SCLEROSING CHOLANGITIS IN CHILDREN AND ADOLESCENTS: CURRENT STATE OF THE PROBLEM

There are presented generalized data on modern methods of diagnosis and treatment of sclerosing cholangitis (SCh), a chronic inflammatory liver disease of unknown etiology affecting the intrahepatic and/or extrahepatic bile ducts resulting in cirrhosis of the liver. In children, 2 forms of SCh are distinguished: primary (PSCh) and autoimmune (ASCh). Diagnosis of SCh requires performing cholangiography. ACh responds to standard immunosuppressive treatment with prednisone/prednisone and azathioprine. Supportive immunosuppressive therapy should last at least 2-3 years. After the end of such treatment, the doctor should continue to monitor changes in the ratios of AST/ALT/IgG autoantibodies in the blood of patients every 3-month for at least 5 years. SCh is often associated with inflammatory bowel disease, which should be excluded in the debut by determining the content of fecal calprotectin and colonoscopy data. In children, liver transplantation is one of the options for the treatment of Ch. However, the frequency of relapses after transplantation remains high, especially in ACh patients.

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A Gentleman with Anemia and Cholestasis

Case Reports in Medicine ◽

10.1155/2010/536207 ◽

2010 ◽

Vol 2010 ◽

pp. 1-4

Author(s):

Siu-Tong Law ◽

Wai-Ki Lee ◽

Michael Kin-Kong Li ◽

Ka-Ho Lok

Keyword(s):

Inflammatory Bowel Disease ◽

Portal Hypertension ◽

Bile Duct ◽

Primary Sclerosing Cholangitis ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Bile Ducts ◽

Colonoscopic Surveillance ◽

Extrahepatic Bile Ducts ◽

Inflammatory Bowel

Primary sclerosing cholangitis is a rare cause of cholestasis caused by progressive inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts leading to multifocal ductal strictures. Herein, we report a case of primary sclerosing cholangitis and inflammatory bowel disease. The concomitant diagnosis of these two diseases is not typical. The management includes the treatment of inflammatory bowel disease and potential complications of primary sclerosing cholangitis, including dominant strictures of bile duct, portal hypertension, gallbladder diseases, cholangiocarcinoma, and colonoscopic surveillance.

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Biomarkers of Inflammatory Bowel Disease

Disease Markers ◽

10.1155/2014/710915 ◽

2014 ◽

Vol 2014 ◽

pp. 1-11 ◽

Cited By ~ 39

Author(s):

Yi Fengming ◽

Wu Jianbing

Keyword(s):

Inflammatory Bowel Disease ◽

Chronic Disease ◽

Bowel Disease ◽

Fecal Calprotectin ◽

Unknown Etiology ◽

Inflammatory Bowel

Inflammatory bowel disease (IBD) is a chronic disease mostly involved with intestine with unknown etiology. Diagnosis, evaluation of severity, and prognosis are still present as challenges for physicians. An ideal biomarker with the characters such as simple, easy to perform, noninvasive or microinvasive, cheap, rapid, and reproducible is helpful for patients and clinicians. Currently biomarkers applied in clinic include CRP, ESR, pANCA, ASCA, and fecal calprotectin. However, they are far from ideal. Lots of studies are focused on seeking for ideal biomarker for IBD. Herein, the paper reviewed recent researches on biomarkers of IBD to get advances of biomarkers in inflammatory bowel disease.

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Rapid Progression of Primary Sclerosing Cholangitis Complicated with Ulcerative Colitis

Case Reports in Gastrointestinal Medicine ◽

10.1155/2015/125718 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Piotr Pardak ◽

Ewa Walczak ◽

Rafał S. Filip

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Liver Transplant ◽

Primary Sclerosing Cholangitis ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Unknown Etiology ◽

Rapid Progression ◽

Inflammatory Bowel ◽

Progressive Course

Primary sclerosing cholangitis is a cholestatic condition with unknown etiology and long-standing, progressive course, leading to cirrhosis and requiring orthotropic liver transplant. In approximately 80%, primary sclerosing cholangitis is accompanied by inflammatory bowel disease, and in most cases the recognition of bowel disease precedes the diagnosis of primary sclerosing cholangitis. We describe a case of 22-year-old male diagnosed simultaneously with primary sclerosing cholangitis and ulcerative colitis, with a medical history suggesting uncommon prior development of the liver disease. Five months after the initial diagnosis, we observed advanced lesions of bile tree due to progression of primary sclerosing cholangitis, which led to the unusually fast necessity for the orthotopic liver transplant.

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P175 COLORECTAL CANCER RISK IN INFLAMMATORY BOWEL DISEASE FOLLOWING LIVER TRANSPLANTATION FOR PRIMARY SCLEROSING CHOLANGITIS

Gastroenterology ◽

10.1053/j.gastro.2017.11.227 ◽

2018 ◽

Vol 154 (1) ◽

pp. S93-S94

Author(s):

Derrick Eichele ◽

Benita McVicker ◽

Renee Young ◽

Marco Olivera

Keyword(s):

Colorectal Cancer ◽

Inflammatory Bowel Disease ◽

Liver Transplantation ◽

Cancer Risk ◽

Primary Sclerosing Cholangitis ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Colorectal Cancer Risk ◽

Inflammatory Bowel

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Impact of primary sclerosing cholangitis on the disease course in patients with inflammatory bowel disease – evidence from a large retrospective study with matched cohorts

10.26226/morressier.59a6b343d462b80290b54389 ◽

2017 ◽

Author(s):

Friederike Cordes

Keyword(s):

Inflammatory Bowel Disease ◽

Retrospective Study ◽

Primary Sclerosing Cholangitis ◽

Bowel Disease ◽

Sclerosing Cholangitis ◽

Disease Course ◽

Inflammatory Bowel ◽

Large Retrospective Study

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Home monitoring of disease activity and fecal calprotectin in adult patients with inflammatory bowel disease – interim analysis of 68 patients

10.26226/morressier.59a6b343d462b80290b5443a ◽

2017 ◽

Author(s):

Dorit Ankersen

Keyword(s):

Inflammatory Bowel Disease ◽

Disease Activity ◽

Bowel Disease ◽

Interim Analysis ◽

Home Monitoring ◽

Fecal Calprotectin ◽

Adult Patients ◽

Inflammatory Bowel

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Optimal Range of Fecal Calprotectin for Predicting Mucosal Healing in Patients with Inflammatory Bowel Disease: A Systematic Review and Meta-Analysis

Visceral Medicine ◽

10.1159/000514196 ◽

2021 ◽

pp. 1-11

Author(s):

Bing-Jie Xiang ◽

Min Jiang ◽

Ming-Jun Sun ◽

Cong Dai

Keyword(s):

Inflammatory Bowel Disease ◽

Diagnostic Accuracy ◽

Likelihood Ratio ◽

Sensitivity And Specificity ◽

Bowel Disease ◽

Meta Analysis ◽

Positive Likelihood Ratio ◽

Fecal Calprotectin ◽

Mucosal Healing ◽

Inflammatory Bowel

Objective: Fecal calprotectin (FC) is a promising marker for assessment of inflammatory bowel disease (IBD) activity. However, the utility of FC for predicting mucosal healing (MH) of IBD patients has yet to be clearly demonstrated. The objective of our study was to perform a meta-analysis evaluating the diagnostic accuracy of FC in predicting MH of IBD patients. Methods: We systematically searched the databases for studies from inception to April 2020 that evaluated MH in IBD. The methodological quality of each study was assessed according to the Quality Assessment of Diagnostic Accuracy Studies checklist. The extracted data were pooled using a summary receiver operating characteristic curve model. Random-effects model was used to summarize the diagnostic odds ratio, sensitivity, specificity, positive likelihood ratio, and negative likelihood ratio. Results: Sixteen studies comprising 1,682 ulcerative colitis (UC) patients and 4 studies comprising 221 Crohn’s disease (CD) patients were included. The best performance of FC for predicting MH in UC was at cut-off range of 60–75 μg/g with area under the curve (AUC) of 0.88 and pooled sensitivity and specificity of 0.87 and 0.79, respectively. The pooled sensitivity and specificity values of cutoff range 180–250 μg/g for predicting MH in CD were 0.67 and 0.76, respectively. The AUC of 0.79 also revealed improved discrimination for identifying MH in CD with FC concentration. Conclusion: Our meta-analysis has found that FC is a simple, reliable noninvasive marker for predicting MH in IBD patients. FC cutoff range 60–75 μg/g appears to have the best overall accuracy in UC patients.

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Predicting disease course in ulcerative colitis using stool proteins identified through an aptamer-based screen

Nature Communications ◽

10.1038/s41467-021-24235-0 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Sanam Soomro ◽

Suresh Venkateswaran ◽

Kamala Vanarsa ◽

Marwa Kharboutli ◽

Malavika Nidhi ◽

...

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Bowel Disease ◽

Fecal Calprotectin ◽

Disease Monitoring ◽

Disease Course ◽

Lipocalin 2 ◽

Time Points ◽

Inflammatory Bowel ◽

Stool Samples

AbstractIn the search for improved stool biomarkers for inflammatory bowel disease (IBD), an aptamer-based screen of 1129 stool proteins was conducted using stool samples from an IBD cohort. Here we report that of the 20 proteins subsequently validated by ELISA, stool Ferritin, Fibrinogen, Haptoglobin, Hemoglobin, Lipocalin-2, MMP-12, MMP-9, Myeloperoxidase, PGRP-S, Properdin, Resistin, Serpin A4, and TIMP-1 are significantly elevated in both ulcerative colitis (UC) and Crohn’s disease (CD) compared to controls. When tested in a longitudinal cohort of 50 UC patients at 4 time-points, fecal Fibrinogen, MMP-8, PGRP-S, and TIMP-2 show the strongest positive correlation with concurrent PUCAI and PGA scores and are superior to fecal calprotectin. Unlike fecal calprotectin, baseline stool Fibrinogen, MMP-12, PGRP-S, TIMP-1, and TIMP-2 can predict clinical remission at Week-4. Here we show that stool proteins identified using the comprehensive aptamer-based screen are superior to fecal calprotectin alone in disease monitoring and prediction in IBD.

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Leucine-rich alpha-2 glycoprotein is a potential biomarker to monitor disease activity in inflammatory bowel disease receiving adalimumab: PLANET study

Journal of Gastroenterology ◽

10.1007/s00535-021-01793-0 ◽

2021 ◽

Author(s):

Shinichiro Shinzaki ◽

Katsuyoshi Matsuoka ◽

Hiroki Tanaka ◽

Fuminao Takeshima ◽

Shingo Kato ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Crohn’S Disease ◽

Crohn's Disease ◽

Disease Activity ◽

Bowel Disease ◽

Fecal Calprotectin ◽

Potential Biomarker ◽

Adalimumab Therapy ◽

Adalimumab Treatment ◽

Inflammatory Bowel

Abstract Background This multicenter prospective study (UMIN000019958) aimed to evaluate the usefulness of serum leucin-rich alpha-2 glycoprotein (LRG) levels in monitoring disease activity in inflammatory bowel disease (IBD). Methods Patients with moderate-to-severe IBD initiated on adalimumab therapy were enrolled herein. Serum LRG, C-reactive protein (CRP), and fecal calprotectin (fCal) levels were measured at week 0, 12, 24, and 52. Colonoscopy was performed at week 0, 12, and 52 for ulcerative colitis (UC), and at week 0, 24, and 52 for Crohn’s disease (CD). Endoscopic activity was assessed using the Simple Endoscopic Score for Crohn’s Disease (SES-CD) for CD and the Mayo endoscopic subscore (MES) for UC. Results A total of 81 patients was enrolled. Serum LRG levels decreased along with improvements in clinical and endoscopic outcomes upon adalimumab treatment (27.4 ± 12.6 μg/ml at week 0, 15.5 ± 7.7 μg/ml at week 12, 15.7 ± 9.6 μg/ml at week 24, and 14.5 ± 6.8 μg/ml at week 52), being correlated with endoscopic activity at each time point (SES-CD: r = 0.391 at week 0, r = 0.563 at week 24, r = 0.697 at week 52; MES: r = 0.534 at week 0, r = 0.429 at week 12, r = 0.335 at week 52). Endoscopic activity better correlated with LRG compared to CRP and fCal on pooled analysis at all time points (SES-CD: LRG: r = 0.636, CRP: r = 0.402, fCal: r = 0.435; MES: LRG: r = 0.568, CRP: 0.389, fCal: r = 0.426). Conclusions Serum LRG is a useful biomarker of endoscopic activity both in CD and UC during the adalimumab treatment.

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