Purpose: Studies on intestinal Behçet’s disease (BD) complicated by myelodysplastic syndrome (MDS) are rare, and no established therapeutic guidelines exist. This study aimed to evaluate the clinical presentation and outcomes of patients with intestinal BD complicated by MDS (intestinal BD–MDS) and suggest a treatment strategy. Materials and Methods: Data from patients with intestinal BD–MDS from four referral centers in Korea who were diagnosed between December 2000 and December 2022 were retrospectively analyzed. Clinical features and prognosis of intestinal BD–MDS compared with age-, sex-matched intestinal BD without MDS were investigated. Results: Thirty-five patients with intestinal BD–MDS were included, and 24 (70.6%) had trisomy 8. Among the 35 patients, 23 (65.7%) were female, and the median age at diagnosis for intestinal BD was 46.0 years (range, 37.0–56.0 years). Medical treatments only benefited eight of the 32 patients, and half of the patients underwent surgery due to complications. Compared to 70 matched patients with intestinal BD alone, patients with intestinal BD–MDS underwent surgery more frequently (51.4% vs. 24.3%; p=0.010), showed a poorer response to medical and/or surgical treatment (75.0% vs. 11.4%; p<0.001), and had a higher mortality (28.6% vs. 0%; p<0.001). Seven out of 35 patients with intestinal BD–MDS underwent hematopoietic stem cell transplantation (HSCT), and four out of the seven patients had a poor response to medical treatment prior to HSCT, resulting in complete remission of both diseases. Conclusion Patients with intestinal BD–MDS frequently have refractory diseases with high mortalities. HSCT can be an effective treatment modality for medically refractory patients with intestinal BD–MDS.

Background/Aims: To date, there is no prospective study that specifically investigated the efficacy of infliximab in intestinal Behçet’s disease (BD). This study evaluated the efficacy of infliximab in patients with moderate-to-severe active intestinal BD that are refractory to conventional therapies. Methods: This phase 3, interventional, open-label, single-arm study evaluated clinical outcomes of infliximab treatment in patients with moderate-to-severe intestinal BD. The coprimary endpoints were clinical response, decrease in disease activity index for intestinal BD (DAIBD) score ≥20 from weeks 0 to 8 for the induction therapy and week 32 for the maintenance therapy. Results: A total of 33 patients entered the induction therapy and were treated with infliximab 5 mg/kg intravenously at weeks 0, 2, and 6. The mean DAIBD score changed from 90.8±40.1 at week 0 to 40.3±36.4 at week 8, with a significant mean change of 50.5±36.4 (95% confidence interval, 37.5 to 63.4; p<0.001). Thirty-one (93.9%) continued to receive 5 mg/kg infliximab every 8 weeks during the maintenance therapy. The mean change in the DAIBD score after the maintenance therapy was statistically significant (61.5±38.5; 95% confidence interval, 46.0 to 77.1; p<0.001, from weeks 0 to 32). The proportion of patients who maintained a clinical response was 92.3% at week 32. No severe adverse reactions occurred during the induction and maintenance therapies. Conclusions This study provided evidence that infliximab 5 mg/kg induction and maintenance therapies are efficacious and well-tolerated in patients with moderate-to-severe active intestinal BD. (ClinicalTrials.gov identifier: NCT02505568)

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.

BACKGROUND/AIMS: We aimed to evaluate the clinical characteristics and prognosis of Crohn's disease (CD) in patients who showed proximal small bowel involvement using a nationwide Korean CD cohort. METHODS: We reviewed the data from a cohort of patients diagnosed with CD. The clinical outcomes of patients were evaluated according to the presence of proximal small bowel involvement. RESULTS: Among 1,329 patients with CD for whom complete disease location data were available, 222 patients (16.7%) showed involvement of the proximal small bowel. Compared to patients without proximal small bowel involvement, those with small bowel involvement were more likely to display stricturing behavior (19.8% vs 12.7%, p=0.020). The surgery-free survival of patients who showed proximal small bowel involvement was inferior to that of patients without proximal small bowel involvement (10-year surgery-free survival: 58.4% vs 67.7%, respectively, p < 0.001). Additionally, upper gastrointestinal involvement was more common in patients with proximal small bowel involvement than in those without involvement (odds ratio, 1.643; 95% confidence interval, 1.008 to 2.677). CONCLUSIONS: Proximal small bowel involvement is a poor prognostic factor for the surgery-free survival of Korean patients with CD. Proximal small bowel involvement should be evaluated in patients with CD for predicting long-term clinical outcomes.
Cohort Studies ; Crohn Disease ; Humans ; Jejunum ; Prognosis

BACKGROUND/AIMS: The disease course and factors associated with poor prognosis in Korean patients with Crohn’s disease (CD) have not been fully determined. The aim of this study was to explore potential associations between the clinical characteristics and long-term outcomes of CD and perianal fistulas in a Korean population. METHODS: The retrospective Crohn’s Disease Clinical Network and Cohort (CONNECT) study enrolled patients diagnosed with CD between July 1982 and December 2008 from 32 hospitals. Those followed for < 12 months were excluded. Clinical outcomes were CD-related surgery and complications, including nonperianal fistulas, strictures, and intra-abdominal abscesses. RESULTS: The mean follow-up period was 8.77 years (range, 1.0 to 25.8 years). A total of 1,193 CD patients were enrolled, of whom 465 (39.0%) experienced perianal fistulas. Perianal fistulizing CD was significantly associated with younger age, male gender, CD diagnosed at primary care clinics, and ileocolonic involvement. Both nonperianal fistulas (p=0.034) and intra-abdominal abscesses (p=0.020) were significantly more common in CD patients with perianal fistulas than in those without perianal fistulas. The rates of complicated strictures and CD-related surgery were similar between the groups. Independently associated factors of nonperianal fistulas were perianal fistulas (p=0.015), female gender (p=0.048), CD diagnosed at referral hospital (p=0.003), and upper gastrointestinal (UGI) involvement (p=0.001). Furthermore, perianal fistulas (p=0.048) and UGI involvement (p=0.012) were independently associated with the risk of intra-abdominal abscesses. CONCLUSIONS: Perianal fistulas predicted the development of nonperianal fistulas and intra-abdominal abscesses in Korean CD patients. Therefore, patients with perianal fistulizing CD should be carefully monitored for complicated fistulas or abscesses.
Abdominal Abscess ; Abscess ; Cohort Studies ; Constriction, Pathologic ; Crohn Disease ; Female ; Fistula* ; Follow-Up Studies ; Humans ; Korea* ; Male ; Primary Health Care ; Prognosis* ; Rectal Fistula ; Referral and Consultation ; Retrospective Studies

“Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” is the 3rd edition of clinical practice guideline (CPG) for stroke rehabilitation in Korea, which updates the 2nd edition published in 2014. Forty-two specialists in stroke rehabilitation from 21 universities and 4 rehabilitation hospitals and 4 consultants participated in this update. The purpose of this CPG is to provide optimum practical guidelines for stroke rehabilitation teams to make a decision when they manage stroke patients and ultimately, to help stroke patients obtain maximal functional recovery and return to the society. The recent two CPGs from Canada (2015) and USA (2016) and articles that were published following the 2nd edition were used to develop this 3rd edition of CPG for stroke rehabilitation in Korea. The chosen articles' level of evidence and grade of recommendation were decided by the criteria of Scotland (2010) and the formal consensus was derived by the nominal group technique. The levels of evidence range from 1++ to 4 and the grades of recommendation range from A to D. Good Practice Point was recommended as best practice based on the clinical experience of the guideline developmental group. The draft of the developed CPG was reviewed by the experts group in the public hearings and then revised. “Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” consists of ‘Chapter 1; Introduction of Stroke Rehabilitation’, ‘Chapter 2; Rehabilitation for Stroke Syndrome, ‘Chapter 3; Rehabilitation for Returning to the Society’, and ‘Chapter 4; Advanced Technique for Stroke Rehabilitation’. “Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” will provide direction and standardization for acute, subacute and chronic stroke rehabilitation in Korea.
Canada ; Consensus ; Consultants ; Humans ; Korea* ; Practice Guidelines as Topic ; Rehabilitation* ; Scotland ; Specialization ; Stroke*

BACKGROUND/AIMS: C-reactive protein (CRP) is an easily measured index of disease activity, but its ability to predict clinical course is controversial. We therefore designed a study to determine whether the CRP level at Crohn's disease (CD) diagnosis is a valuable indicator of the disease phenotype, activity, and clinical course. METHODS: We retrospectively analyzed 705 CD patients from 32 institutions. The patients were classified into two groups according to CRP level. The patients' demographic and clinical characteristics and their use of immunosuppressive or biological agents were recorded. Disease location and behavior, hospitalization, and surgery were analyzed. RESULTS: A high CRP was associated with younger age, steroid use, colonic or ileocolonic location, high CD activity index, and active inflammation at colonoscopy (p<0.001). As the disease progressed, patients with high CRP were more likely to exhibit strictures (p=0.027). There were significant differences in the use of 5-aminosalicylic acid, antibiotics, corticosteroids, azathioprine, and infliximab (p<0.001, p<0.001, p<0.001, p<0.001, and p=0.023, respectively). Hospitalization was also more frequent in patients with high CRP. CONCLUSIONS: The CRP level at diagnosis is useful for evaluating the phenotype, activity, and clinical course of CD. Closer follow-up strategies, with early aggressive treatment, could be considered for patients with high CRP.
Adrenal Cortex Hormones ; Anti-Bacterial Agents ; Azathioprine ; Biological Factors ; C-Reactive Protein* ; Colon ; Colonoscopy ; Constriction, Pathologic ; Crohn Disease* ; Diagnosis* ; Follow-Up Studies ; Hospitalization ; Humans ; Inflammation ; Infliximab ; Mesalamine ; Phenotype* ; Retrospective Studies

We investigated trends in perioperative chemotherapy use, and determined factors associated with neoadjuvant chemotherapy (NAC) and adjuvant chemotherapy (AC) use in Korean patients with muscle-invasive bladder cancer (MIBC). We recruited 1,324 patients who had MIBC without nodal invasion or metastases and had undergone radical cystectomies (RC) between 2003 and 2013. The study's cut-off time for AC was three months after surgery, and the study's timespan was divided into three periods based on NAC use, namely, 2003-2005, 2006-2009, and 2010-2013. Complete remission was defined as histologically confirmed T0N0M0 after RC. NAC and AC were administered to 7.3% and 18.1% of the patients, respectively. The median time interval between completing NAC and undergoing RC was 32 days and the mean number of cycles was 3.2. The median time interval between RC and AC was 43 days and the mean number of cycles was 4.1. Gemcitabine and cisplatin were most frequently used in combination for NAC (49.0%) and AC (74.9%). NAC use increased significantly from 4.6% between 2003 and 2005 to 8.4% between 2010 and 2013 (P < 0.05), but AC use did not increase. Only 1.9% of patients received NAC and AC. Complete remission after NAC was achieved in 12 patients (12.5%). Multivariable modeling revealed that an advanced age, the earliest time period analyzed, and clinical tumor stage < or = cT2 bladder cancer were negatively associated with NAC use (P < 0.05). While NAC use has slowly increased over time, it remains an underutilized therapeutic approach in Korean clinical practice.
Adult ; Aged ; Aged, 80 and over ; Antineoplastic Agents/*therapeutic use ; Chemotherapy, Adjuvant/trends/utilization ; Cystectomy/trends/*utilization ; Drug Administration Routes ; Drug Administration Schedule ; Female ; Health Services Misuse/statistics & numerical data/trends ; Humans ; Male ; Middle Aged ; Muscle, Smooth/*pathology ; Neoadjuvant Therapy/statistics & numerical data/trends ; Neoplasm Invasiveness ; Practice Patterns, Physicians'/statistics & numerical data/trends ; Prevalence ; Republic of Korea/epidemiology ; Risk Factors ; Treatment Outcome ; Urinary Bladder Neoplasms/epidemiology/*pathology/*therapy

BACKGROUND/AIMS: Cytomegalovirus (CMV) reactivations are frequently observed in patients with active ulcerative colitis (UC), and ganciclovir therapy is effective in patients with steroid-refractory UC. This study aimed to determine the long-term outcomes of CMV reactivation and the long-term therapeutic efficacy of ganciclovir treatment. METHODS: This retrospective multicenter study included a cohort of 72 patients with moderate-to-severe UC who were evaluated for CMV reactivation at the time of their initial UC flare. Colectomy, disease relapse, and the recurrence rate of CMV reactivation were investigated. RESULTS: The mean duration of follow-up for the 72 patients was 43.16+/-19.78 months (range, 1 to 67 months). The cumulative colectomy (log-rank, p=0.025) and disease flare-up rates (log-rank, p=0.048) were significantly higher in the CMV-positive group. Of the 11 patients who were successfully treated with ganciclovir in the initial treatment, three patients (27.3%) experienced CMV reactivation, and six patients (54.5%) experienced poor outcomes, such as the need for colectomy or a steroid-dependent state. CONCLUSIONS: The patients who had CMV-reactivated UC showed poor outcomes at the long-term follow-up, and the long-term efficacy of ganciclovir therapy was marginal. Careful assessment is necessary for patients who exhibit evidence of CMV reactivation.
Antiviral Agents/*therapeutic use ; Case-Control Studies ; Cohort Studies ; Colectomy/utilization ; Colitis, Ulcerative/complications/*drug therapy ; *Cytomegalovirus ; Cytomegalovirus Infections/complications/*drug therapy ; Ganciclovir/*therapeutic use ; Humans ; Longitudinal Studies ; Remission Induction ; Retrospective Studies ; Severity of Illness Index ; Treatment Outcome ; *Virus Activation

PURPOSE: Continuous renal replacement therapy (CRRT) has become an essential modality for the care of critically ill pediatric patients who require renal support. However, experience with CRRT in the neonatal population is not common in Korea. In this study, we aimed to investigate the clinical features, outcomes, and complications of CRRT in neonates in a single neonatal intensive care unit (NICU). METHODS: We reviewed the medical records of 17 neonates who underwent CRRT at a NICU of a tertiary hospital. The data included demographic characteristics, diagnosis, complications, and laboratory and CRRT parameters. RESULTS: The median age at initiation of CRRT was 6 days after birth. All patients were treated with CRRT in continuous venovenous hemodiafiltration mode, with a median treatment duration of 57 hours. The main indication for CRRT was an inborn error of metabolism (IEM), followed by congenital renal disease and multiorgan failure. In patients with an IEM, the median plasma ammonia level at the CRRT initiation was 1,232 micromol/L, and the mean duration until the ammonia level decreased to half of the peak ammonia level was 7.3+/-2.5 hours. The overall hospital mortality rate was 41.2%. The outcomes of the 10 survivors after discharge included death (n=2), loss to follow-up (n=3), and survival with developmental delay (n=4). CONCLUSION: Although CRRT was effective in lowering the plasma ammonia level of neonates with IEM, the associated mortality and morbidity were high. Hence, further studies are needed to optimize the CRRT protocol and to establish an effective patient referral system in Korea.
Ammonia ; Critical Illness ; Diagnosis ; Follow-Up Studies ; Hemodiafiltration ; Hospital Mortality ; Humans ; Hyperammonemia ; Infant, Newborn ; Intensive Care, Neonatal* ; Korea ; Medical Records ; Metabolism ; Metabolism, Inborn Errors ; Mortality ; Parturition ; Plasma ; Referral and Consultation ; Renal Replacement Therapy* ; Survivors ; Tertiary Care Centers