Background/Aims: The prospective Crohn’s Disease Clinical Network and Cohort Study is a nationwide multicenter cohort study of patients with Crohn’s disease (CD) in Korea, aiming to prospectively investigate the clinical features and long-term prognosis associated with CD. Methods: Patients diagnosed with CD between January 2009 and September 2019 were prospectively enrolled. They were divided into two cohorts according to the year of diagnosis: cohort 1 (diagnosed between 2009 and 2011) versus cohort 2 (between 2012 and 2019). Results: A total of 1,175 patients were included, and the median follow-up duration was 68 months (interquartile range, 39.0 to 91.0 months). The treatment-free durations for thiopurines (p<0.001) and anti-tumor necrosis factor agents (p=0.018) of cohort 2 were shorter than those of cohort 1. Among 887 patients with B1 behavior at diagnosis, 149 patients (16.8%) progressed to either B2 or B3 behavior during follow-up. Early use of thiopurine was associated with a reduced risk of behavioral progression (adjusted hazard ratio [aHR], 0.69; 95% confidence interval [CI], 0.50 to 0.90), and family history of inflammatory bowel disease was associated with an increased risk of behavioral progression (aHR, 2.29; 95% CI, 1.16 to 4.50). One hundred forty-one patients (12.0%) underwent intestinal resection, and the intestinal resection-free survival time was significantly longer in cohort 2 than in cohort 1 (p=0.003). The early use of thiopurines (aHR, 0.35;95% CI, 0.23 to 0.51) was independently associated with a reduced risk of intestinal resection. Conclusions The prognosis of CD in Korea appears to have improved over time, as evidenced by the decreasing intestinal resection rate. Early use of thiopurines was associated with an improved prognosis represented by a reduced risk of intestinal resection.

Objectives: Monkeypox outbreaks in nonendemic countries have been reported since early May 2022. The first case of monkeypox in the Republic of Korea was confirmed in a patient who traveled to Europe in June 2022, and an attempt was made to isolate and identify the monkeypox virus (MPXV) from the patient’s specimens. Methods: Clinical specimens from the patient were inoculated in Vero E6 cells. The isolated virus was identified as MPXV by the observation of cytopathic effects on Vero E6 cells, transmission electron microscopy, conventional polymerase chain reaction (PCR), and sequencing of PCR products. Results: Cytopathic effects were observed in Vero E6 cells that were inoculated with skin lesion swab eluates. After multiple passages from the primary culture, orthopoxvirus morphology was observed using transmission electron microscopy. In addition, both MPXV-specific (F3L and ATI) and orthopoxvirus-specific genes (A39R, B2R, and HA) were confirmed by conventional PCR and Sanger sequencing. Conclusion These results indicate the successful isolation and identification of MPXV from the first patient in the Republic of Korea. The isolated virus was named MPXV-ROK-P1-2022.

Since a novel beta-coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was first reported in December 2019, there has been a rapid global spread of the virus. Genomic surveillance was conducted on samples isolated from infected individuals to monitor the spread of genetic variants of SARS-CoV-2 in Korea. The Korea Disease Control and Prevention Agency performed whole genome sequencing of SARS-CoV-2 in Korea for 1 year (January 2020 to January 2021). A total of 2,488 SARSCoV-2 cases were sequenced (including 648 cases from abroad). Initially, the prevalent clades of SARSCoV-2 were the S and V clades, however, by March 2020, GH clade was the most dominant. Only international travelers were identified as having G or GR clades, and since the first variant 501Y.V1 was identified (from a traveler from the United Kingdom on December 22 nd , 2020), a total of 27 variants of 501Y.V1, 501Y.V2, and 484K.V2 have been classified (as of January 25 th , 2021). The results in this study indicated that quarantining of travelers entering Korea successfully prevented dissemination of the SARS-CoV-2 variants in Korea.

Objectives: The Korea Centers for Disease Control and Prevention has published “A Guideline for Unknown Disease Outbreaks (UDO).” The aim of this report was to introduce tabletop exercises (TTX) to prepare for UDO in the future. Methods: The UDO Laboratory Analyses Task Force in Korea Centers for Disease Control and Prevention in April 2018, assigned unknown diseases into 5 syndromes, designed an algorithm for diagnosis, and made a panel list for diagnosis by exclusion. Using the guidelines and laboratory analyses for UDO, TTX were introduced. Results: Since September 9th , 2018, the UDO Laboratory Analyses Task Force has been preparing TTX based on a scenario of an outbreak caused by a novel coronavirus. In December 2019, through TTX, individual missions, epidemiological investigations, sample treatments, diagnosis by exclusions, and next generation sequencing analysis were discussed, and a novel coronavirus was identified as the causal pathogen. Conclusion Guideline and laboratory analyses for UDO successfully applied in TTX. Conclusions drawn from TTX could be applied effectively in the analyses for the initial response to COVID-19, an ongoing epidemic of 2019 - 2020. Therefore, TTX should continuously be conducted for the response and preparation against UDO.

Background/Aims: Plug-assisted retrograde transvenous obliteration (PARTO) is widely used to manage gastric varices with a portosystemic shunt. It is not clear whether portal pressure and the incidence of complications increase after PARTO. The aim of this study was to determine the changes in portal pressure and the associated changes in liver func-tion, ascites, hepatic encephalopathy, and especially esopha-geal varix (EV) after PARTO. Methods: From March 2012 to February 2018, 54 patients who underwent PARTO were analyzed retrospectively. The parameters collected included liver function and episodes of cirrhotic complications before and at 1 and 6 months after PARTO. Results: The analysis of 54 patients showed improvement in liver function during the 6-month follow-up period (Model for End-Stage Liver Disease score: change from 11.46±4.35 to 10.33±2.96, p=0.021).Among these 54 patients, 25 patients were evaluated for their hepatic venous pressure gradient (HVPG) before and after PARTO (change from 12.52±3.83 to 14.68±5.03 mm Hg; p<0.001). Twenty-five patients with portal pressure mea-sured before and after PARTO were evaluated for risk factors affecting liver function improvement and EV deterioration. No factor associated with portal pressure was affected by liver function improvement. Post-PARTO portal pressure was a risk factor affecting EV deterioration (HVPG-post: odds ratio, 1.341; 95% confidence interval, 1.017 to 1.767; p=0.037). Conclusions The artificial blockade of the portosystemic shunt evidently leads to an increase in HVPG. Liver function was improved over the 6-month follow-up period. Portal pres-sure after PARTO was a significant risk factor for EV deterioration. Portal pressure measurement is helpful for predicting the patient’s clinical outcome.

BACKGROUND/AIMS: Presence of enhanced mural nodules, which can be visualized using computed tomography (CT), is one of high-risk stigmata in branch-duct intraductal papillary mucinous neoplasms (BD-IPMNs). Conversely, the absence of enhanced mural nodules on preoperative imaging does not exclude malignant risk. The present study aimed to investigate other morphological features as predictors of malignancy in “pure” BD-IPMNs without enhanced mural nodules on CT. METHODS: This retrospective study included 180 patients with surgically confirmed “pure” BD-IPMNs of the pancreas and no enhanced mural nodules on preoperative CT. The study was conducted at 15 tertiary referral centers throughout South Korea. Univariate and multivariate analyses were used to identify significant predictors of malignancy. RESULTS: BD-IPMNs with low-grade (n=84) or moderate-grade (n=76) dysplasia were classified as benign; those with high-grade dysplasia (n=8) or invasive carcinoma (n=12) were classified as malignant. The multivariate analysis revealed that cyst size ≥30 mm (odds ratio, 8.6; p=0.001) and main pancreatic duct diameter ≥5 mm (odds ratio, 4.1; p=0.01) were independent risk factors for malignancy in “pure” BD-IPMNs without enhanced mural nodules on CT. Endoscopic ultrasound detected enhanced mural nodules (6/82) that had been missed on CT, and two IPMNs with enhanced mural nodules were malignant. CONCLUSIONS: In patients with “pure” BD-IPMNs who have no enhanced mural nodules on CT, cyst size ≥30 mm and main pancreatic duct diameter ≥5 mm may be associated with malignancy.
Christianity ; Humans ; Korea ; Mucins* ; Multivariate Analysis ; Pancreas* ; Pancreatic Ducts ; Retrospective Studies ; Risk Factors ; Tertiary Care Centers ; Ultrasonography

OBJECTIVETo evaluate the efficacy of Bawu Decoction (, BWD, Palmul-tang in Korean) against benign prostatic hyperplasia (BPH).

METHODSTwenty-four male Wistar rats were divided into 4 groups, with 6 rats in each group. The 4 study groups included sham-operated group (CON), BPH model group, fifinasteride-treated group, and BWD-treated group. All the groups except CON group received a subcutaneous injection of 10 mg/kg of testosterone, while CON group received saline. Finasteride at a dose of 5 mg/kg was administered to the finasteride-treated group for a period of 4 weeks. BWD group received BWD at a dose of 200 mg/kg for 4 weeks. The prostatic weight, prostate weight to body weight ratio, relative prostate weight ratio, serum testosterone and dihydrotestosterone (DHT) level, and histological analysis of prostatic tissue were analyzed.

RESULTSCompared to BPH model group, BWD administration was associated with reductions in prostatic weight, prostate and relative prostate weight ratio weight to body weight ratio (P<0.05). The concentration of serum testosterone and DHT were higher in BPH group compared with CON group (P<0.05). Administration of finasteride and BWD suppressed the elevation of serum testosterone and DHT levels signifificantly (both P<0.05). In addition, BWD suppressed the growth of prostatic tissue (P<0.05).

CONCLUSIONBWD has suppressant effects on development of BPH through inhibition of serum testosterone and DHT.

Niemann-Pick disease, type C (NP-C), is caused by NPC1 or NPC2 gene mutations. Progressive neurological, psychiatric, and visceral symptoms are characteristic. Here, we present cases of a brother (Case 1) and sister (Case 2) in their mid-20s with gait disturbance and psychosis. For the Case 1, neurological examination revealed dystonia, ataxia, vertical supranuclear-gaze palsy (VSGP), and global cognitive impairment. Case 2 showed milder, but similar symptoms, with cortical atrophy. Abdominal computed tomography showed hepatosplenomegaly in both cases. NPC1 gene sequencing revealed compound heterozygote for exon 9 (c.1552C>T [R518W]) and exon 18 (c.2780C>T [A927V]). Filipin-staining tests were also positive. When a young patient with ataxia or dystonia shows VSGP, NP-C should be considered.
Abdomen/diagnostic imaging ; Asian Continental Ancestry Group/genetics ; Carrier Proteins/genetics ; DNA Mutational Analysis ; Exons ; Female ; Gait Disorders, Neurologic/etiology ; Humans ; Male ; Membrane Glycoproteins/genetics ; Niemann-Pick Disease, Type C/*diagnosis/genetics ; Psychotic Disorders/etiology ; Republic of Korea ; Siblings ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVES: Restless legs syndrome (RLS) is a common sleep disorder in adults with diabetes. This study investigated the frequency of RLS and clinical correlations in children and adolescents with type 1 diabetes. METHODS: This study included 55 consecutive patients (21 males, age 12.6 +/- 3.4 years) with type I diabetes that were regularly treated at the Department of Pediatric Endocrinology. RLS was diagnosed by intensive interviews which also included the Epworth Sleepiness Scale (ESS) and International RLS Rating Scale (IRLSRS). Patients also received neurological examinations and laboratory tests for diabetes, iron metabolism and renal function. RESULTS: Thirteen patients (23.6%, 6 males) were compatible for the diagnostic criteria of RLS. None of the RLS patients showed abnormal findings in neurological evaluations and 7 patients had familial history of RLS. Demographic and laboratory findings were not different between the patients with or without RLS. The RLS group showed significantly increased ESS and IRLSRS scores. CONCLUSION: RLS was prevalent in children and adolescents with type I diabetes. The association between RLS and diabetes-related laboratory findings requires further investigation.
Adolescent* ; Adult ; Child* ; Endocrinology ; Humans ; Iron ; Male ; Metabolism ; Neurologic Examination ; Restless Legs Syndrome*

Cardiovascular beriberi is caused by thiamine deficiency and usually presents as high cardiac output failure associated with predominantly right-sided heart failure and rapid recovery after treatment with thiamine. Because of its rarity in developed countries, the diagnosis can often be delayed and missed. We recently experienced a case of cardiovascular beriberi with pulmonary hypertension which successfully treated with thiamine infusion. A 50-year-old man with chronic heavy alcoholics was refered to our department for dyspnea with mental change. Echocardiography showed marked right ventricular (RV) dilatation and flattening of the interventricular septum with a D-shaped deformation of the left ventricle. Moderate tricuspid valve regurgitation was found and estimated RV systolic pressure was 52 mm Hg. Because of his confused mentality and history of chronic alcohol intake, neurological disorder due to thiamine deficiency was suspected and intravenous thiamine was administered and he continuously received a daily dose of 100 mg of thiamine. Follow up echocardiography showed marked reduction of RV dilatation and improvement of a D-shaped deformation of the left ventricle. He finally diagnosed as cardiovascular beriberi on the basis of dramatic response to intravenous thiamine. Thiamine deficiency can cause reversible pulmonary hypertension, and can still be encountered in the clinical setting. Thus high index of suspicion is critically needed for diagnosis.
Alcoholics ; Beriberi* ; Blood Pressure ; Cardiac Output, High ; Developed Countries ; Diagnosis ; Dilatation ; Dyspnea ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Heart Ventricles ; Humans ; Hypertension, Pulmonary* ; Middle Aged ; Nervous System Diseases ; Thiamine ; Thiamine Deficiency ; Tricuspid Valve Insufficiency