Clinical Characteristics and Long-term Outcomes of Pediatric Ulcerative Colitis: A Single-Center Experience in Korea

Background/Aims@#Although pediatric ulcerative colitis (UC) has a different phenotype and clinical course than adult UC, its clinical features and outcomes are poorly defined, especially in Asian populations. This study investigated the clinical features and long-term outcomes of pediatric UC in a Korean population. @*Methods@#We retrospectively analyzed 208 patients aged <18 years diagnosed with UC between 1987 and 2013. The patient characteristics at diagnosis according to the Paris classification and the clinical course were analyzed. @*Results@#The male-to-female ratio was 1.3:1, and the median patient age was 15.5 years. At diagnosis, 28.8% of patients had proctitis (E1), 27.8%, left-sided colitis (E2); 5.2%, extensive colitis (E3); and 38.2%, pancolitis (E4). The cumulative probabilities of extension after 5, 10, 15, and 20 years were 32.7%, 40.4%, 52.5%, and 65.8%, respectively. Eighteen patients underwent colectomy, and three patients had colorectal cancer. The cumulative probabilities of colectomy after 5, 10, 15, and 20 years were 7.1%, 8.9%, 12.6%, and 15.6%, and those of colorectal cancer after 10, 15, and 20 years were 0%, 2.1%, and 12.0%, respectively. The disease extent, Pediatric Ulcerative Colitis Activity Index severity, and systemic corticosteroid therapy were significant risk factors for colectomy. The development of primary sclerosing cholangitis was significantly associated with colorectal cancer. @*Conclusions@#This study provides detailed information on the disease phenotype and long-term clinical outcomes in a large cohort of Korean children with UC. They have extensive disease at diagnosis, a high rate of disease extension, and a low rate of cumulative colectomy.

Relation of Enteric α-Synuclein to Gastrointestinal Dysfunction in Patients With Parkinson's Disease and in Neurologically Intact Subjects

BACKGROUND/AIMS: α-Synucleinopathy in the brain is the neuropathological hallmark of Parkinson’s disease (PD). However, the functional impact of α-synucleinopathy in the enteric nervous system remains unknown. We aim to evaluate the association between gastrointestinal (GI) dysfunction and α-synuclein (αSYN) pathology in the stomach and colon of PD patients and controls, as well as to investigate the association between the αSYN pathology in GI tract and future PD risk. METHODS: A total of 35 PD patients and 52 neurologically intact subjects were enrolled in this study. Endoscopic biopsies were performed, and then immunohistochemical staining for αSYN was performed. All subjects completed the validated Rome III questionnaire for the assessment of GI symptoms. The association between GI symptoms and the αSYN pathology in GI mucosa was evaluated. Incident PD cases were assessed during a median follow-up of 46 months. RESULTS: The proportion of self-reported constipation and functional constipation through the Rome III questionnaire was significantly higher in PD patients than in controls (P 0.05). No incident PD cases were diagnosed during study period. CONCLUSIONS: Our present study suggests that the deposition of αSYN in the mucosal enteric nervous system may not be reflected by functional impairment of the affected segment of the gut.

Clinical applications of interferon-γ releasing assays for cytomegalovirus to differentiate cytomegalovirus disease from bystander activation: a pilot proof-of-concept study

BACKGROUND/AIMS: We evaluated the proposed clinical application of the combined interpretation of host factors and viral factors in two different cytomegalovirus (CMV) co-infection models. METHODS: We prospectively enrolled all human immunodeficiency virus non-infected patients with confirmed Pneumocystitis jirovecii pneumonia (PCP) and those with suspected gastrointestinal CMV disease in a tertiary hospital. All patients underwent CMV interferon-γ releasing assay (IGRA) for CMV (T-track CMV, Lophius Biosciences). We created the 2-axis model with the CMV IGRA results as the x-axis and the results for CMV virus replication as the y-axis, and hypothesized that cases falling in the left upper quadrant (high viral load and low CMV-specific immunity) of the model would be true CMV infections. The CMV IGRA results were concealed from the attending physicians. RESULTS: Of 39 patients with PCP, four (10%) were classified as combined CMV pneumonia, 13 (33%) as bystander activation, and the remaining 22 (56%) as no CMV infection. The data for all four patients with PCP and CMV pneumonia fell in the left upper quadrant of the 2-axis model. Of 24 patients with suspected gastrointestinal CMV disease, 12 (50%) were classified as gastrointestinal CMV disease and the remaining 12 (50%) as bystander activation with no gastrointestinal CMV disease. The data for 11 of the 12 patients (92%) with gastrointestinal CMV disease were located in the left upper quadrant of the 2-axis model. CONCLUSIONS: Cases yielding low CMV IGRA results and high CMV viral replication appear to be true CMV infections. Further studies with large number of cases in different types of CMV disease should be proposed.

Underestimation of Smoking Rates in an East Asian Population with Crohn's Disease

BACKGROUND/AIMS: The reported rates of current smoking at the time of Crohn’s disease (CD) diagnosis tend to be low in East Asian studies. However, we hypothesized that East Asian patients may be reluctant to disclose their smoking history, likely because of the influence of the Confucian culture. METHODS: We prospectively re-evaluated the smoking status at diagnosis in 1,437 Korean CD patients whose smoking status had been reported in our previous study. RESULTS: After re-evaluation, the current smokers at diagnosis increased from 388 patients (27.0%) to 445 patients (31.0%), indicating that 12.8% (57 of 445 patients) of the current smokers at diagnosis did not disclose their smoking status at their initial evaluation. The proportion of current smokers at diagnosis who had initially concealed their smoking status was significantly higher among the female patients (29.7%, 11/37) compared with the male patients (11.3%, 46/408) (p18 years old at diagnosis (8.6%, 35/406) (p<0.001). CONCLUSIONS: Subgroups of Korean CD patients, particularly young patients and female patients, are reluctant to disclose their smoking history. Therefore, the suggestion that smoking is not a risk factor for the development of CD in East Asians should be made with caution.

Change in the diagnosis of inflammatory bowel disease: a hospital-based cohort study from Korea

BACKGROUND/AIMS: Accurately diagnosing inflammatory bowel disease (IBD) remains a challenge, but is crucial for providing proper management for affected patients. The aim of the present study was to evaluate the frequency of change in diagnosis in Korean patients who were referred to our institution with a diagnosis of IBD. METHODS: We enrolled 1,444 patients diagnosed with ulcerative colitis (UC) and 1,452 diagnosed with Crohn's disease (CD), who had been referred to the Asan Medical Center between January 2010 and December 2014. These patients were assessed and subsequently classified as having UC, CD, indeterminate colitis, possible IBD, or non-IBD. RESULTS: During a median follow-up of 15.9 months, 400 of the 2,896 patients (13.8%) analyzed in this study experienced a change in diagnosis. A change in diagnosis from UC to CD, or vice-versa, was made in 24 of 1,444 patients (1.7%) and 23 of 1,452 patients (1.6%), respectively. A change to a non-IBD diagnosis was the most common modification; 7.5% (108 of 1444) and 12.7% (184 of 1452) of the patients with a referral diagnosis of UC and CD, respectively, were reclassified as having non-IBD. Among the 292 patients who were ultimately determined not to have IBD, 135 (55 UC and 80 CD cases) had received IBD-related medication. CONCLUSIONS: There are diagnostic uncertainties and difficulties in relation to IBD. Therefore, precise assessment and systematic follow-up are essential in the management of this condition.

Endoscopic and Clinical Factors Affecting the Prognosis of Colorectal Endoscopic Submucosal Dissection-Related Perforation

BACKGROUND/AIMS: Although colorectal endoscopic submucosal dissection (ESD)-related perforation is not uncommon, the factors affecting clinical outcomes after perforation have not been investigated. This study was designed to investigate the factors influencing the clinical course of ESD-related colon perforation. METHODS: Forty-three patients with colorectal ESD-related perforation were evaluated. The perforations were classified as endoscopic or radiologic perforations. The patients' medical records and endoscopic pictures were analyzed. RESULTS: The clinical outcomes were assessed by the duration of nil per os, intravenous antibiotics administration, and hospital stays, which were 2.7±1.5, 4.9±2.3, and 5.1±2.3 days, respectively. Multivariate analyses revealed that a larger tumor size, ESD failure, specific endoscopists, and abdominal pain were independently related to a poorer outcome. The time between perforation and clipping was 15.8±25.4 minutes in the endoscopic perforation group. The multivariate analysis of this group indicated that delayed clipping, specific endoscopists, and abdominal pain were independently associated with poorer outcomes. CONCLUSIONS: Tumor size, ESD failure, abdominal pain, and the endoscopist were factors that affected the clinical outcomes of patients with colorectal ESD-related perforation. The time between the perforation and clipping was an additional factor influencing the clinical course of endoscopic perforation. Decreasing this time period may improve outcomes.

The Clinical Usefulness of Endoscopic Ultrasound-Guided Fine Needle Aspiration and Biopsy for Rectal and Perirectal Lesions

BACKGROUND/AIMS: Endoscopic ultrasound-guided fine needle aspiration and/or biopsy (EUS-FNA/B) have been used to diagnose subepithelial tumors (SETs) and extraluminal lesions in the gastrointestinal tract. Our group previously reported the usefulness of EUS-FNA/B for rectal and perirectal lesions. This study reports our expanded experience with EUS-FNA/B for rectal and perirectal lesions in terms of diagnostic accuracy and safety. We also included our new experience with EUS-FNB using the recently introduced ProCore needle. METHODS: From April 2009 to March 2014, EUS-FNA/B for rectal and perirectal lesions was performed in 30 consecutive patients. We evaluated EUS-FNA/B performance by comparing histological diagnoses with final results. We also investigated factors affecting diagnostic accuracy. RESULTS: Among 10 patients with SETs, EUS-FNA/B specimen results revealed a gastrointestinal stromal tumor in 4 patients and malignant lymphoma in 1 patient. The diagnostic accuracy of EUS-FNA/B was 50% for SETs (5/10). Among 20 patients with non-SET lesions, 8 patients were diagnosed with malignant disease and 7 were diagnosed with benign disease based on both EUS-FNA/B and the final results. The diagnostic accuracy of EUS-FNA/B for non-SET lesions was 75% (15/20). The size of lesions was the only factor related to diagnostic accuracy (P=0.027). Two complications of mild fever and asymptomatic pneumoperitoneum occurred after EUS-FNA/B. CONCLUSIONS: The overall diagnostic accuracy of EUS-FNA/B for rectal and perirectal lesions was 67% (20/30). EUS-FNA/B is a clinically useful method for cytological and histological diagnoses of rectal and perirectal lesions.

Patient Descriptions of Rectal Effluents May Help to Predict the Quality of Bowel Preparation With Photographic Examples

BACKGROUND/AIMS: Previous studies have suggested a weak correlation between self-reported rectal effluent status and bowel preparation quality. We aim to evaluate whether photographic examples of rectal effluents could improve the correlation between patient descriptions of rectal effluents and bowel preparation quality. METHODS: Before colonoscopy, patients were asked to describe the nature of their last three rectal effluents. Photographic examples of rectal effluents were provided as a reference for scoring. Bowel preparation was subsequently assessed by a single endoscopist using a global preparation assessment scale. Preparation outcomes were grouped into two levels (excellent to good vs. fair to inadequate). Both univariate and multivariate logistic regression models were used to find any association between bowel preparation quality and patient characteristics. RESULTS: A total of 138 patients completed the questionnaires. The mean age was 56.5+/-10.4 years. The mean sum of the last three rectal effluent scores was 5.9+/-2.0. Higher rectal effluent scores (odds ratio [OR], 0.82; P=0.043) and the presence of diverticula (OR, 0.16; P<0.001) were risk factors for suboptimal preparation. CONCLUSIONS: Photographic example-guided patient descriptions of rectal effluents showed a statistically significant association with bowel preparation quality. However, clinical significance seemed to be low. The presence of diverticula was an independent predictive factor for suboptimal bowel preparation quality.

The Clinical Usefulness of Endoscopic Ultrasound-Guided Fine Needle Aspiration and Biopsy for Rectal and Perirectal Lesions

BACKGROUND/AIMS: Endoscopic ultrasound-guided fine needle aspiration and/or biopsy (EUS-FNA/B) have been used to diagnose subepithelial tumors (SETs) and extraluminal lesions in the gastrointestinal tract. Our group previously reported the usefulness of EUS-FNA/B for rectal and perirectal lesions. This study reports our expanded experience with EUS-FNA/B for rectal and perirectal lesions in terms of diagnostic accuracy and safety. We also included our new experience with EUS-FNB using the recently introduced ProCore needle. METHODS: From April 2009 to March 2014, EUS-FNA/B for rectal and perirectal lesions was performed in 30 consecutive patients. We evaluated EUS-FNA/B performance by comparing histological diagnoses with final results. We also investigated factors affecting diagnostic accuracy. RESULTS: Among 10 patients with SETs, EUS-FNA/B specimen results revealed a gastrointestinal stromal tumor in 4 patients and malignant lymphoma in 1 patient. The diagnostic accuracy of EUS-FNA/B was 50% for SETs (5/10). Among 20 patients with non-SET lesions, 8 patients were diagnosed with malignant disease and 7 were diagnosed with benign disease based on both EUS-FNA/B and the final results. The diagnostic accuracy of EUS-FNA/B for non-SET lesions was 75% (15/20). The size of lesions was the only factor related to diagnostic accuracy (P=0.027). Two complications of mild fever and asymptomatic pneumoperitoneum occurred after EUS-FNA/B. CONCLUSIONS: The overall diagnostic accuracy of EUS-FNA/B for rectal and perirectal lesions was 67% (20/30). EUS-FNA/B is a clinically useful method for cytological and histological diagnoses of rectal and perirectal lesions.

Patient Descriptions of Rectal Effluents May Help to Predict the Quality of Bowel Preparation With Photographic Examples

BACKGROUND/AIMS: Previous studies have suggested a weak correlation between self-reported rectal effluent status and bowel preparation quality. We aim to evaluate whether photographic examples of rectal effluents could improve the correlation between patient descriptions of rectal effluents and bowel preparation quality. METHODS: Before colonoscopy, patients were asked to describe the nature of their last three rectal effluents. Photographic examples of rectal effluents were provided as a reference for scoring. Bowel preparation was subsequently assessed by a single endoscopist using a global preparation assessment scale. Preparation outcomes were grouped into two levels (excellent to good vs. fair to inadequate). Both univariate and multivariate logistic regression models were used to find any association between bowel preparation quality and patient characteristics. RESULTS: A total of 138 patients completed the questionnaires. The mean age was 56.5+/-10.4 years. The mean sum of the last three rectal effluent scores was 5.9+/-2.0. Higher rectal effluent scores (odds ratio [OR], 0.82; P=0.043) and the presence of diverticula (OR, 0.16; P<0.001) were risk factors for suboptimal preparation. CONCLUSIONS: Photographic example-guided patient descriptions of rectal effluents showed a statistically significant association with bowel preparation quality. However, clinical significance seemed to be low. The presence of diverticula was an independent predictive factor for suboptimal bowel preparation quality.

Benign Colorectal Stricture: An Answer to the Balloon or Stent Question?

No abstract available.

Clinical Features and Prognosis of Resectable Primary Colorectal Signet-Ring Cell Carcinoma

BACKGROUND/AIMS: We attempted to investigate the prognosis of signet-ring cell carcinoma (SRC) patients who underwent curative surgery by comparing them with age-, sex-, and stage-matched non-mucinous adenocarcinoma (NMAC) patients. METHODS: Between January 2003 and December 2011, 19 patients with primary SRC of the colorectum underwent curative surgery. Four SRC patients under the age of 40 were excluded, and the clinicopathological data of 15 patients (7 men; median age, 56 years) were reviewed and compared with the data of 75 NMAC patients matched by age, sex, and pathologic stage. RESULTS: The median follow-up duration was 30.1 months for the SRC group and 43.7 months for the NMAC group (P=0.141). Involvement of the left side of the colon (73.3% vs. 26.7%, P=0.003) and infiltrative lesions such as Borrmann types 3 and 4 (85.7% vs. 24.0%, P=0.001) were more common in the SRC group than in the NMAC group. The five-year overall survival rate was significantly lower for patients with SRC than for those with NMAC (46.0% vs. 88.7%, hazard ratio, 6.99; 95% confidence interval, 2.33-20.95, P=0.001). CONCLUSIONS: Patients with even resectable primary colorectal SRC had a poorer prognosis than age-, sex-, and stage-matched colorectal NMAC patients.

Usefulness of C-Reactive Protein as a Disease Activity Marker in Crohn's Disease according to the Location of Disease

BACKGROUND/AIMS: C-reactive protein (CRP) is a serologic activity marker in Crohn's disease (CD), but it may be less useful in evaluating CD activity in ileal CD patients. We aimed to investigate the usefulness of CRP as a disease activity marker in CD according to disease location. METHODS: Korean CD patients in a single hospital were evaluated. Factors associated with elevated CRP concentration at the time of diagnosis of CD and the association between the physician's prediction regarding upcoming surgery and the sites of the lesions directly related to surgery were analyzed. RESULTS: Of 435 CD patients, 25.7%, 6.9%, and 67.4% had ileal, colonic, and ileocolonic CD, respectively. Multivariate analysis revealed that an elevated erythrocyte sedimentation rate, reduced serum albumin, CD activity index (CDAI) >220, and ileocolonic/colonic location were associated with an elevated CRP level and that the CRP level was significantly correlated with the CDAI in all CD patients (gamma=0.466, p<0.01). However, the correlation coefficient was dependent on the location, with values of 0.395, 0.456, and 0.527 in patients with an ileal, ileocolonic, and colonic disease location, respectively. Surgery for ileal lesions was less predictable than surgery for ileocolonic or colonic lesions during follow-up. CONCLUSIONS: CRP is less useful as a disease activity marker in patients with ileal CD than those with ileocolonic or colonic CD.

Natural Course of an Untreated Metastatic Perirectal Lymph Node After the Endoscopic Resection of a Rectal Neuroendocrine Tumor

Lymph node metastasis is rare in small (i.e., <10 mm) rectal neuroendocrine tumors (NETs). In addition to tumor size, pathological features such as the mitotic or Ki-67 proliferation index are associated with lymph node metastasis in rectal NETs. We recently treated a patient who underwent endoscopic treatment of a small, grade 1 rectal NET that recurred in the form of perirectal lymph node metastasis 7 years later. A 7-mm-sized perirectal lymph node was noted at the time of the initial endoscopic treatment. The same lymph node was found to be slightly enlarged on follow-up and finally confirmed as a metastatic NET. Therefore, the perirectal lymph node metastasis might have been present at the time of the initial diagnosis. However, the growth rate of the lymph node was extremely low, and it took 7 years to increase in size from 7 to 10 mm. NETs with low Ki-67 proliferation index and without mitotic activity may grow extremely slowly even if they are metastatic.

Bowel Preparation, the First Step for a Good Quality Colonoscopy

No abstract available.

A Case of Cronkhite-Canada Syndrome Showing Spontaneous Remission

Cronkhite-Canada syndrome (CCS) is a rare, noninherited gastrointestinal polyposis syndrome associated with ectodermal changes such as alopecia, nail dystrophy, and cutaneous hyperpigmentation. The etiology and pathogenesis of CCS are not known, but diarrhea, malnutrition, gastrointestinal bleeding, and infection may occur in the affected patient; moreover, this condition could be fatal. However, previous reports have described several cases of spontaneous remission. We report a 60-year-old man who was incidentally found to have colonic polyposis, alopecia, and hypogeusia and was diagnosed to have CCS. However, this patient experienced spontaneous remission, including regrowth of body hair and alleviation of bowel inflammation, without any specific medications such as steroids, antibiotics, or proton pump inhibitors.

Advances in Endoscopic Ultrasound-Guided Biliary Drainage: A Comprehensive Review

Endoscopic retrograde cholangiopancreatography (ERCP) has become the first-line therapy for bile duct drainage. In the hands of experienced endoscopists, conventional ERCP results in a failed cannulation rate of 3% to 5%. This failure can occur more commonly in the setting of altered anatomy or technically difficult cases due to either duodenal or biliary obstruction. In cases of ERCP failure, patients have traditionally been referred for either percutaneous transhepatic biliary drainage (PTBD) or surgery. However, both PTBD and surgery have higher than desirable complication rates. Within the last decade, endoscopic ultrasound-guided biliary drainage (EUS-BD) has become an attractive alternative to PTBD after failed ERCP. Many groups have reported on the feasibility, efficacy and safety of this technique. This article reviews the indications for ERCP and the currently practiced EUS-BD techniques, including EUS-guided rendezvous, EUS-guided choledochoduodenostomy and EUS-guided hepaticogastrostomy.

Electrical Stimulation Therapy in Chronic Functional Constipation: Five Years' Experience in Patients Refractory to Biofeedback Therapy and With Rectal Hyposensitivity

BACKGROUND/AIMS: Biofeedback therapy (BFT) can be unsuccessful in constipated patients, even those with pelvic floor dysfunction. Electrical stimulation therapy (EST) has been introduced as a novel therapeutic modality in patients with chronic constipation, especially those who have rectal hyposensitivity. We evaluated the efficacy of EST based on five years' clinical experience. METHODS: From January 2002 to February 2007, 159 patients underwent EST. After exclusion of 12 drop-outs, 147 (M:F = 61:86, 49 +/- 17 years) finished all treatment sessions. Among them, 88 (M:F = 29:59, 49 +/- 17 years) were refractory to BFT without rectal hyposensitivity (RH), and 59 (M:F = 32:27, 54 +/- 17 years) were those with RH. RESULTS: The overall response to EST was 59.2% (87/147) by per-protocol analysis. In the EST-responsive group, overall satisfaction improved significantly (from 7.3 +/- 3.0 to 4.3 +/- 2.5, P < 0.05). Subgroup analysis showed that the response rate was 64.8% (57/88) in patients refractory to BFT without RH, and 50.8% (30/59) in those with RH. CONCLUSIONS: EST may have additional therapeutic efficacy in patients who are refractory to BFT. EST may also be effective in patients with RH, including restoration of rectal sensation. Therefore, EST could be considered as an alternative choice in patients refractory to BFT and with or without RH.

A Case of Pulmonary Alveolar Proteinosis Improved with Inhaled Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) / 대한내과학회지

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Several studies have recently found that autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) play a major role in the pathogenesis of idiopathic PAP. Consequently, inhaled or systemic injection of GM-CSF has been suggested as a promising treatment for PAP. A 54-year-old male visited our hospital for progressive dyspnea. Four years earlier, he was diagnosed with PAP based on a surgical lung biopsy in another institution. Whole-lung lavage was performed four times before he visited our hospital. We administered high-dose inhaled GM-CSF therapy for 12 weeks followed by 12 weeks of low-dose therapy. After the GM-CSF treatment, the patient's symptoms, lung function, and radiological findings were improved significantly.

A Case of Severe Community-acquired Acinetobacter baumannii Pneumonia with Bacteremia

Acinetobacter baumannii is a significant pathogen in nosocomial infections, especially in intensive care units. However, community-acquired A. baumannii (CAAB) pneumonia is an uncommon disease. Most of the CAAB pneumonia in the literature is characterized by an abrupt onset and rapid progression to respiratory failure and hemodynamic instability. In our case, a 51-year-old man without underlying diseases developed severe pneumonia. Respiratory distress rapidly worsened and mechanical ventilation was applied. Extra-corporeal membrane oxygenation was applied due to refractory septic shock. Fully sensitive A. baumannii pneumonia was confirmed by the sputum culture and blood culture. The patient was effectively treated by the meropenem. However, the patient died of uncontrolled ventilator-associated pneumonia, developed on the 10th hospital day, and refractory septic shock. We report the case of severe CAAB pneumonia with bacteremia in a patient without underlying diseases in Korea.