What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Objective: To explore disease characteristics of primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) and compare the differences between PSC with and without IBD. Methods: Study design was cross sectional. Forty-two patients with PSC who were admitted from January 2000 to January 2021 were included. We analyzed their demographic characteristics, clinical manifestations, concomitant diseases, auxiliary examination, and treatment. Results: The 42 patients were 11-74(43±18) years of age at diagnosis. The concordance rate of PSC with IBD was 33.3%, and the age at PSC with IBD diagnosis was 12-63(42±17) years. PSC patients with IBD had higher incidences of diarrhea and lower incidences of jaundice and fatigue than in those without IBD (all P<0.05). Alanine aminotransferase, total bilirubin, direct bilirubin, total bile acid and carbohydrate antigen 19-9 levels were higher in PSC patients without IBD than in those with IBD (all P<0.05). The positive rates for antinuclear antibodies and fecal occult blood were higher in PSC patients with IBD than in those without IBD (all P<0.05). Patients with PSC complicated with ulcerative colitis mainly experienced extensive colonic involvement. The proportion of 5-aminosalicylic acid and glucocorticoid application in PSC patients with IBD was significantly increased compared with that of PSC patients without IBD (P=0.025). Conclusions: The concordance rate of PSC with IBD is lower at Peking Union Medical College Hospital than in Western countries. Colonoscopy screening may benefit PSC patients with diarrhea or fecal occult blood-positive for early detection and diagnosis of IBD.
Humans ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Cholangitis, Sclerosing/therapy* ; Cross-Sectional Studies ; Inflammatory Bowel Diseases/diagnosis* ; Colitis, Ulcerative/complications* ; Diarrhea

OBJECTIVES: To investigate the nutritional status and its influencing factors in children with newly diagnosed inflammatory bowel disease (IBD). METHODS: A retrospective analysis was conducted on the clinical data of children who were diagnosed with IBD for the first time in Hunan Children's Hospital from January 2015 to December 2021. Diagnostic delay was defined as the time from the symptom onset to IBD diagnosis being in the upper quartile (P₇₆-P₁₀₀) of all IBD children in the study. Multivariate logistic regression analysis was used to explore the risk factors for emaciation and growth retardation. RESULTS: A total of 125 children with newly diagnosed IBD were included, with Crohn's disease being the main type (91.2%). The rates of emaciation and growth retardation were 42.4% (53 cases) and 7.2% (9 cases), respectively, and the rate of anemia was 77.6% (97 cases). Diagnostic delay was noted in 31 children (24.8%), with the time from the symptom onset to IBD diagnosis of 366 to 7 211 days. Multivariate logistic regression analysis showed that diagnostic delay was a risk factor for emaciation and growth retardation (OR=2.73 and OR=4.42, respectively; P<0.05) and that age was positively associated with emaciation (OR=1.30, P<0.05). CONCLUSIONS Children with newly diagnosed IBD have poor nutritional status, and the rates of anemia, emaciation, and growth retardation are high. Diagnostic delay is associated with malnutrition in children with IBD.
Humans ; Child ; Colitis, Ulcerative/diagnosis* ; Nutritional Status ; Retrospective Studies ; Emaciation/complications* ; Delayed Diagnosis ; Inflammatory Bowel Diseases/complications* ; Malnutrition/complications* ; Growth Disorders/complications*

Child ; Chronic Disease ; Humans ; Inflammatory Bowel Diseases/diagnosis* ; Prognosis

diagnosis according to the Paris classification by comparison with patients from the European multicenter 5-years recruitment of children with newly developed IBD (EUROKIDS registry).METHODS: Korean children and adolescents who had been newly diagnosed with CD at the age of < 18 years during 2013–2016 were included in this multicenter retrospective study. Disease phenotype at diagnosis was classified according to the Paris classification, and compared with the published data from the EUROKIDS study.RESULTS: A total of 255 patients were included. The median diagnosis age was 14.7 years (range, 0.8–17.9 years). No significant difference was observed in male-to-female ratio with EUROKIDS (1.9:1 vs. 1.45:1, p=0.062). The proportion of children aged < 10 years was significantly lower in Koreans (7.1% vs. 19.6%, p < 0.001). Colonic disease was less prominent (10.0% vs. 27.3%, p < 0.001), while upper GI involvement was more prominent in Korean children (59.3% vs. 46.2%, p < 0.001). The proportion with perianal fistulizing disease at diagnosis was significantly higher in Korean patients (44.8% vs. 8.2%, p < 0.001). A separate analysis of Korean patients revealed that perianal fistulizing disease at diagnosis was positively associated with male sex and body mass index z-score (odds ratio [OR]=2.12, 95% confidence interval [CI]=1.20–3.76, p=0.010; and OR=1.29, 95% CI=1.05–1.58, p=0.015, respectively).CONCLUSION: Approximately half of pediatric CD patients in Korea present with perianal fistulas and/or abscesses at diagnosis, which is a distinct feature of CD in Korean children and adolescents compared to their European counterparts. An underlying genetic difference between ethnicities may play a role in this expression of different phenotypes in pediatric CD.]]>
Abscess ; Adolescent ; Body Mass Index ; Child ; Classification ; Colonic Diseases ; Crohn Disease ; Diagnosis ; Europe ; Fistula ; Humans ; Inflammatory Bowel Diseases ; Korea ; Male ; Pediatrics ; Phenotype ; Retrospective Studies

Fecal calprotectin (FC) is a calcium- and zinc-binding protein of the S100 family, mainly expressed by neutrophils and released during inflammation. FC became an increasingly useful tool both for gastroenterologists and for general practitioners for distinguishing inflammatory bowel disease (IBD) from irritable bowel syndrome. Increasing evidences support the use of this biomarker for diagnosis, follow-up and evaluation of response to therapy of several pediatric gastrointestinal diseases, ranging from IBD to nonspecific colitis and necrotizing enterocolitis. This article summarizes the current literature on the use of FC in clinical practice.
Colitis ; Diagnosis ; Enterocolitis, Necrotizing ; Follow-Up Studies ; Gastrointestinal Diseases ; General Practitioners ; Humans ; Inflammation ; Inflammatory Bowel Diseases ; Irritable Bowel Syndrome ; Leukocyte L1 Antigen Complex ; Neutrophils

PURPOSE: There is increasing prevalence of psychiatric disorders among inflammatory bowel Disease (IBD) population. Further, presence of psychiatric disorders has been shown as an independent predictor of quality of life among patients with IBD. We intended to explore the prevalence of various psychiatric disorders among pediatric and young adult population with IBD as a population-based analysis. METHODS: We did a retrospective case control analysis using a deidentified cloud-based database including health care data across 26 health care networks comprising of more than 360 hospitals across USA. Data collected across different hospitals were classified and stored according to Systematized Nomenclature of Medicine-Clinical Terms. We preidentified 10 psychiatric disorders and the queried the database for the presence of at least one of the ten psychiatric disorders among IBD patients between 5 and 24 years of age and compared with controls. RESULTS: Total of 11,316,450 patients in the age group between 5 and 24 years and the number of patients with a diagnosis of IBD, Crohn's disease or ulcerative colitis were 58,020. The prevalence of psychiatric disorders was 21.6% among IBD mainly comprising of depression and anxiety disorder. Multiple logistic regression analysis showed, IBD is 5 times more likely associated with psychiatric disorders than controls, p<0.001). We showed a steady increasing trend in the incidence of psychiatric disorders among IBD patients (2% in 2006 to 15% in 2017). CONCLUSION: Largest population-based analysis demonstrated an increased prevalence of psychiatric disorders among IBD patients. Our study emphasizes the need for psychological and mental health services to be incorporated as a part of the routine IBD clinic.
Adjustment Disorders ; Anxiety ; Anxiety Disorders ; Case-Control Studies ; Colitis, Ulcerative ; Crohn Disease ; Delivery of Health Care ; Depression ; Diagnosis ; Humans ; Incidence ; Inflammatory Bowel Diseases ; Logistic Models ; Mental Health Services ; Prevalence ; Quality of Life ; Retrospective Studies ; Young Adult

Inflammatory bowel disease (IBD) is a chronic inflammatory disease of the gastrointestinal tract with an unknown etiology and pathogenesis. The incidence and prevalence of IBD are increasing rapidly in Korea. Approximately one-third of patients with IBD appear to develop extra-intestinal manifestations with the skin being one of the most commonly affected organs. They may precede, occur simultaneously, or follow the diagnosis of IBD. In addition, they may parallel with the luminal symptoms or independent from the disease activity of IBD. This review outlines the skin manifestations associated with IBD and discusses their management. Skin manifestations should be managed in close collaboration with a dermatologist.
Colitis, Ulcerative ; Cooperative Behavior ; Crohn Disease ; Diagnosis ; Gastrointestinal Tract ; Humans ; Incidence ; Inflammatory Bowel Diseases ; Korea ; Phenobarbital ; Prevalence ; Skin ; Skin Manifestations

Almost 50% of patients with inflammatory bowel disease (IBD) exhibit at least one extra-intestinal manifestation in their lifetime. Extra-intestinal manifestations of IBD are often associated with the intestinal disease activity, reducing the quality of life of the patient but rarely leading to fatal complications. Musculoskeletal involvement is the most frequent extra-intestinal manifestation of patients with IBD but this rarely occurs before IBD is diagnosed. They are manifested in various forms, such as arthropathy, fibromyalgia, and osteoporosis. Therefore, a multidisciplinary team approach including gastroenterologists and rheumatologists are necessary for optimal treatment. This review focuses on the diagnosis and treatment of musculoskeletal manifestations of IBD from the perspectives of rheumatologists who can assist gastroenterologists.
Diagnosis ; Fibromyalgia ; Humans ; Inflammatory Bowel Diseases ; Intestinal Diseases ; Joint Diseases ; Musculoskeletal Diseases ; Osteoporosis ; Quality of Life

The incidence of inflammatory bowel disease (IBD) is increasing rapidly and extra-intestinal manifestations in IBD are also increasing. The prevalence of renal and urinary involvement in IBD ranges from 4–23%. Nephrolithiasis is the most common urinary complication in IBD patients. Parenchymal renal disease is rare but has been well documented and presents most commonly as glomerulonephritis or tubulointerstitial nephritis. The overall morbidity of IBD-related renal manifestations is significant. Therefore, a high index of clinical suspicion and optimal monitoring of the renal function are needed for the early diagnosis and prevention of IBD-related renal manifestations and complications.
Early Diagnosis ; Glomerulonephritis ; Humans ; Incidence ; Inflammatory Bowel Diseases ; Kidney ; Nephritis, Interstitial ; Nephrolithiasis ; Prevalence