Cholangitis In Children Research Articles

Deep learning radiomics nomogram for preoperatively identifying moderate-to-severe chronic cholangitis in children with pancreaticobiliary maljunction: a multicenter study

BackgroundLong-term severe cholangitis can lead to dense adhesions and increased fragility of the bile duct, complicating surgical procedures and elevating operative risk in children with pancreaticobiliary maljunction (PBM). Consequently, preoperative diagnosis of moderate-to-severe chronic cholangitis is essential for guiding treatment strategies and surgical planning. This study aimed to develop and validate a deep learning radiomics nomogram (DLRN) based on contrast-enhanced CT images and clinical characteristics to preoperatively identify moderate-to-severe chronic cholangitis in children with PBM.MethodsA total of 323 pediatric patients with PBM who underwent surgery were retrospectively enrolled from three centers, and divided into a training cohort (n = 153), an internal validation cohort (IVC, n = 67), and two external test cohorts (ETC1, n = 58; ETC2, n = 45). Chronic cholangitis severity was determined by postoperative pathology. Handcrafted radiomics features and deep learning (DL) radiomics features, extracted using transfer learning with the ResNet50 architecture, were obtained from portal venous-phase CT images. Multivariable logistic regression was used to establish the DLRN, integrating significant clinical factors with handcrafted and DL radiomics signatures. The diagnostic performances were evaluated in terms of discrimination, calibration, and clinical usefulness.ResultsBiliary stones and peribiliary fluid collection were selected as important clinical factors. 5 handcrafted and 5 DL features were retained to build the two radiomics signatures, respectively. The integrated DLRN achieved satisfactory performance, achieving area under the curve (AUC) values of 0.913 (95% CI, 0.834–0.993), 0.916 (95% CI, 0.845–0.987), and 0.895 (95% CI, 0.801–0.989) in the IVC, and two ETCs, respectively. In comparison, the clinical model, handcrafted signature, and DL signature had AUC ranges of 0.654–0.705, 0.823–0.857, and 0.840–0.872 across the same cohorts. The DLRN outperformed single-modality clinical, handcrafted radiomics, and DL radiomics models, with all integrated discrimination improvement values > 0 and P < 0.05. The Hosmer–Lemeshow test and calibration curves showed good consistency of the DLRN (P > 0.05), and the decision curve analysis and clinical impact curve further confirmed its clinical utility.ConclusionsThe integrated DLRN can be a useful and non-invasive tool for preoperatively identifying moderate-to-severe chronic cholangitis in children with PBM, potentially enhancing clinical decision-making and personalized management strategies.

Phenotypes of primary sclerosing cholangitis in children

Background. Primary sclerosing cholangitis (PSC) in children is a rare chronic immune-mediated disease of the biliary tract, which, unlike in adults, has a less aggressive course with da­mage to the intrahepatic bile ducts and is combined with autoimmune hepatitis, creating a special clinical phenotype of PSC, autoimmune sclerosing cholangitis (ASC). Although immunosuppressive therapy is effective in controlling autoimmune inflammation, it does not inhibit the progression of fibrotic changes around the bile ducts, which, unfortunately, leads to the formation of biliary cirrhosis of the liver. Research aimed at studying the clinical course of PSC in children and improving early diagnosis is relevant, especially given the limited access to modern diagnostic methods, such as magnetic resonance cholangiopancreatography, the need for invasive studies, and the lack of standardized diagnostic criteria adapted to childhood, which complicates the diagnosis and treatment of these patients. Objective: to investigate the clinical features of primary sclerosing cholangitis in children and adolescents depending on di­sease phenotype with the aim of develo­ping individualized treatment approaches. Materials and methods. Retrospective and prospective analysis of the clinical course of primary sclerosing cholangitis in children and adolescents who were treated and followed at the Department of Pediatric Hepatology from 2016 to 2024 was conducted. The study included 68 children (62 % boys and 38 % girls) with PSC aged 3 to 18 years (mean age at diagnosis was (11.0 ± 3.9) years). Of these, 38 patients (56 %) had autoimmune sclerosing cholangitis, and 30 (44 %) had isolated PSC without clinical or histological signs of autoimmune hepatitis. Results. At disease onset, 40 % of children with PSC had liver fibrosis graded F3-F4 on METAVIR, with 24 % showing cirrhosis. The most common phenotype in children with PSC was a mixed one involving both large and small bile ducts (63 %). PSC with large bile duct involvement alone was observed in 11 % of cases, 70 % of these children were diagnosed with cirrhosis. Small duct involvement alone was present in 26 % of cases, with cirrhosis in 12.5 % (p = 0.01). Inflammatory bowel disease (IBD) was diagnosed in 84 % of children with PSC: 32 % had ulcerative colitis, 38 % had indeterminate IBD, and 16 % had Crohn’s disease. Pancolitis occurred in 56 % of cases, while 19 % of patients had histological signs of IBD without clinical or endoscopic manifestations. Asymptomatic IBD was diagnosed in 58 % of cases. The clinical course of ASC differed from isolated PSC with significantly higher rates of anemia (47 vs. 27 %, p = 0.03), elevated serum IgG levels (23.9 vs. 12.5 g/l, р &lt; 0.01), and higher levels of alanine aminotransferase, aspartate aminotransferase, total bilirubin (p &lt; 0.01 for all), non-invasive fibrosis markers (APRI, FIB-4, 2D-SWE) at onset (p &lt; 0.01; p = 0.01; p = 0.04, respectively). Liver fibrosis F3-F4 on METAVIR was more frequently diagnosed in ASC group than in patients with isolated PSC (73 vs. 40 %, p = 0.06). Large bile duct involvement alone was found only in isolated PSC (p &lt; 0.0009). No differences were observed between ASC and isolated PSC in terms of IBD phenotype. Conclusions. PSC in children is represented by 2 phenotypes (ASC and isolated PSC) that occur with almost equal frequency. ASC and isolated PSC have similar phenotypes of biliary and intestinal lesions, however, they differ in terms of clinical course and therapeutic approaches. Involvement of the large bile duct alone in children with PSC is associated with rapid formation of liver fibrosis and cirrhosis. PSC phenotype with involvement of small bile ducts alone has a favorable course in children. Most children with PSC exhibit the PSC-IBD phenotype. Active diagnostic search for biliary and intestinal lesions in children with PSC will facilitate the development of effective personalized approaches to treatment and monitoring, thereby improving disease prognosis.

Clinical Profile and Treatment Outcomes Of Acute Cholangitis in Children in a Tertiary Government Hospital in the Philippines: A Five-Year Retrospective Study.

Clinical Profile and Treatment Outcomes Of Acute Cholangitis in Children in a Tertiary Government Hospital in the Philippines: A Five-Year Retrospective Study.

Prophylactic Antibiotics to Prevent Cholangitis in Children with Biliary Atresia After Kasai Portoenterostomy: A Meta-Analysis.

A connection between the bowel and bile ducts after the Kasai hepatoportoenterostomy (HPE) procedure poses a risk of ascending cholangitis. There were only a few evidence-based consensuses on the benefits of prophylactic antibiotics. This study aims to assess the value of prophylactic antibiotics in reducing the risk of cholangitis following the Kasai HPE procedure. Meta-analysis is performed using random-effects model from the search result of 5 online databases (PubMed, Google Scholar, EBSCO MEDLINE, ClinicalTrials.gov , and EuropePMC) from inception to October 27, 2021. The keywords used were "antibiotic," "antimicrobial," "Kasai," "portoenterostomy," "biliary atresia," and "bile duct atresia." Cochrane Risk of Bias tool and Newcastle-Ottawa Scale is used to assess the risk of bias. The outcomes are incidence of cholangitis and native liver survival. Six studies consisting of 4 cohorts and 2 cross-sectional studies were extracted. A total of 714 patients reported different cholangitis incidence after prophylactic antibiotics administration post-Kasai HPE. The incidence of cholangitis following Kasai HPE was not statistically significant among participants. There is conflicting evidence on the efficacy of antibiotics in prolonging native liver survival. The existing evidence does not support the administration of prophylactic antibiotics in preventing cholangitis after Kasai HPE among biliary atresia patients. Additionally, their roles in native liver survival are still inconclusive. The fact that there were heterogeneous method and antibiotic usage between existing studies must also be highlighted for better design in future studies.

Treatment of intractable cholangitis in children with biliary atresia: Impact on outcome.

To evaluate the response and outcome with prolonged intravenous antibiotics including home-based intravenous antibiotics in children with intractable cholangitis (IC) after Kasai portoenterostomy (KPE) for biliary atresia (BA). A retrospective review of treatment and outcome of children with IC post KPE (no resolution after fourweeks of antibiotics) was donebetween 2014 and 2020. A protocol-based antibiotic regimen was used based on sensitivity and hospital antibiogram. Children afebrile for more than threedays were discharged on home intravenous antibiotics (HIVA). Twenty children with IC were managed with prolonged antibiotic regimen, including HIVA. All patients were initially listed for liver transplantation (LT) with indication being IC (n = 20) with portal hypertension (n = 12). Seven patients had bile lakes of which four underwent percutaneous transhepatic biliary drainage. Bile culture grew Klebsiella in four and Escherichia coli and Pseudomonas one each. There were eight children with IC who had positive blood culture with most of these organisms being gram-negative (Escherichia coli: 5, Klebsiella pneumoniae: 2, Enterococcus: 1). Median duration of antibiotics was 58days (interquartile range [IQR] 56-84). Median follow-up period post cholangitis was threeyears (IQR 2-4). Following treatment, 14 patients were successfully delisted from LT waitlist and are presently jaundice-free. Two of the five patients undergoing LT died of sepsis. One patient died awaiting LT. Timely and aggressive step-up antibiotic regimen may successfully treat IC and prevent/delay LT. HIVA provides a cost-effective and comfortable environment for a child which might improve compliance with intravenous antibiotics.

The Experience of Using Multipotent Mesenchymal Stromal Cells in the Treatment of Severe Recurrent Cholangitis in Children with Biliary Atresia after Kasai Surgery.

This article describes the experience of application of multipotent mesenchymal stromal cells in the complex therapy of severe recurrent cholangitis in 2 children with biliary atresia after Kasai surgery. In both children, hepatic cellular insufficiency and portal hypertension developed against the background of long-term inflammatory process poorly controlled by standard therapy, which was the indication for liver transplantation. During the course of mesenchymal stromal cells therapy, the relief of the inflammatory process and functional recovery of the liver were achieved. At the time of preparing the article, the follow-up of two children since the start of multipotent mesenchymal stromal cell therapy was 3 years 9 months and 2 years 6 months. No recurrence of cholangitis was observed in the patients during the follow-up period, the liver function was preserved. There are no indications for liver transplantation at this moment. Thus, despite the fact that the mechanisms of therapeutic action of multipotent mesenchymal stromal cells in biliary atresia require further investigation, we obtained promising results suggesting the possibility of using mesenchymal stromal cells in the treatment of postoperative complications in children with biliary atresia.

Primary sclerosing cholangitis in children with inflammatory bowel disease

Introduction. Currently, in pediatric practice, there are no clear data on the features of the onset of primary sclerosing cholangitis (PSC) in inflammatory bowel diseases (IBD), the frequency of detection of markers of cytolysis and cholestasis, the nature of the lesion of the hepatobiliary system and changes in the endoscopic picture. Purpose: assessment of clinical, laboratory, and instrumental features of PSC course in IBD children (PSC+IBD). Materials and methods. For the period 2015-2021, a comprehensive examination of 54 children with PSC+IBD (main group) and 79 patients with isolated IBD (comparison group): ulcerative colitis (UC) or Crohn’s disease (CD) was carried out. Results. In 54 children of the main group, the combination of PSC+UC was detected in 48 (88.9%) patients with a predominance of boys. At the onset of the disease, the most frequent manifestations in children with PSC+IBD were diarrhea, abdominal pain syndrome, cytolysis and cholestasis syndromes, and hemocolitis. In children with PSC+IBD, compared with patients with IBD, there was a statistically significant increase in serum concentrations of ALT, AST and total protein, indicating an increase in hepatitis activity; GGT, alkaline phosphatase and direct bilirubin - about an increase in the severity of cholestasis syndrome; IgG - as a marker of the severity of mesenchymal-inflammatory syndrome in the framework of liver damage. According to the endoscopic data, with PSC+UC, pancolitis with more pronounced involvement of the right colon or isolated proctitis prevails, and with PSC+CD, ileocolitis is more common. Indices PUCAI (p &lt; 0.001) and PCDAI (p = 0.027) were statistically significantly lower in the PSC+IBD group than in the isolated IBD. According to the data of transient liver elastometry, no statistically significant difference was obtained between the medians of the parameters of the biochemical blood test at different stages of fibrosis. According to the results of magnetic resonance imaging of the abdominal organs and MR-cholangiopancreatography, the most characteristic changes in PSC+IBD are heterogeneity of the liver parenchyma, unevenness of the intra- and extrahepatic bile ducts, the presence of areas of expansion and narrowing of their diameter. Conclusions. The phenotype of PSC+IBD in children is manifested mainly by intestinal symptoms, more often UC with involvement of the right parts of the intestine in boys and a lower activity index than the isolated course of IBD, in combination with laboratory syndromes and cholestasis.

Primary schlerosing cholangitis in children with inflammatory bowel disease

The article provides an overview of current data on clinical features, modern diagnostic methods and therapeutic strategies for primary sclerosing cholangitis in children with inflammatory bowel disease. Primary sclerosing cholangitis can be asymptomatic for a long time, acquiring a progressive character with liver cirrhosis. The disease is one of the most important risk factors for the development of cholangiocarcinoma. The article presents a diagnostic algorithm for increased transaminases in the serum of the children with inflammatory bowel disease, with an emphasis on various variants of hepatobiliary pathology including primary sclerosing cholangitis. The widespread application of the pediatric scale SCOPE (Sclerosing Cholangitis Outcomes in Pediatrics index) will allow us to timely diagnose and prescribe treatment for a patient with primary sclerosing cholangitis at an early stage improving the prognosis of the disease.

Anti-Proteinase 3 Antibodies as a Biomarker for Ulcerative Colitis and Primary Sclerosing Cholangitis in Children.

Anti-neutrophil cytoplasmic antibody (ANCA) directed against proteinase 3 (PR3) is a marker for granulomatosis with polyangiitis, but is also found in patients with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). The aim of our study was to investigate ANCA and PR3-ANCA in paediatric IBD. We tested 326 paediatric IBD patients and 164 controls for anti-Saccharomyces cerevisiae antibodies (ASCA), ANCA (indirect immunofluorescence, IIF) and PR3-ANCA (chemiluminescence immunoassay). We applied the Paris classification for paediatric IBD and documented liver manifestations such as primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH). We found PR3-ANCA in 49/121 (40%) of UC, 20/187 (11%) of Crohn disease (CD) and 2/18 (11%) of IBD-unclassified (IBD-U) patients but in none of the controls. 54% UC and 12% CD patients were positive for ANCA (IIF). PR3-ANCA positive UC patients were characterised by more extensive disease (P = .070). Fourteen of 21 (67%) of UC patients with backwash ileitis were anti-PR3 ANCA-positive (P = .011). We diagnosed PSC or PSC/AIH in 19 UC and 3 IBD-U patients. Fifteen of 22 (68%) patients with PSC or PSC/AIH were anti-PR3-ANCA positive in contrast to 36 of 117 (32%) patients without PSC (P = .001). PR3-ANCA positive patients showed higher levels of gamma-glutamyl transferase, alanine transaminase and aspartate transferase (P < 0.001, 0.001, 0.006, respectively). Forty-seven percent of CD and 6% of UC patients were ASCA-IgA positive. PR3-ANCA-positive and -negative patients showed no significant differences concerning age at diagnosis, disease activity, need for drugs, and number of hospitalisations. Our study provides data for PR3-ANCA as a potential serological marker for paediatric UC and PSC.

Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population-Single Center Study.

Background and Objectives: Primary sclerosing cholangitis (PSC) is a rare cholestatic disease of the liver of unknown etiology, severe course and poor prognosis. PSC most often co-occurs with inflammatory bowel diseases (IBD), especially with ulcerative colitis (UC). The aim of the study was the analysis of the clinical course of primary sclerosing cholangitis in children, hospitalized in the Gastroenterology Unit in Katowice. Materials and Methods: The analysis included 30 patients, aged from 7 to 18 years, 21/30 boys (70%) and 9/30 girls (30%), diagnosed with PSC in the years 2009–2019. The analysis included the age at diagnosis, clinical symptoms, course of the disease, coexisting diseases, laboratory and imaging results, and complications. Results: The average age at diagnosis was 13 years. 22/30 (73.3%) patients suffered from UC, 4/30 (13.3%) were diagnosed with Crohn’s disease (CD), 2/30 (6.66%) with Eosinophilic Colitis (EC). 2/30 patients (6.66%) had no clinical evidence of coexistent IBD to date. In addition, 7/30 (23.3%) had an overlap syndrome of primary sclerosing cholangitis/autoimmune hepatitis. When PSC was detected before IBD (6/30–20%), patients had complications more often compared to those diagnosed with IBD first or PSC and IBD at the same time. At the moment of diagnosis 6/30 (20%) patients presented with abdominal pain, which was the most common symptom, 3/30 (10%) jaundice, while 17/30 (56.6%) were asymptomatic but had abnormal results of the laboratory tests. Conclusions: Monitoring liver markers in IBD patients is important since most PSC cases are asymptomatic and their elevation might be the first sign of the disease. Patients diagnosed with PSC before IBD diagnosis are more likely to have a more aggressive course of the disease.

Langerhans cell histiocytosis confined to extrahepatic bile duct causing sclerosing cholangitis in child: a case report

BackgroundLangerhans cell histiocytosis (LCH) is an abnormal accumulation of Langerhans cells in various organs that sometimes induces organ dysfunction. LCH can affect the liver, resulting in sclerosing cholangitis and biliary cirrhosis. However, liver and bile duct involvement is usually observed in the disseminated form of LCH. We herein report a rare case of LCH localized only in the extrahepatic bile duct that resulted in severe liver cirrhosis.Case presentationA 3-year-old boy with elevated liver enzymes, obstructive jaundice, and dilation of the common bile duct was referred to our institution. Contrast-enhanced computed tomography showed atrophy of the right hepatic lobe, relative hypertrophy of the left hepatic lobe, choledocholiths, and biliary debris extensively with biliary duct dilation. Magnetic resonance cholangiopancreatography revealed dilation of the intrahepatic and extrahepatic bile ducts and multiple choleliths in the gallbladder and common bile duct. Laparoscopic cholecystectomy, intraoperative cholangiography, liver biopsy, and gastrointestinal fiberscopy were performed. A liver specimen showed severe biliary cirrhosis due to sclerosing cholangitis. The patient then underwent living-donor liver transplantation because of severe liver cirrhosis 3 months after the first surgery. The common bile duct was not suitable for duct-to-duct anastomosis and was resected because of severe inflammation. Histologic sections of the common bile duct showed histiocytic cell proliferation. Immunohistochemistry revealed histiocytoses that were positive for Langerin, S-100 protein, and CD1a. However, no histiocytic cell proliferation was noted in the liver tissue. The definitive diagnosis was LCH localized to the extrahepatic bile duct. LCH in the extrahepatic bile duct seemed to cause sclerosing cholangitis. The patient was discharged uneventfully 2 months after living-donor liver transplantation.ConclusionsLCH localized to the extrahepatic bile duct is extremely rare; however, LCH can still affect the extrahepatic bile ducts on occasion. LCH should be considered as a differential diagnosis if pediatric patients show the presence of sclerosing cholangitis.

Primary Sclerosing Cholangitis in Children

Primary Sclerosing Cholangitis in Children

Primary Sclerosing Cholangitis in Children With Inflammatory Bowel Diseases Is Associated With Milder Clinical Activity But More Frequent Subclinical Inflammation and Growth Impairment

Although inflammatory bowel diseases (IBD) associated with primary sclerosing cholangitis (PSC) have been well characterized in adults, there have been few pediatric studies, and these were small and produced conflicting results. We investigated features of PSC-IBD in children, compared with children with IBD without PSC. We performed a retrospective study of 74 children with PSC-IBD, diagnosed from 2000 through 2018, who were each matched with 2 children with ulcerative colitis or IBD-unclassified (controls) based on sex, date of birth, and type of IBD. We compared IBD distribution and clinical activity (remission, medication use, hospitalization, or colectomy) and patient growth between groups. Data were extracted from each hospital contact and analyzed using mixed effects analyses or Cox proportional hazards regression, adjusting for time-dependent medication exposure. Higher proportions of children with PSC-IBD had backwash ileitis, pancolitis, and rectal sparing, andmore severe right-sided disease, than controls (P < .05). Patients with PSC-IBD were more likely to be treated with only 5-ASA, compared with controls (odds ratio [OR], 3.04; 95% CI, 1.44-6.41) and to have IBD in clinical remission (OR, 2.94; 95% CI, 1.78-4.87). Risk of colectomy or treatment with a biologic agent was lower in patients with PSC-IBD than controls (hazard ratio, 0.24; 95% CI, 0.12-0.52). However, determination of IBD severity based on symptoms underestimated severity based on endoscopic activity in patients with PSC-IBD. Among patients with IBD in clinical remission, those with PSC were less likely to have endoscopic remission (OR, 0.44; 95% CI, 0.20-0.96). Patients with PSC-IBD were shorter and had lower weight over time, compared with controls. In a retrospective study, we found that features of IBD differed between children with vs without PSC, similar to adults. Despite the mild clinical activity of IBD in patients with PSC, lack of symptoms does not always indicate lack of mucosal inflammation. Children with PSC-IBD have greater growth impairments compared with children with ulcerative colitis or IBD-unclassified.

Treatment of primary sclerosing cholangitis in children.

Primary sclerosing cholangitis (PSC) is a rare disease of stricturing and destruction of the biliary tree with a complex genetic and environmental etiology. Most patients have co-occurring inflammatory bowel disease. Children generally present with uncomplicated disease, but undergo a variable progression to end-stage liver disease. Within ten years of diagnosis, 50% of children will develop clinical complications including 30% requiring liver transplantation. Cholangiocarcinoma is a rare but serious complication affecting 1% of children. Ursodeoxycholic acid and oral vancomycin therapy used widely in children as medical therapy, and may be effective in a subset of patients. Gamma glutamyltransferase is a potential surrogate endpoint for disease activity, with improved survival in patients who achieve a normal value. Endoscopic retrograde cholangiopancreatography is a necessary adjunct to medical therapy to evaluate mass lesions or dominant strictures for malignancy, and also to relieve biliary obstruction. Liver transplantation remains the only option for patients who progress to end-stage liver disease. We review special considerations for patients before and after transplant, and in patients with inflammatory bowel disease. There is presently no published treatment algorithm or guideline for the management of children with PSC. We review the evidence for drug efficacy, dosing, duration of therapy, and treatment targets in PSC, and provide a framework for endoscopic and medical management of this complex problem.

Autoimmune Sclerosing Cholangitis: a Review of the Overlap between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children

Pediatric patients with autoimmune liver disease (ALD) frequently demonstrate clinical, histopathologic, cholangiographic, and serologic overlap between primary sclerosing cholangitis and autoimmune hepatitis, termed autoimmune sclerosing cholangitis (ASC). Several large, collaborative studies have shed light on the epidemiology and natural history of ASC in the last 5 years. A detailed genetic and environmental description of the pathogenesis remains lacking however. Here, we review recent advancements in knowledge on ASC in children, and their implications on the management of this rare disease. Consensus diagnostic criteria for ASC do not exist, and we recommend labeling a patient as ASC only when clinical criteria for each of primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) are separately met. Treatment of ASC is based on the lobular component of inflammation, which behaves similar to AIH and responds to standard immunosuppression regimens. The cholangiopathy of ASC behaves like PSC and currently dictates prognosis as there is no effective therapy and no response to immunosuppression. Rates of liver transplantation for ASC are identical to those of PSC. Rather than specifically labeling patients as ASC, the most important tasks for clinicians managing ALD are to detect treatable autoimmune lobular inflammation in PSC patients and to identify cholangiopathy in AIH patients with persistent biochemical abnormalities prior to therapeutic escalation.

Prospective Evaluation of Sclerosing Cholangitis in Children With Autoimmune Liver Disease

Background and Aim: Autoimmune liver disease encompass “overlap syndromes” of autoimmune hepatitis (AIH) with primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). In children only AIH-PSC overlap syndrome also known as ‘autoimmune sclerosing cholangitis’ (ASC) has been recognized. We aimed to study prevalence of ASC by using MRC in children diagnosed as AIH, and to compare the clinical presentation and outcome of children with AIH and ASC, and compare cholangiographic appearance in AIH and in children with cirrhosis due to non-autoimmune, non-biliary etiology. Methods: We prospectively got Magnetic resonance cholangiography (MRC) done in 39 children with AIH and 19 controls with non-autoimmune, non-cholestatic causes of chronic liver disease. Based on MRC, children with definitive changes of sclerosing cholangitis (SC) were considered as ASC. Details of clinical features, laboratory parameters, liver histopathology were analysed. One child was excluded due to poor MRC imaging. Results: The median age of 38 children with AIH was 11.5 (range 3–18) years and 22 (55%) were girls. Majority (74%) were diagnosed as type 1 AIH. MRC was done in 11 children (29%) at the time of diagnosis while in 21 (71%) it was done after a median follow-up of 2.5 (range 0.3–10) years. Abnormal MRC changes were seen in 14/38 (37%) and 8/19 (42%) in children with AIH and controls, however definite changes of SC were present in 4 children (10.5%) of AIH group, none in controls. None of the clinical, laboratory and histological parameters were significantly different between ASC (n = 4) and AIH (n = 34) groups. Conclusion: Prevalence of ASC was seen in 10% of children with AIH. There was no difference in clinical, biochemical, histological features and treatment response between children with ASC and AIH. We suggest MRC in select group of children instead of all children with AIH. The authors have none to declare.

FRI-385 - Diagnosis of autoimmune sclerosing cholangitis in children with autoimmune hepatitis

FRI-385 - Diagnosis of autoimmune sclerosing cholangitis in children with autoimmune hepatitis

Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children and Adolescents.

Clinical presentation and histopathology of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) are similar, but their management is different. We conducted a pediatric retrospective cross-sectional study of 34 patients with AIH and PSC. AIH had female predominance (74%) and was lower in PSC (45%). There was a trend toward higher frequency of blacks in PSC/OS (55%) compared to Caucasians (36%) and Hispanics (9%), but not race differences in AIH. Inflammatory bowel disease (IBD) was present in 75% of PSC/OS. Plasma cells were not specific for AIH (found in 42% of PSC). Concentric fibrosis was not reliable for PSC as was found in 46% of AIH. Conclusion: A combination of clinical history, laboratory tests, imaging studies and liver biopsy are required to confirm and properly treat AIH and PSC. Liver biopsy should be used to grade severity and disease progression, but cannot be used alone to diagnose these conditions.

Cholangitis in children with biliary atresia: Health‐care resource utilisation

Cholangitis is a well-known complication that contributes to morbidity, mortality, as well as health-care utilisation in children with biliary atresia who have undergone the Kasai portoenterostomy. The aim of the study was to determine the common causative organisms for cholangitis and characterise its burden, health-care resource and service utilisation and cost. This was a retrospective chart review of children who underwent Kasai portoenterostomy in our institution from 1988 to 2011. The causative organisms were identified based on culture reports. The burden of the disease was estimated based on the number of patients experiencing one or more episodes of cholangitis. Health-care resource and service utilisation were based on different categories, and cost was computed based on the charges at the institution. Twenty-seven (64.3%) out of 42 children included in the analysis experienced at least one episode of cholangitis. There were a total of 97 episodes of cholangitis, with an average of 3.6 (1-15) episodes per patient. The average length of stay per episode of cholangitis was 14.8 (2-64) days. Common organisms isolated during blood cultures were Klebsiella pneumoniae, Enterococcus, Escherichia coli and Pseudomonas aeruginosa. The estimated cost per in-patient admission of 15 days (rounded off) for a single episode of cholangitis was $SG 8986.61 ($US 7369.02). The knowledge about the incidence and cost of cholangitis will allow physicians to counsel parents of children newly diagnosed with biliary atresia and to better prepare them both emotionally and financially for what to expect.

Diagnosis of sclerosing cholangitis in children: Blinded, comparative study of magnetic resonance versus endoscopic cholangiography

Magnetic resonance cholangiography (MRC) has been validated as comparable to endoscopic retrograde cholangiography (ERC) for the diagnosis of sclerosing cholangitis (SC) in adult patients. In children, MRC is widely used based mainly on non-comparative studies. ERCs and MRCs of seven children (median age 9, range: 7-20 years) with SC and 17 controls (median age 6, range: 2 months-20 years) with other chronic liver diseases were reviewed in a blinded, random and independent way. All patients underwent both examinations within a 6-months slot. All ERCs and 17 MRCs were performed under general anesthesia. One radiologist evaluated both ERCs and MRCs and one interventional endoscopist independently reviewed only ERCs. Reviewers did not receive any clinical information. Diagnosis of SC, established on the basis of history, laboratory data, radiological examinations and clinical course, was used as gold standard to compare ERC and MRC diagnostic accuracy. Overall image quality was graded as very good in 57% of MRC and in 71% of ERC cases; difference was not statistically significant (P=0.24) although the probability for MRC to be diagnostic increased with patient's age. Depiction of first, second and fourth-order intrahepatic bile duct was better in ERC (P=0.004, 0.02 and 0.023, respectively); depiction of the extrahepatic bile duct was comparable (P=0.052). Diagnostic accuracy of MRC and ERC was very high, without statistically significant difference (P=0.61). Despite an overall better depiction of the biliary tree by ERC, MRC is comparable for the diagnosis of SC in children. These data support MRC as the first imaging approach in children with suspected SC.