Lymphoma ; Male ; Methotrexate ; Middle Aged ; Neoplasm Metastasis ; Recurrence ; RNA ; Uveitis ; Vitrectomy

BACKGROUND/AIMS: Helicobacter pylori (H. pylori) transmission route is not yet clearly understood. Isolating H. pylori from stool, saliva, and vomitus is very difficult. However, H. pylori could be cultured from feces in the setting of rapid gastrointestinal tract transit. The aim of this study was to isolate H. pylori by culture and PCR in the rectum and terminal ileum during colonoscopy. METHODS: Twenty subjects with positive UBT (urea breath test) were included. We performed polymerase chain reaction (PCR) test and culture of H. pylori with the rectal fluid and terminal ileal fluid during colonoscopy. RESULTS: H. pylori was cultured with rectal fluid from 9 (45.0%) of 20 subjects and with ileal fluid from 11 (55.0%) of 20 subjects. H. pylori was a little more frequently cultured from the terminal ileal fluid than the rectal fluid without statistical significance (p>0.05). PCR test detected flaA (16/20, 80.0% and 17/20, 85.0%), 16S rRNA gene (16/20, 80.0% and 17/20, 85.0%), cagA (10/20, 50.0% and 12/20, 60.0%), and ureC (9/20, 45% and 11/20, 54.5%) from the rectal fluid and the terminal ileal fluid, respectively. The specificity and sensitivity of ureC were 100%. CONCLUSIONS: H. pylori could be cultured from the rectal fluid and terminal ileal fluid in the setting of rapid gastrointestinal tract transit. These results suggest of fecal-oral transmission of H. pylori.
Adult ; Antigens, Bacterial/genetics ; Bacterial Proteins/genetics ; Breath Tests ; Electrolytes/administration & dosage ; Feces/microbiology ; Female ; Helicobacter Infections/*diagnosis/transmission ; Helicobacter pylori/genetics/*isolation & purification ; Humans ; Ileum/*microbiology ; Male ; Middle Aged ; Polyethylene Glycols/administration & dosage ; Polymerase Chain Reaction ; RNA, Ribosomal, 16S/genetics ; Rectum/*microbiology ; Sensitivity and Specificity ; Urea/analysis ; Urease/genetics