OBJECTIVE: To summarize the current evidence and to make recommendations for the diagnosis and management of intrahepatic cholestasis of pregnancy.
METHODS: Pregnant people with intrahepatic cholestasis of pregnancy.
METHODS: Diagnosing the condition using fasting or non-fasting bile acids, classifying disease severity, determining what treatment to offer, establishing how to monitor for antenatal fetal wellbeing, identifying when to perform elective birth.
RESULTS: Individuals with intrahepatic cholestasis of pregnancy are at increased risk of adverse perinatal outcomes including preterm birth, neonatal respiratory distress and admission to a neonatal intensive care unit, with an increased risk of stillbirth when bile acid levels are ≥100 μmol/L. There is inequity in bile acid testing availability and timely access to results, along with uncertainly of how to treat, monitor. and ultimately deliver these pregnancies. Optimization of diagnostic and management protocols can improve maternal and fetal postnatal outcomes.
METHODS: Medline, PubMed, Embase, and the Cochrane Library were searched from inception to March 2023, using medical subject headings (MeSH) and keywords related to pregnancy, intrahepatic cholestasis of pregnancy, bile acids, pruritis, ursodeoxycholic acid, and stillbirth. This document presents an abstraction of the evidence rather than a methodological review.
METHODS: The authors rated the quality of evidence and strength of recommendations using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. See Appendix A (Tables A1 for definitions and A2 for interpretations).
UNASSIGNED: Obstetric care providers, including obstetricians, family physicians, nurses, midwives, maternal-fetal medicine specialists, and radiologists.
UNASSIGNED: Intrahepatic cholestasis of pregnancy requires adequate diagnosis with non-fasting bile acid levels which guide optimal management and delivery timing.

阿拉杰里综合征是儿科胆汁淤积性肝病的重要遗传病因，也是伴有多系统受累的最常见的胆汁淤积症病因。因为相对罕见，且临床表现多变，造成误诊和漏诊相当常见，进而延误治疗。为改进对阿拉杰里综合征相关肝病的管理，中国罕见病联盟遗传性肝病分会等组织了儿科肝病相关专家经过证据筛查和反复讨论共同制订了“阿拉杰里综合征相关肝病诊治共识（2024）”，旨在更好地指导临床实践，帮助儿科医生尽早识别该病，并通过科学管理以改善患儿预后。.

Genetic cholestatic liver diseases are caused by (often rare) mutations in a multitude of different genes. While these diseases differ in pathobiology, clinical presentation and prognosis, they do have several commonalities due to their cholestatic nature. These Clinical Practice Guidelines (CPGs) offer a general approach to genetic testing and management of cholestatic pruritus, while exploring diagnostic and treatment approaches for a subset of genetic cholestatic liver diseases in depth. An expert panel appointed by the European Association for the Study of the Liver has created recommendations regarding diagnosis and treatment, based on the best evidence currently available in the fields of paediatric and adult hepatology, as well as genetics. The management of these diseases generally takes place in a tertiary referral centre, in order to provide up-to-date approaches and expertise. These CPGs are intended to support hepatologists (for paediatric and adult patients), residents and other healthcare professionals involved in the management of these patients with concrete recommendations based on currently available evidence or, if not available, on expert opinion.

Improved outcomes of liver disease in childhood and young adulthood have resulted in an increasing number of young adults (YA) entering adult liver services. The adult hepatologist therefore requires a working knowledge in diseases that arise almost exclusively in children and their complications in adulthood.
To provide adult hepatologists with succinct guidelines on aspects of transitional care in YA relevant to key disease aetiologies encountered in clinical practice.
A systematic literature search was undertaken using the Pubmed, Medline, Web of Knowledge and Cochrane database from 1980 to 2023. MeSH search terms relating to liver diseases (\'cholestatic liver diseases\', \'biliary atresia\', \'metabolic\', \'paediatric liver diseases\', \'autoimmune liver diseases\'), transition to adult care (\'transition services\', \'young adult services\') and adolescent care were used. The quality of evidence and the grading of recommendations were appraised using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system.
These guidelines deal with the transition of YA and address key aetiologies for the adult hepatologist under the following headings: (1) Models and provision of care; (2) screening and management of mental health disorders; (3) aetiologies; (4) timing and role of liver transplantation; and (5) sexual health and fertility.
These are the first nationally developed guidelines on the transition and management of childhood liver diseases in adulthood. They provide a framework upon which to base clinical care, which we envisage will lead to improved outcomes for YA with chronic liver disease.

OBJECTIVE: Single-operator cholangioscopy (SOC) offer a diagnostic and therapeutic alternative with an improved optical resolution over conventional techniques; however, there are no standardized clinical practice guidelines for this technology. This evidence-based guideline from the Colombian Association of Digestive Endoscopy (ACED) intends to support patients, clinicians, and others in decisions about using in adults the SOC compared to endoscopic retrograde cholangiopancreatography (ERCP), to diagnose indeterminate biliary stricture and to manage difficult biliary stones.
METHODS: ACED created a multidisciplinary guideline panel balanced to minimize potential bias from conflicts of interest. Universidad de los Andes and the Colombia Grading of Recommendations Assessment, Development and Evaluation (GRADE) Network supported the guideline-development process, updating and performing systematic evidence reviews. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The GRADE approach was used, including GRADE Evidence-to-Decision frameworks.
RESULTS: The panel agreed on one recommendation for adult patients with indeterminate biliary strictures and one for adult patients with difficult biliary stones when comparing SOC versus ERCP.
CONCLUSIONS: For adult patients with indeterminate biliary strictures, the panel made a conditional recommendation for SOC with stricture pattern characterization over ERCP with brushing and/or biopsy for sensitivity, specificity, and procedure success rate outcomes. For the adult patients with difficult biliary stones the panel made conditional recommendation for SOC over ERCP with large-balloon dilation of papilla. Additional research is required on economic estimations of SOC and knowledge translation evaluations to implement SOC intervention in local contexts.

OBJECTIVE: The aim of this study was to clarify the clinical characteristics of acute cholangitis (AC) after bilioenteric anastomosis and stent-related AC in a multi-institutional retrospective study, and validate the TG18 diagnostic performance for various type of cholangitis.
METHODS: We retrospectively reviewed 1079 AC patients during 2020, at 16 Tokyo Guidelines 18 (TG 18) Core Meeting institutions. Of these, the post-biliary reconstruction associated AC (PBR-AC), stent-associated AC (S-AC) and common AC (C-AC) were 228, 307, and 544, respectively. The characteristics of each AC were compared, and the TG18 diagnostic performance of each was evaluated.
RESULTS: The PBR-AC group showed significantly milder biliary stasis compared to the C-AC group. Using TG18 criteria, definitive diagnosis rate in the PBR-AC group was significantly lower than that in the C-AC group (59.6% vs. 79.6%, p < .001) because of significantly lower prevalence of TG 18 imaging findings and milder bile stasis. In the S-AC group, the bile stasis was also milder, but definitive-diagnostic rate was significantly higher (95.1%) compared to the C-AC group. The incidence of transient hepatic attenuation difference (THAD) and pneumobilia were more frequent in PBR-AC than that in C-AC. The definitive-diagnostic rate of PBR-AC (59.6%-78.1%) and total cohort (79.6%-85.3%) were significantly improved when newly adding these items to TG18 diagnostic imaging findings.
CONCLUSIONS: The diagnostic rate of PBR-AC using TG18 is low, but adding THAD and pneumobilia to TG imaging criteria may improve TG diagnostic performance.

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.
【更新要点】： 自2010年至今指南有哪些新内容？A.纳入原发性和非原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）患者的胆管癌的诊断和管理指引。B.引入术语相关狭窄一词，定义为与梗阻性胆汁淤积和/或细菌性胆管炎的体征或症状相关的任何胆总管或肝管的胆道狭窄。C.对于磁共振成像和胰胆管造影结果不明确的患者，出于诊断目的，应复查高质量的磁共振成像/胰胆管造影。应避免为了诊断PSC进行内镜逆行胰胆管造影。D.对于未知炎症性肠病（inflammatory bowel disease，IBD）的PSC患者，应进行诊断性结肠镜组织学取样检查，每5年复查1次，直至检测到IBD。E.对于合并IBD的PSC患者，应从15岁开始进行结肠癌监测。F.PSC的临床风险新工具可用于风险分层，但应谨慎解释个体发生率。G.所有PSC患者应考虑参与临床试验；然而，如果熊去氧胆酸（13~23 mg·kg(-1)·d(-1)）耐受良好，且在治疗12个月后碱性磷酸酶(儿童中的γ-谷氨酰转移酶)和/或症状有显著改善，则可以考虑继续使用。H.所有疑似肝门部或远端胆管癌的患者均应进行内镜逆行胰胆管造影胆道细胞学刷检和荧光原位杂交分析。I.除了PSC和复发性胆管炎的患者，终末期肝病模型标准有统一的网络器官共享新政策。对于不可切除的肝门部胆管癌，直径< 3 cm或合并PSC且无肝内(外)转移的患者，建议新辅助治疗后进行肝移植。.

This clinical practice guideline from the American Society for Gastrointestinal Endoscopy provides an evidence-based approach for strategies to manage biliary strictures in liver transplant recipients. This document was developed using the Grading of Recommendations Assessment, Development and Evaluation framework. The guideline addresses the role of ERCP versus percutaneous transhepatic biliary drainage and covered self-expandable metal stents (cSEMSs) versus multiple plastic stents for therapy of post-transplant strictures, use of MRCP for diagnosing post-transplant biliary strictures, and administration of antibiotics versus no antibiotics during ERCP. In patients with post-transplant biliary strictures, we suggest ERCP as the initial intervention and cSEMSs as the preferred stent for extrahepatic strictures. In patients with unclear diagnoses or intermediate probability of a stricture, we suggest MRCP as the diagnostic modality. We suggest that antibiotics should be administered during ERCP when biliary drainage cannot be ensured.

This clinical practice guideline from the American Society for Gastrointestinal Endoscopy provides an evidence-based approach for strategies to manage biliary strictures in liver transplant recipients. This document was developed using the Grading of Recommendations Assessment, Development and Evaluation framework. The guideline addresses the role of ERCP versus percutaneous transhepatic biliary drainage and covered self-expandable metal stents (cSEMSs) versus multiple plastic stents for therapy of strictures, use of MRCP for diagnosing post-transplant biliary strictures, and administration of antibiotics versus no antibiotics during ERCP. In patients with post-transplant biliary strictures, we suggest ERCP as the initial intervention and cSEMSs as the preferred stent. In patients with unclear diagnosis or intermediate probability of a stricture, we suggest MRCP as the diagnostic modality. We suggest that antibiotics should be administered during ERCP when biliary drainage cannot be assured.

This study reviewed the literature regarding the diagnosis, antepartum surveillance, and timing of delivery of pregnancies complicated by intrahepatic cholestasis of pregnancy, comparing the guidelines published by the Society for Maternal-Fetal Medicine in February 2021 and those published by the Royal College of Obstetricians and Gynaecologists in the United Kingdom in June 2022. Several key differences exist in the clinical guidelines between the 2 organizations. With regard to the diagnosis of intrahepatic cholestasis of pregnancy, the Society for Maternal-Fetal Medicine considers any elevation in bile acids above the upper limit of normal in the setting of maternal pruritus diagnostic of intrahepatic cholestasis of pregnancy, whereas the Royal College of Obstetricians and Gynaecologists requires a pregnancy-specific elevated bile acid level of ≥19 mmol/L for diagnosis. Regarding the treatment of intrahepatic cholestasis of pregnancy, the Society for Maternal-Fetal Medicine recommends ursodeoxycholic acid as the first-line treatment of maternal symptoms. In contrast, the Royal College of Obstetricians and Gynaecologists specifically recommends against the routine use of ursodeoxycholic acid for intrahepatic cholestasis of pregnancy because of a lack of evidence regarding both maternal and fetal benefit. The Society for Maternal-Fetal Medicine recommends fetal surveillance at a gestational age when abnormal fetal testing would result in delivery being performed, whereas the Royal College of Obstetricians and Gynaecologists does not recommend any fetal testing beyond fetal kick count assessment. The Society for Maternal-Fetal Medicine recommends delivery at 36 to 39 weeks\' gestation for intrahepatic cholestasis of pregnancy with bile acids <100 mmol/L and delivery at 36 weeks for bile acid levels >100 mmol/L. The Royal College of Obstetricians and Gynaecologists recommends serial assessment of bile acids with delivery timing stratified between 35- and 40-weeks\' gestation according to bile acid levels.