Crohn’s disease (CD) is a relapsing and progressive condition characterized by diarrhea, abdominal pain, weight loss, and hematochezia that results in serious complications such as perforations, fistulas, and abscesses. Various medications, interventions, and surgical treatments have been used to treat CD. The Korean guidelines for CD management were distributed in 2012 and revised in 2017 by the Inflammatory Bowel Disease (IBD) Research Group of the Korean Association for the Study of Intestinal Diseases. Substantial progress in mucosal immunologic research has elucidated the pathophysiology of IBD, leading to development of biological agents for treatment of CD. The first developed biologic agent, tumor necrosis factor-α agents, were shown to be efficacious in CD, heralding a new era in management of CD. Subsequently, vedolizumab, a monoclonal antibody against integrin α4β7, and ustekinumab, a human monoclonal antibody that inhibits the common p40 subunit of interleukin-12 and interleukin-23, were both approved for clinical use and are efficacious and safe for both induction and maintenance of remission in moderate-to-severe CD patients. Moreover, a recent study showed the non-inferiority of CT-P13, an infliximab biosimilar, compared with infliximab in CD patients. The third Korean guidelines for CD management provide updated information regarding treatment of moderate-to-severe CD patients with biologic agents.

Objective: To report our experience with management of fetuses with congenital high airway obstruction syndrome (CHAOS). Methods: We retrospectively reviewed the cases of fetuses who were prenatally diagnosed and postnatally confirmed with CHAOS between 2010 and 2019 at Asan Medical Center, Seoul, Korea. Results: Of 13 fetuses prenatally diagnosed with CHAOS, 7 were lost to follow-up and 6 were postnatally confirmed as having CHAOS. All fetuses, except one were delivered via cesarean section with an ex utero intrapartum treatment (EXIT) procedure. Two patients had coexisting congenital heart diseases requiring several cardiac surgeries following birth. Both of these patients demonstrated developmental delay; however, the remaining 4 had a normal development except for expressive language. Two infants died of respiratory complications, and the remaining 4 were alive at the end of the follow-up period. All 4 live patients underwent tracheostomy with planned reconstruction surgery. Three children are now able to phonate, and 1 can maintain a conservation. Conclusion The proper management of CHAOS using the EXIT procedure results in high survival and low hypoxemia-induced complication rates. Therefore, an accurate prenatal diagnosis is necessary for an appropriate perinatal management.

BACKGROUND/AIMS: In some cases, chronic diarrhea is unexplained, and small bowel disorders may be one of the causes. The aim of this study was to assess the diagnostic yield and clinical impact of video capsule endoscopy (VCE) in patients with chronic diarrhea. METHODS: We retrospectively analyzed records from October 2002 to August 2013 in the VCE nationwide database registry (n=2,964). Ninety-one patients from 15 medical centers (60 males and 31 females; mean age, 47±19 years) were evaluated for VCE as a result of chronic diarrhea. RESULTS: The duration of chronic diarrhea was 8.3±14.7 months. The positive diagnostic yield of VCE was 42.9% (39/91). However, 15.4% (14/91) exhibited an inconsistent result, and 41.8% (38/91) were negative. Abnormal findings consistent with chronic diarrhea included erosions/aphthous ulcers (19.8%), ulcers (17.6%), mucosal erythema (3.3%), edema (1.1%), and luminal narrowing (1.1%). The most common diagnoses were functional diarrhea associated with irritable bowel syndrome in 37 patients (40.7%) and Crohn’s disease in 18 patients (19.8%). After VCE examination, the diagnosis was changed in 34.1% of the patients (31/91). Hematochezia (odds ratio [OR], 8.802; 95% confidence interval [CI], 2.126 to 36.441) and hypoalbuminemia (OR, 4.811; 95% CI, 1.241 to 18.655) are predictive factors of a positive diagnostic yield. CONCLUSIONS: VCE had a favorable diagnostic yield and clinical impact on the management of patients with chronic diarrhea.
Capsule Endoscopy* ; Diagnosis ; Diarrhea* ; Edema ; Erythema ; Female ; Gastrointestinal Hemorrhage ; Humans ; Hypoalbuminemia ; Irritable Bowel Syndrome ; Male ; Phenobarbital ; Retrospective Studies ; Ulcer

BACKGROUND/AIMS: Gastric pathology and Helicobacter pylori (H. pylori) infection among Asian patients with Crohn's disease (CD) are still unclear. We evaluated gastric histologic features and frequency of H. pylori infection in Korean patients with CD. METHODS: Among 492 patients with CD receiving upper gastrointestinal (GI) endoscopic evaluation in 19 Korean hospitals, we evaluated the endoscopic findings and gastric histopathologic features of 47 patients for our study. Histopathologic classification was performed using gastric biopsy tissues, and H. pylori infection was determined using the rapid urease test and histology. RESULTS: There were 36 men (76.6%), and the median age of patients at the time of upper GI endoscopy was 23.8 years (range, 14.2-60.5). For CD phenotype, ileocolonic disease was observed in 38 patients (80.9%), and non-stricturing, non-penetrating disease in 31 patients (66.0%). Twenty-eight patients (59.6%) complained of upper GI symptoms. Erosive gastritis was the most common gross gastric feature (66.0%). Histopathologically, H. pylori-negative chronic active gastritis (38.3%) was the most frequent finding. H. pylori testing was positive in 11 patients (23.4%), and gastric noncaseating granulomata were detected in 4 patients (8.5%). Gastric noncaseating granuloma showed a statistically significant association with perianal abscess/fistula (P=0.0496). CONCLUSIONS: H. pylori-negative chronic active gastritis appears to be frequent among Korean patients with CD. The frequency of H. pylori infection was comparable with previous studies. An association with perianal complications suggests a prognostic value for gastric noncaseating granuloma in patients with CD.
Asian Continental Ancestry Group ; Biopsy ; Classification ; Crohn Disease* ; Endoscopy ; Gastritis ; Granuloma ; Helicobacter pylori ; Humans ; Korea* ; Male ; Pathology ; Phenotype ; Stomach ; Urease

Video capsule endoscopy (VCE) is an ingestible video camera that transmits high-quality images of the small intestinal mucosa. This makes the small intestine more readily accessible to physicians investigating the presence of small bowel disorders, such as Crohn's disease (CD). Although VCE is frequently performed in Korea, there are no evidence-based guidelines on the appropriate use of VCE in the diagnosis of CD. To provide accurate information and suggest correct testing approaches for small bowel diseases, the Korean Gut Image Study Group, part of the Korean Society of Gastrointestinal Endoscopy, developed guidelines on VCE. Teams were set up to develop guidelines on VCE. Four areas were selected: diagnosis of obscure gastrointestinal bleeding, small bowel preparation for VCE, diagnosis of CD, and diagnosis of small bowel tumors. Three key questions were selected regarding the role of VCE in CD. In preparing these guidelines, a systematic literature search, evaluation, selection, and meta-analysis were performed. After writing a draft of the guidelines, the opinions of various experts were solicited before producing the final document. These guidelines are expected to play a role in the diagnosis of CD. They will need to be updated as new data and evidence become available.
Capsule Endoscopy* ; Crohn Disease* ; Diagnosis ; Endoscopy, Gastrointestinal ; Hemorrhage ; Intestinal Mucosa ; Intestine, Small ; Korea ; Writing

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

Although a metastasis to the gastrointestinal tract (GI) is rare in patients with hepatocellular carcinoma (HCC), it can occur by hematogenous or lymphatic spread, or by direct invasion of a tumor. A 61-year old woman who had a progressing large primary liver cancer presented with upper gastrointestinal (UGI) bleeding. UGI endoscopy showed a large duodenal submucosal tumor-like mass with a central ulcer and adherent blood clots. Endoscopic biopsy and coagulation using argon plasma probe were performed. The microscopic examination revealed a HCC. We report this unusual case of HCC with direct invasion of the duodenum.
Argon ; Biopsy ; Carcinoma, Hepatocellular* ; Duodenum* ; Endoscopy ; Female ; Gastrointestinal Tract ; Hemorrhage* ; Humans ; Liver Neoplasms ; Middle Aged ; Neoplasm Metastasis ; Plasma ; Ulcer

Guillain-Barre syndrome (GBS) is clinically characterized by acute, progressive, symmetrical weakness and areflexia. However there have been several reports of patients with the acute motor axonal neuropathy type of GBS showing hyperreflexia. We experienced a patient with acutely developed ascending progressive weakness of low extremities up to face following enteritis and concomitantly developed hyperreflexia following initial treatment with IVIG. The spinal MRI scan revealed radiological features of acute diffuse myelitis. The treatment with steroid resulted in fairly good clinical and electrophysiological outcome.
Axons ; Enteritis ; Extremities ; Guillain-Barre Syndrome* ; Humans ; Immunoglobulins, Intravenous ; Magnetic Resonance Imaging ; Myelitis* ; Reflex, Abnormal

BACKGROUND: Propranolol is only known drug effective in preventing variceal bleeding by ameliorating portal hypertension. The optimal dose of propranolol is variable due to racial differences of cardiovascular receptor sensitivity. In this prospective study, we tried to evaluate the effect of propranolol on portal hypertension, required optimal dose and to find out factors that influence drug responses in Korean cirrhotic patients with portal hypertension. METHODS: This study included 25 patients with liver cirrhosis who had variceal bleeding episode. Propranolol was given orally at an initial dose of 20 mg twice daily. The dose was subsequently adjusted over a period of 2 days until the resting heart rate had been reduced by 25% or less than 55 beats per minute. The hemodynamic studies including measurements of heart rate (HR), mean blood pressure (MBP), hepatic venous pressure gradient (HVPG), portal venous flow (PVF) were evaluated both prior to and 3 months after commencing treatment. Patients who showed a reduction in HPVG of more than 20% of baseline or absolute value under 12 mmHg were defined as being responders. RESULTS: The mean required dose of propranolol to reach target heart rate was 165 mg (80~280 mg). Propranolol induced significant reduction in HVPG (-29.0+/-21.4%, p<0.01), PVF (-19.6+/-17.8%, p<0.01) and HR (-29.3+/-9.1%, p<0.01). Drug responders were 15 (60%) and non-responders were 10 (40%). There was no significant factor for drug responders in multivariate analysis. The main complication of propranolol was dizziness with incidence of 24%, but was not serious enough to stop the administration of the drug. CONCLUSION: Propranolol is effective in reducing portal pressure in Korean cirrhotic patient and considered as relatively safe, and might be useful in preventing variceal bleeding. To obtain effective improvement of portal hypertension, it is necessary to increase the dosage until the targeted heart rate is reached when the measurement of HVPG is not be available.
Blood Pressure ; Dizziness ; Esophageal and Gastric Varices ; Heart Rate ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Portal* ; Incidence ; Liver Cirrhosis ; Multivariate Analysis ; Portal Pressure* ; Propranolol* ; Prospective Studies ; Ultrasonography ; Venous Pressure

PURPOSE: The 8 pancreatic cancer cell lines (BxPC-3, Capan-2, CFPAC-1, HPAC, Capan-1, AsPC-1, MIA PaCa-2, and PANC-1) were investigated to identify the factors which would increase CA19-9 related to the Lewis antigen. CA19-9 in serum is a well-known tumor marker, and is frequently used for the clinical diagnosis of pancreatic cancer. The oligosaccharide on the CA19-9 epitope is a sialylated Lewis A blood group antigen. METHODS: beta3Gal-T was detected by reverse transcriptase polymerase chain reaction (RT-PCR). The phenotypes and genotypes of Lewis antigen were determined by flow cytometry analysis and restriction fragment length polymorphism (RFLP), respectively. The phenotypes of sLe(a) were assessed by flow cytometry analysis and the sLe(a) on supernatants was detected by sodium dodecyl sulfate - polyacrylamide gel electrophoresis (SDS-PAGE). CA19-9 and DUPAN-2 on supernatants were measured by enzyme immunoassay. RESULTS: CA19-9 productions were possible from all cell lines since they all had beta3Gal-T and there were no genotypical Lewis negative (le/le). The elevation of CA19-9 was noted on Capan-2 and CFPAC-1, which were phenotypically Lewis positive (Le(a+b+)), as expected. Interestingly, it was also elevated in BxPC-3 even though the line was known to be phenotypically Lewis negative (Le(a-b-)). Sialyl Le(a) appeared to play an important role in this phenomenon. Although CA 19-9 was not detected in the phenotypically Lewis negative pancreatic cell line without sialyl Le(a), the levels of DUPAN-2 were variable. CONCLUSION: It was revealed that an elevated CA19-9 was related with increased expression of Lewis gene, not merely the existence of the gene. Further investigations on the role of ST3Gal are warranted to explain the mechanisms of the variable levels of DUPAN-2 in Le(a-b-) cell lines.
Cell Line* ; Diagnosis ; Electrophoresis, Polyacrylamide Gel ; Flow Cytometry ; Genotype ; Immunoenzyme Techniques ; Pancreatic Neoplasms* ; Phenotype ; Polymorphism, Restriction Fragment Length ; Reverse Transcriptase Polymerase Chain Reaction ; Sodium Dodecyl Sulfate