Purpose: Stem cell transplantation (SCT) has historically played a major role in the long-term remission of mantle cell lymphoma (MCL), an incurable hematological malignancy. Using data from the Korean Society of Bone and Marrow Transplantation registry, we retrospectively analyzed the role of autologous (auto) and allogeneic (allo) SCT in longterm MCL survival. Methods: This study analyzed data from 188 patients (age ≥ 19 years at the time of transplantation) who underwent a transplant for MCL from 2011 to 2020. Progression-free survival (PFS) was defined as the time from transplantation to disease progression, relapse, or death from any cause. Overall survival (OS) was defined as the time from transplan‑ tation to death from any cause or the last follow-up. Results: In total, 109 patients underwent consolidative SCT after first-line chemotherapy. The 3-year PFS and OS rates were 65.4% and 78.5%, respectively, in the auto-SCT group, and 66.7% and 71.4%, respectively, in the allo-SCT group. The PFS and OS did not differ significantly between the auto- and allo-SCT groups. As part of salvage treatment, 52 patients with relapsed or refractory disease underwent auto- or allo-SCT. Patients who underwent auto-SCT with complete remis‑ sion/partial remission status reported better outcomes. In patients with refractory status, allogeneic transplantation using human leukocyte antigen (HLA) fully matched donors was a significantly favorable factor for PFS and OS. Conclusion The long-term survival of patients who underwent consolidative transplantation was similar to that reported in previous studies. Auto-SCT may be beneficial in patients who respond to salvage therapy, whereas allo-SCT with HLA-matched donors may be an alternative for patients with refractory disease. 

Multiple myeloma (MM), a hematological malignancy, is characterized by malignant plasma cell proliferation in the bone marrow. Recent treatment advances have significantly improved patient outcomes associated with MM.In this study, we aimed to develop comprehensive, evidence-based guidelines for the diagnosis, prognosis, and treat‑ ment of MM. We identified 12 key clinical questions essential for MM management, guiding the extensive literature review and meta-analysis of the study. Our guidelines provide evidence-based recommendations by integrating patient preferences with survey data. These recommendations include current and emerging diagnostic tools, thera‑ peutic agents, and treatment strategies. By prioritizing a patient-centered approach and rigorous data analysis, these guidelines were developed to enhance MM management, both in Korea and globally.

Purpose: In general, bile is normally sterile. However, there are reports bactibilia may occur in certain instances, though the causal factors are unclear. We analyzed possible preoperative predictors of bactibilia upon cholecystectomy. Methods: Bile samples were collected during cholecystectomies from November 2018 to November 2019. A total of 428 open or laparoscopic cholecystectomies were performed. Preoperative, intraoperative, and postoperative variables were compared between the culture-positive and culture-negative groups. Results: One hundred fifty-seven patients (36.7%) were culture-positive. Gram-negative bacteria (95 [61.0%]) were more common. Escherichia coli (38 [40.0%]) and Enterobacter (22 [23.2%]) were the most common species. In univariate analysis, age of ≥70 years (P < 0.001), male sex (P < 0.001), high American Society of Anesthesiologists physical status grades (P = 0.001), diabetes mellitus (P = 0.002), jaundice (P = 0.007), high Tokyo Guideline grades (P = 0.008), percutaneous transhepatic gallbladder drainage (PTGBD; P < 0.001), endoscopic retrograde cholangiopancreatography (ERCP; P < 0.001) were identified as a risk factors for bactibilia. In multivariate analysis, age of ≥70 years (hazard ratio [HR], 2.874; 95% confidence interval [CI], 1.769–4.670; P = 0.001), ERCP (HR, 9.001; 95% CI, 4.833–16.75; P < 0.001), and PTGBD (HR, 2.866; 95% CI, 1.440–4.901; P = 0.002) were independent risk factors for bactibilia. Conclusion Among patients who underwent cholecystectomy, those who were elderly, symptomatic, and underwent preoperative drainage were more likely to have bactibilia. In such cases, surgeons should take care to prevent bile leakage during surgery and consider administering appropriate antibiotics.

Objective: The Functioning Assessment Short Test (FAST) is a relatively specific test for bipolar disorders designed to assess the main functioning problems experienced by patients. This brief instrument includes 24 items assessing impairment or disability in 6 domains of functioning: autonomy, occupational functioning, cognitive functioning, financial issues, interpersonal relationships, and leisure time. It has already been translated into standardized versions in several languages. The aim of this study is to measure the validity and reliability of the Korean version of FAST (K-FAST). Methods: A total of 209 bipolar disorder patients were recruited from 14 centers in Korea. K-FAST, Young Mania Rating Scale (YMRS), Bipolar Depression Rating Scale (BDRS), Global Assessment of Functioning (GAF) and the World Health Organization Quality of Life Assessment Instrument Brief Form (WHOQOL-BREF) were administered, and psychometric analysis of the K-FAST was conducted. Results: The internal consistency (Cronbach’s alpha) of the K-FAST was 0.95. Test-retest reliability analysis showed a strong correlation between the two measures assessed at a 1-week interval (ICC = 0.97; p ＜ 0.001). The K-FAST exhibited significant correlations with GAF (r = −0.771), WHOQOL-BREF (r = −0.326), YMRS (r = 0.509) and BDRS (r = 0.598). A strong negative correlation with GAF pointed to a reasonable degree of concurrent validity. Although the exploratory factor analysis showed four factors, the confirmatory factor analysis of questionnaires had a good fit for a six factors model (CFI = 0.925; TLI = 0.912; RMSEA = 0.078). Conclusion The K-FAST has good psychometric properties, good internal consistency, and can be applicable and acceptable to the Korean context.

Background/Aims: Several prediction models for evaluating the prognosis of nonmetastatic resected pancreatic ductal adenocarcinoma (PDAC) have been developed, and their performances were reported to be superior to that of the 8th edition of the American Joint Committee on Cancer (AJCC) staging system. We developed a prediction model to evaluate the prognosis of resected PDAC and externally validated it with data from a nationwide Korean database. Methods: Data from the Surveillance, Epidemiology and End Results (SEER) database were utilized for model development, and data from the Korea Tumor Registry System-Biliary Pancreas (KOTUS-BP) database were used for external validation. Potential candidate variables for model development were age, sex, histologic differentiation, tumor location, adjuvant chemotherapy, and the AJCC 8th staging system T and N stages. For external validation, the concordance index (C-index) and time-dependent area under the receiver operating characteristic curve (AUC) were evaluated. Results: Between 2004 and 2016, data from 9,624 patients were utilized for model development, and data from 3,282 patients were used for external validation. In the multivariate Cox proportional hazard model, age, sex, tumor location, T and N stages, histologic differentiation, and adjuvant chemotherapy were independent prognostic factors for resected PDAC. After an exhaustive search and 10-fold cross validation, the best model was finally developed, which included all prognostic variables. The C-index, 1-year, 2-year, 3-year, and 5-year time-dependent AUCs were 0.628, 0.650, 0.665, 0.675, and 0.686, respectively. Conclusions The survival prediction model for resected PDAC could provide quantitative survival probabilities with reliable performance. External validation studies with other nationwide databases are needed to evaluate the performance of this model.

BACKGROUND: Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare inherited disorder characterized by infantile-onset macrocephaly, slow neurologic deterioration, and seizures. Mutations in the causative gene, MLC1, are found in approximately 75% of patients and are inherited in an autosomal recessive manner. We analyzed MLC1 mutations in five unrelated Korean patients with MLC. METHODS: Direct Sanger sequencing was used to identify MLC1 mutations. A founder effect of the p.Ala275Asp variant was demonstrated by haplotype analysis using single-nucleotide polymorphic (SNP) markers. Multiple ligation-dependent probe amplification (MLPA) and comparative genomic hybridization plus SNP array were used to detect exonic deletions or uniparental disomy (UPD). RESULTS: The most prevalent pathogenic variant was c.824C>A (p.Ala275Asp) found in 7/10 (70%) alleles. Two pathogenic frameshift variants were found: c.135delC (p.Cys46Alafs*12) and c.337_353delinsG (p.Ile113Glyfs*4). Haplotype analysis suggested that the Korean patients with MLC harbored a founder mutation in p.Ala275Asp. The p.(Ile113Glyfs*4) was identified in a homozygous state, and a family study revealed that only the mother was heterozygous for this variant. Further analysis of MLPA and SNP arrays for this patient demonstrated loss of heterozygosity of chromosome 22 without any deletion, indicating UPD. The maternal origin of both chromosomes 22 was demonstrated by haplotype analysis. CONCLUSIONS: This study is the first to describe the mutational spectrum of Korean patients with MLC, demonstrating a founder effect of the p.Ala275Asp variant. This study also broadens our understanding of the mutational spectrum of MLC1 by demonstrating a homozygous p.(Ile113Glyfs*4) variant resulting from UPD of chromosome 22.
Alleles ; Chromosomes, Human, Pair 22* ; Comparative Genomic Hybridization ; Exons ; Founder Effect ; Haplotypes ; Humans ; Leukoencephalopathies* ; Loss of Heterozygosity ; Megalencephaly ; Mothers ; Seizures ; Uniparental Disomy*

PURPOSE: To investigate short term clinical outcomes of the open surgical repair for triangular fibrocartilage complex (TFCC) foveal detachment. METHODS: We retrospectively reviewed 8 patients (5 men, 3 women) who had been treated with open surgical repair of the TFCC type 1B injury, from 2005 to 2013 and who were followed up for more than one year after surgery. Mean age at time of surgery was 34 years. The right side was involved in 3 patients, and the left in 5. The clinical results of surgery were assessed with modified Mayo wrist score (MMWS), disabilities of the arm, shoulder and hand (DASH) score and pain-visual analogue scale (VAS). Physical examination was performed to evaluate the prescence of distal radioulnar instability, preoperatively and at the latest follow-up. RESULTS: The mean follow up period were 36.5 months (range, 12-64 months). The mean MMWS improved from 52.5 (range, 25-85) preoperatively to 82.5 (range, 75-100) postoperatively (p=0.02). The mean DASH score improved from 39.6 (range, 65-13.5) preoperatively to 13.4 (range, 2.5-33.3) postoperatively (p=0.012). The preoperative mean pain-VAS was 4.6 (range, 6-3); these value was reduced to mean 2 (range, 0-3) at the latest follow-up (p=0.016). There were no patients remaining instability after the surgery, although four patients showed distal radioulnar joint (DRUJ) instability before surgery. CONCLUSION: The surgical outcomes of open repair for TFCC foveal detachment (type 1B) was contentable. Also, in cases of type 1B injury associated with DRUJ instability were managed sucessfully without additional procedure.
Arm ; Follow-Up Studies ; Hand ; Humans ; Joints ; Male ; Physical Examination ; Retrospective Studies ; Shoulder ; Triangular Fibrocartilage* ; Wrist

BACKGROUND/AIMS: Narrow band imaging (NBI) endoscopy can be used for gross differentiation between the types of colonic polyps. This study was conducted as a retrospective study for estimation of the interobserver and intra-observer agreement of the pit pattern of the mucosal surface and the accuracy of histology prediction. METHODS: A total of 159 patients underwent complete colonoscopy and 219 polyps examined by NBI endoscopy without magnification were assessed. Interobserver and intra-observer agreement were calculated by investigators in each group for determination of the surface pattern and prediction of histology based on the modified Kudo's classification using intraclass correlation coefficient. RESULTS: Interobserver agreement for the surface pit pattern and prediction of polyp type was 0.84 and 0.73 in experienced endoscopists, and 0.86 and 0.62 in trainees, respectively. Intra-observer agreement for the surface pit patterns and prediction of polyp type was 0.81, 0.83, 0.85, 0.83, 0.56, 0.84, 0.51, 0.83, and 0.71; and 0.71, 0.70, 0.82, 0.54, 0.72, 0.37, 0.51, 0.34, and 0.30, respectively. The diagnostic accuracy for prediction of polyp type was 69.4% for experienced endoscopists and 72.9% for trainees. CONCLUSIONS: NBI endoscopy without magnification showed fairly good inter and intra-observer agreement for the pit pattern of the mucosal surface and the accuracy of histology prediction; however, it had some limitation for differentiation of colon polyp histologic type. Training and experience with NBI is needed for improvement of accuracy.
Adenoma/diagnosis ; Adult ; Aged ; Aged, 80 and over ; Carcinoma/diagnosis ; Colonic Neoplasms/*diagnosis/pathology ; Colonic Polyps/*pathology ; Colonoscopy ; Diagnosis, Differential ; Female ; Humans ; Male ; Middle Aged ; Narrow Band Imaging ; Retrospective Studies

PURPOSE: This study was performed to evaluate the compliance with, and adequacy of, the Korean national guidelines which had been recommended until 2011 for isolation of patients with group 1 nationally notifiable infectious diseases (NNIDs), namely cholera, typhoid fever, paratyphoid fever, shigellosis, and enterohemorrhagic Escherichia coli (EHEC) infection. MATERIALS AND METHODS: We evaluated the clinical and microbiological characteristics of confirmed cases of group 1 NNIDs and compliance with the guidelines in 20 Korean hospitals nationwide in 2000-2010. We also compared the Korean guidelines with international guidelines. RESULTS: Among 528 confirmed cases (8 cases of cholera, 232 of typhoid fever, 81 of paratyphoid fever, 175 of shigellosis, and 32 EHEC infections), strict compliance with the Korean guideline was achieved in only 2.6% to 50.0%, depending on the disease. While the Korean guidelines recommend isolation of all patients with group 1 NNIDs, international guidelines recommend selective patient isolation and screening for fecal shedding, depending on the type of disease and patient status. CONCLUSION: Compliance with the previous national guidelines for group 1 NNIDs in Korea was generally very low. Further studies are needed to evaluate whether compliance was improved after implementation of the new guideline in 2012.
Cholera ; Communicable Disease Control ; Communicable Diseases* ; Compliance* ; Dysentery, Bacillary ; Enterohemorrhagic Escherichia coli ; Guideline Adherence ; Humans ; Korea* ; Mass Screening ; Methods ; Paratyphoid Fever ; Patient Isolation ; Typhoid Fever

Esophageal carcinoid tumors remain some of the rarest of all carcinoid tumors, with only several cases previously reported in the literature. The endoscopic mucosal resection of selected carcinoid tumors has been shown to be a valid, safe, and effective method of treatment. Endoscopic ultrasonography is the technique of choice to select patients eligible for endoscopic resection. Here, we report successful endoscopic mucosal resection of a low esophageal carcinoid tumor and review the relevant literature. The present case is the first reported case of esophageal carcinoid tumor in Korea.
Carcinoid Tumor ; Endosonography ; Humans ; Korea