Prolonged Cholestasis Research Articles

Prediabetes persistence or remission and subsequent risk of gallbladder cancer: A nationwide cohort study

Background & aimsHyperglycemia is associated with an increased risk of gallbladder cancer (GBC), potentially by inhibiting gallbladder motility and inducing prolonged cholestasis. Although intermediate hyperglycemia (or prediabetes) is highly reversible, evidence is lacking about whether prediabetes persistence or remission is associated with an altered GBC risk. MethodsThis nationwide cohort study included 6058,662 adults without diabetes or cancer who underwent national health examinations twice in 2-year intervals between 2009 (S1) and 2011 (S2) and were followed-up until 2018. Prediabetes was defined as a fasting plasma glucose level of 100–125 mg/dL. We categorized changes in prediabetes status into: stable normoglycemia, new-onset prediabetes, prediabetes remission, and persistent prediabetes groups. GBC risk was estimated using Cox proportional hazards models, after adjusting for potential confounders. ResultsDuring 38.6 million person-years (median 6.4 years) of follow-up, 1349 new GBC cases were identified. Among 1409,474 individuals with prediabetes at S1, 768,515 achieved prediabetes remission at S2, outnumbering the 640,959 individuals with persistent prediabetes. GBC incidence probability was consistently higher among individuals with persistent prediabetes than in individuals with stable normoglycemia or prediabetes remission (all log-rank P < 0.01). Compared with stable normoglycemia, persistent prediabetes was associated with increased GBC risk (adjusted hazard ratio [aHR], 95 % CI: 1.21, 1.04 to 1.41). The aHRs of GBC were 1.14 (95 % CI, 0.99 to 1.33) and 1.03 (95 % CI, 0.88 to 1.21) for new-onset prediabetes and prediabetes remission, respectively. ConclusionsIndividuals with persistent prediabetes had a significantly increased risk of GBC, whereas those with prediabetes remission had no increased risk. Achieving prediabetes remission has a significant potential to reduce the risk of GBC.

Are Medical Students and Primary Health-care Professionals Aware of Neonatal Cholestasis and Acholic Stool.

Early diagnosis of biliary atresia (BA) and the timing of Kasai hepatic portoenterostomy are associated with improved survival rates of the native liver. Acholic stool is a major and earliest sign of BA. We evaluated the awareness and recognition of medical students and primary health care professionals (PHCPs) about neonatal cholestasis and acholic stool as a marker of BA. The knowledge of students and PHCP about prolonged jaundice and acholic stool was evaluated through a questionnaire. In the first step, 5 questions evaluating the knowledge of prolonged jaundice were asked. The sixth question was "Have you ever seen acholic stool before?" Following this question, stool color cards with 9 colors were shown, and participants were asked "Which of the following stool pictures would you define as acholic?" A total 724 students and 88 PHCPs were included in the study. In both groups, about half of the participants could not answer the first 4questions related to prolonged jaundice and cholestasis correctly. Twenty-four percent of the students and 11.4% of PHCP answered correctly to all of the stool colors. The rate of correct answers to acholic stool colors were approximately 43.9%-87.6% and 23.9%-86.4% for students and PHCP, respectively. Whitish acholic stool colors were better known than mild yellowish pale stool colors. The percentages of recognition were less than about 50% for these stool colors. This study showed that recognition and awareness of prolonged jaundice are low, and acholic stool is not well known. This may lead to delay in diagnosis. Considering the international success of stool color cards, using stool color cards will improve the outcomes of biliary atresia in our country as well.

Acute Pancreatitis in a Case of Hepatitis A Virus-Related Prolonged Cholestasis: Case Report and Review of Literature

Acute Pancreatitis in a Case of Hepatitis A Virus-Related Prolonged Cholestasis: Case Report and Review of Literature

Use of Steroids in a Case of Hepatitis A Virus-Related Severe Cholestasis: Case Report and Review of Literature

Hepatitis A virus (HAV) infection is endemic in many underdeveloped countries. Prolonged cholestasis has been reported in 0.8% of HAV hepatitis related patients. We report a case of HAV related prolonged cholestasis and discuss the role of steroids in the management. Keywords: Hepatitis A; Prolonged Cholestasis; Liver Disease; Real World Evidence

Treatment Options for Hepatitis A and E: A Non-Systematic Review.

Hepatitis A and hepatitis E are relatively common causes of liver disease. Both viruses are mainly transmitted through the faecal-oral route and, consequently, most outbreaks occur in countries with poor sanitation. An important role of the immune response as the driver of liver injury is also shared by the two pathogens. For both the hepatitis A (HAV) and hepatitis E (HEV) viruses, the clinical manifestations of infection mainly consist of an acute disease with mild liver injury, which results in clinical and laboratory alterations that are self-limiting in most cases. However, severe acute disease or chronic, long-lasting manifestations may occur in vulnerable patients, such as pregnant women, immunocompromised individuals or those with pre-existing liver disease. Specifically, HAV infection rarely results in fulminant hepatitis, prolonged cholestasis, relapsing hepatitis and possibly autoimmune hepatitis triggered by the viral infection. Less common manifestations of HEV include extrahepatic disease, acute liver failure and chronic HEV infection with persistent viraemia. In this paper, we conduct a non-systematic review of the available literature to provide a comprehensive understanding of the state of the art. Treatment mainly consists of supportive measures, while the available evidence for aetiological treatment and additional agents in severe disease is limited in quantity and quality. However, several therapeutic approaches have been attempted: for HAV infection, corticosteroid therapy has shown outcome improvement, and molecules, such as AZD 1480, zinc chloride and heme oxygenase-1, have demonstrated a reduction in viral replication in vitro. As for HEV infection, therapeutic options mainly rely on the use of ribavirin, and some studies utilising pegylated interferon-alpha have shown conflicting results. While a vaccine for HAV is already available and has led to a significant reduction in the prevalence of the disease, several vaccines for HEV are currently being developed, with some already available in China, showing promising results.

Prolonged Cholestasis in Patient with Acute HAV Infection

Background: Hepatitis A virus (HAV) is a virus which infects yearly more than 1.5 million persons worldwide and is usually spread through contaminated food and water. Most HAV infection in children under the six years of age are asymptomatic and they develop lifelong immunity. However, HAV infection in older children and adults often symptomatic with development of jaundice, nausea, vomiting, poor appetite, fever, fatigue, abdominal pain, etc. most of those infected patients usually fully recovering from HAV infection naturally within 2 months, it has not been a cause for major concern of public health). With more and more patients suffering from hepatitis A, there is also increase in the number of patients suffering from unusual presentation of HAV infection also. Most hepatitis A patients tend to recover from the illness natural without any complications; however, there are occasions of serious complications among adult hepatitis A patients such as extrahepatic manifestation, acute renal failure, cholestatic hepatitis, relapsing hepatitis, prolonged course and even fulminant hepatic failure. Cholestatic hepatitis accounts for between 0.8 - 5.2% of hepatitis. Together with increasingly more cases of hepatitis A being reported, the number of patients who have cholestatic hepatitis arising from hepatitis A also has increased. The current treatment for hepatitis A is usually symptomatic treated by sufficient nutrition, vitamin supply and sufficient rest. There are also no special treatments for cholestatic hepatitis, with conservative treatment being used. Ursodeoxycholic acid (UDCA) and cholestyramine and antihistamines are used to treat skin itching caused by cholestatic hepatitis; however, there are occasions where symptoms of cholestatic hepatitis are so severe and prolong that required the use of corticosteroids to shorten the duration of symptoms and reduce hospitalization.as to call for liver transplantation. Aim: Is to show the effectiveness of corticosteroids in shorten the duration of severe symptoms and shorten hospitalization in patient with prolong severe cholestatic symptoms of acute hepatitis A virus infection.

Home Parenteral Nutrition for Children: What Are the Factors Indicating Dependence and Mortality?

Parenteral nutrition (PN) in children with short bowel syndrome is crucial and lifesaving. Taking care of such patients requires interprofessional practice and multiple team resource management. Home PN (HPN) usage allows patients and families to live regular lives outside hospitals. We share our experiences for the last two decades and identify the risk factors for complications and mortality. A retrospective study of HPN patients was conducted between January 2000 and February 2022. Medical records of age, body weight, diagnosis, length of residual intestines, HPN period, central line attempts, complications, weaning, and survival were collected and analyzed. The patients were classified as HPN free, HPN dependent, and mortality groups. A total of 25 patients received HPN at our outpatient clinic, and one was excluded for the adult age of disease onset. There were 13 patients (54.1%) who were successfully weaned from HPN until the record-enroled date. The overall mortality rate was 20.8% (five patients). All mortality cases had prolonged cholestasis, Child Class B or C, and a positive Pediatric End-Stage Liver Disease (PELD) score. For HPN dependence, extended resection and multiple central line placement were two significant independent factors. Cholestasis, Child Class B or C, and positive PELD score were the most important risk factors for mortality. The central line-related complication rate was not different in all patient groups. The overall central line infection rate was 1.58 per 1000 catheter days. Caution should be addressed to prevent cholestasis and intestinal failure-associated liver disease during the HPN period, to prevent mortality. By understanding the risks of HPN dependence and mortality, preventive procedures could be addressed earlier.

Acute Viral Hepatitis with Prolonged Cholestasis: Role of steroids and Experimental Therapies in Management

Acute Viral Hepatitis with Prolonged Cholestasis: Role of steroids and Experimental Therapies in Management

HEPATITIS A. THE FEATURES OF DISEASE COURSE IN ADULTS.

The aim: To analyze the incidence of Hepatitis A in Ukraine and Poltava region and to study the clinical and epidemiological features of the course of Hepatitis A in adult patients. Materials and methods: The course of HA in 96 hospitalized patients was analyzed. The diagnosis of HA was established on the basis of clinical and epide¬miological data and confirmed by the results of laboratory studies (serological and molecular biological). Results: In 2019, in the Poltava region, there was an increase in the incidence of Hepatitis A with a predominance among sick people of working age, among the urban population. This part of people aged from 60 to 75 years old constitutes 9.4%. This study showed that the waterway was the dominant way of HA transmission. The course of the disease in most hospitalized patients was typical and cyclic, with a predominance of a mixed variant of the pre-jaundice period and jaundice. One third of patients survey that they had fever, which persisted with jaundice. Conclusions: The findings of this study indicates that the patients older than 40 years were more likely to have concomitant chronic pathology than younger patients, and Hepatitis A was more severe with the development of prolonged cholestasis, wave-like course and recurrence. In most patients under the age of 40, the course of Hepatitis A was mild, but splenomegaly and severe cytolytic syndrome were more common.

A rare case of acute liver failure with intrahepatic cholestasis due to dengue hemorrhagic fever: CytoSorb® and plasma exchange aided in the recovery: case report

BackgroundDengue haemorrhagic fever is a severe form of acute dengue infection characterized by leakage of plasma through capillaries into body spaces resulting in circulatory insufficiency leading to shock. Despite varying degrees of liver involvement occurring in acute dengue infection, intrahepatic cholestasis is very rare in the literature with only two cases reported so far. We report a challenging case of a middle-aged woman with DHF complicated by acute liver failure, coagulopathy, acute renal failure and prolonged intrahepatic cholestasis. She was successfully managed in the intensive care unit with supportive therapy, Cytosorb® and therapeutic plasma exchange.Case presentationA 54-year-old Sri Lankan obese woman with multiple comorbidities presented with fever, headache, vomiting and generalized malaise for 3 days and was diagnosed with dengue haemorrhagic fever. Despite the standard dengue management, she clinically deteriorated due to development of complications such as, acute liver injury, intrahepatic cholestasis and acute renal injury. Acute liver failure was evidenced by transaminitis, lactic acidosis, coagulopathy with pervaginal bleeding and severe encephalopathy necessitating elective intubation and mechanical ventilation. She was immediately transferred to intensive care facilities where she underwent supportive management for liver failure, continuous renal replacement therapy coupled with cytosorb and therapeutic plasma exchange with which she made a remarkable recovery.ConclusionAcute liver failure with a prolonged phase of intrahepatic cholestasis is a very rare complication of acute dengue illness which is sparsely documented in medical literature so far. This patient was managed successfully with supportive therapy, aided by cytoSorb hemo-adsorption and therapeutic plasma exchange.

Pediatric acute viral hepatitis with atypical variants: Clinical dilemmas and natural history.

Classical acute viral hepatitis (AVH) has an uncomplicated outcome. Acute liver failure has a grave prognosis. Atypical manifestations of AVH are a group of disorders that causes significant morbidity and dilemmas in children. These include prolonged cholestasis, relapsing hepatitis, ascitic form of AVH, late-onset hepatic failure (LOHF), intravascular hemolysis, and provoking an autoimmune trigger leading to autoimmune hepatitis. These entities cause significant liver dysfunction or worsening and are often difficult to differentiate from chronic liver disease (CLD). Ascitic form of AVH, LOHF, decompensated CLD and acute-on-chronic liver failure have significant overlapping features that need to be carefully dissected out. In many cases, only on long-term follow-up, these clinical entities can be separately identified. Intravascular hemolysis is usually caused by associated glucose-6-phosphate dehydrogenase deficiency. Rarely CLD such as Wilson disease and autoimmune hepatitis can also present with hemolysis in the initial presentation, which can mimic AVH with hemolysis. Identifying deviations from typical manifestations aid in avoiding unnecessary investigations, allowing focused therapy and alleviating anxiety.

Prolonged Cholestasis Due to Hepatitis A Virus Infection in a Young Adolescent Male with a Known Case of Hemoglobin E Disease: Early Response to Steroid Therapy

Background: Acute hepatitis due to hepatitis A virus infection is a self-limiting mild disease. Sometimes, it may have an atypical course in few percentages. We reviewed with characteristics, response to steroid therapy, and outcome of prolonged cholestatic jaundice in hepatitis A virus infection. Case Summary: We analyzed acute hepatitis with a prolonged cholestatic course due to hepatitis A virus infection in an adolescent with preexisting hemoglobin E disease. Bilirubin was gradually increasing, the maximum total bilirubin was 48.3 mg/dl, and direct 46.6 mg/dl at day 50 of symptoms onset. After that, the steroid was started along with ursodeoxycholic acid. The patient gradually improved clinically and biochemically. Conclusion: Hepatitis A virus infection may cause prolonged cholestatic severe jaundice in a patient with preexisting congenital hemolytic disease. Steroid therapy may induce early recovery.

Prolonged Cholestasis Due to Hepatitis A Virus Infection in a Young Adolescent Male with a Known Case of Hemoglobin E Disease: Early Response to Steroid Therapy

Background: Acute hepatitis due to hepatitis A virus infection is a self-limiting mild disease. Sometimes, it may have an atypical course in few percentages. We reviewed with characteristics, response to steroid therapy, and outcome of prolonged cholestatic jaundice in hepatitis A virus infection. Case Summary: We analyzed acute hepatitis with a prolonged cholestatic course due to hepatitis A virus infection in an adolescent with preexisting hemoglobin E disease. Bilirubin was gradually increasing, the maximum total bilirubin was 48.3 mg/dl, and direct 46.6 mg/dl at day 50 of symptoms onset. After that, the steroid was started along with ursodeoxycholic acid. The patient gradually improved clinically and biochemically. Conclusion: Hepatitis A virus infection may cause prolonged cholestatic severe jaundice in a patient with preexisting congenital hemolytic disease. Steroid therapy may induce early recovery.

Atypical presentations of hepatitis A infection in children

Background Hepatitis A is the most common cause of acute viral hepatitis, with a typical simple, self-limiting course. But it is not free from complications. Atypical presentations, such as in the form of prolonged cholestasis, ascites, pleural effusion, relapsing hepatitis, or fulminant hepatic failure, pose challenges to disease management. Knowledge about varying presentations and identification of factors associated with atypical presentations will help to early diagnosis of atypical courses of disease.&#x0D; Objective To describe various atypical clinical presentations, biochemical findings of hepatitis A infection, and possible related factors.&#x0D; Methods Ninety-five children aged 1 to 18 years, diagnosed with hepatitis A infection, and admitted to the Department of Pediatric Gastroenterology &amp; Nutrition, BSMMU, Dhaka, Bangladesh from January 2015 to May 2018 were studied retrospectively.&#x0D; Results Atypical presentations were manifested in 19 (20%) out of 95 children with hepatitis A virus (HAV) infection. The mean age of atypical patients [6.32 (SD 3.45) years] was significantly lower than that of typical patients [8.22 (SD 3.58) years] (P=0.0041). The most common atypical manifestation was ascites (11/19), followed by hepatic encephalopathy (9/19), acute liver failure (719), thrombocytopenia (2/19), pleural effusion (2/19), and cholestasis (1; 1.1%). Children with atypical features had significantly higher international normalized ratio (INR) and serum bilirubin, as well as lower hemoglobin level than the typical group. Children of atypical group had significantly higher number of organomegaly and coagulopathy.&#x0D; Conclusion Ascites, hepatic encephalopathy, acute liver failure, thrombocytopenia, pleural effusion, and prolonged cholestasis were common forms of atypical presentation. Younger age, organomegaly, higher bilirubin level, prolonged PT, and decreased hemoglobin level could be predictive of an atypical presentation of HAV in children.

Secondary biliary cirrhosis of the liver: case from clinical practice

Presents a clinical case of a patient developing secondary biliary cirrhosis due to prolonged extrahepatic cholestasis caused by cholelithiasis and the presence of choledocholithiasis, which is rare. The article describes the characteristics of secondary biliary cirrhosis of the liver. The course of the disease is considered, the data of laboratory and instrumental studies carried out on the patient in various clinics are analyzed.

S2659 SARS-CoV-2-Associated Cholestatic Liver Injury: A Case Series in Critically Ill Patients

Introduction: Hepatocellular injury has been documented in 14% to 76% of patients suffering from Coronavirus Disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is associated with increased rates of intubation and mortality. SARS-CoV-2 enters target cells by binding to angiotensin-converting enzyme 2 present in the lungs, small intestine, cholangiocytes, and hepatocytes. While most patients experience mild acute liver injury, some critically ill patients develop a prolonged cholestatic injury pathologically similar to secondary sclerosing cholangitis. Histologic assessments have demonstrated microvesicular steatosis, diffuse intrahepatic vascular thrombosis, and chronic fibrous endothelial thickening. We present five cases of cholestatic liver injury in critically ill patients, including a novel case of severe hepatic arteriopathy during endoscopic retrograde cholangiopancreatography (ERCP). Case Description/Methods: We identified 5 patients without preexisting liver disease who developed prolonged cholestatic liver injury following severe COVID-19 infection requiring mechanical ventilation, antibiotics, and prolonged hospitalization (Table 1). Four patients developed shock requiring vasopressor support and two required extracorporeal membrane oxygenation. Additionally, Patient 1 underwent ERCP which revealed friable hepatic parenchyma and vascularture that appeared angiographically similar to secondary sclerosing cholangitis. While liver chemistries were normal or mildly elevated at the time of COVID-19 diagnosis, all patients experienced significant cholestasis with predominantly elevated alkaline phosphatase levels ranging from the 900s to 4000s. In patients who survived their initial hospitalization, liver chemistries remained elevated months beyond resolution of their pulmonary and renal injuries, and none had returned to baseline as of this writing. Discussion: We described several cases of prolonged cholestasis in patients with severe COVID-19 infection without preexisting liver disease. While a component of this acute liver injury could be attributed to drugs or ischemia, the timeframe and degree of injury suggest a different etiology, which we postulate may be a combination of secondary sclerosing cholangitis and direct hepatic injury from SARS-CoV-2. Additional research and longitudinal monitoring of these patients is needed to better characterize this type of injury and determine the risk of clinically relevant, long-term liver dysfunction.Table 1-A.: Patient Characteristics (1) COVID-19, Coronavirus Disease 2019 (2) ARDS, Acute Respiratory Distress Syndrome.Table 1-B.: Patient Characteristics (1) COVID-19, Coronavirus Disease 2019 (2) ARDS, Acute Respiratory Distress Syndrome.

Питання терапії біліарної патології у дітей: вибір оптимального рішення

Актуальність. Захворювання гепатобіліарної системи — одна з найбільш поширених проблем педіатричної гастроентерології. Захворювання біліарного тракту (БТ) супроводжуються стійкими змінами обміну ліпідів, що сприяє тривалому холестазу і прогресуванню біліарної патології. Мета дослідження — вивчити ефективність застосування препарату Урсофальк® у комплексній терапії захворювань БТ і коморбідних станів у дітей. Матеріали та методи. У міському гастроентерологічному відділенні м. Харкова обстежено 55 дітей віком від 5 до 18 років (31 дівчинка та 24 хлопчика). Критерії включення пацієнтів у дослідження: захворювання БТ, що супроводжуються біліарним сладжем (БС) і порушенням ліпідного профілю (ЛП) сироватки крові. Діагностичний комплекс включав: аналіз клінічних даних, УЗД органів черевної порожнини, біохімічне дослідження сироватки крові («печінкові проби», ЛП). Усі діти отримували комплексне лікування з урахуванням коморбідної патології. Тривалість курсу терапії — 1 місяць. Пацієнти були розділені на дві групи залежно від жовчогінної терапії: 1-ша (основна) група отримувала традиційні жовчогінні засоби, 2-га (група порівняння) — препарат Урсофальк®. При статистичній обробці використані ліцензовані програмні продукти (Statistica, Excel). Результати. Клінічні прояви у дітей груп спостереження були представлені больовим і диспептичним синдромами. У всіх обстежених виявлено коморбідні стани і наявність БС до початку лікування. Після закінчення курсу лікування відзначалася позитивна динаміка клінічних симптомів, лабораторних показників і гомогенності структури жовчі. Застосування в комплексній терапії Урсофальку® дозволило повністю компенсувати відчуття тяжкості та іррадіацію болю, тоді як у дітей групи порівняння зберігалися больовий синдром, тяжкість у правому підребер’ї, диспептичні скарги. Слід зазначити, що застосування Урсофальку® дозволяє також нормалізувати структуру жовчі і біохімічні показники, що характеризують ліпідний обмін. Зареєстровано вірогідно (p &lt; 0,05) більш виражений ефект зменшення частоти порушень гомогенності жовчі в основній групі — на 65,5 % порівняно з групою порівняння — 27,3 %. Позитивна динаміка біохімічних показників при контрольному обстеженні відзначалася в обох групах пацієнтів, при цьому вірогідно (p &lt; 0,05) більш вираженими були зміни в групі дітей, які приймали Урсофальк®. Висновки. Застосування урсодезоксихолевої кислоти (УДХК) патогенетично обґрунтоване для корекції функціональних, метаболічних порушень при біліарній патології та коморбідних станах, що підтверджують результати спостереження. При призначенні Урсофальку® в комплексній терапії захворювань жовчовивідної системи у дітей відзначається швидка регресія і купіювання клінічних симптомів, нормалізація лабораторно-інструментальних показників. Урсофальк® є референтним препаратом УДХК. Наявність офіцінальної форми препарату Урсофальк® у спеціальній лікарській формі для дітей у вигляді суспензії дозволяє застосовувати УДХК в дитячому віці, починаючи з перших днів життя.

Secondary sclerosing cholangitis as cause of persistent jaundice in patients with severe COVID-19.

Background & AimsLittle is known about cholestasis, including its most severe variant secondary sclerosing cholangitis (SSC), in critically ill patients with coronavirus disease 19 (COVID‐19). In this study, we analysed the occurrence of cholestatic liver injury and SSC, including clinical, serological, radiological and histopathological findings.MethodsWe conducted a retrospective single‐centre analysis of all consecutive patients admitted to the intensive care unit (ICU) as a result of severe COVID‐19 at the University Hospital Zurich to describe cholestatic injury in these patients. The findings were compared to a retrospective cohort of patients with severe influenza A.ResultsA total of 34 patients with severe COVID‐19 admitted to the ICU were included. Of these, 14 patients (41%) had no cholestasis (group 0), 11 patients (32%, group 1) developed mild and 9 patients (27%, group 2) severe cholestasis. Patients in group 2 had a more complicated disease course indicated by significantly longer ICU stay (median 51 days, IQR 25‐86.5) than the other groups (group 0: median 9.5 days, IQR 3.8‐18.3, P = .001; and group 1: median 16 days, IQR 8‐30, P < .05 respectively). Four patients in group 2 developed SSC compared to none in the influenza A cohort. The available histopathological findings suggest an ischaemic damage to the perihilar bile ducts.ConclusionsThe development of SSC represents an important complication of critically ill COVID‐19 patients and needs to be considered in the diagnostic work up in prolonged cholestasis. The occurrence of SSC is of interest in the ongoing pandemic since it is associated with considerable morbidity and mortality.

Neonatal Dubin-Johnson syndrome: biochemical parameters, characteristics, and genetic variants study.

The clinical characteristics and gene mutation characteristics of children with Dubin-Johnson syndrome (DJS) need in-depth study. The clinical and genomic data of neonatal Dubin-Johnson syndrome (NDJS) and 155 cases with idiopathic cholestasis (IC) were analyzed from June 2016 to August 2020 RESULTS: ABCC2 gene variants were identified in eight patients, including one patient with homozygous variants and seven patients with compound heterozygous variants. A total of 13 different ABCC variants were detected in the NDJS patients, including three nonsense variants, six missense variants, three frameshift variants, and a splice site variant. The variant c.2443C > T (p.R815X), c.4237_4238insCT (p.H1414Lfs*17), c.960_961insGT (p.L322Cfs*3), c.4250delC (p.S1417Ffs*14), c.2224G > A (p.D742N), c.4020G > C (p.K1340N), and c.2439 + 5G > A were not reported in the Human Gene Variant Database. There was no significance in the sex, birth weight, and onset age between the NDJS and IC groups. Compared with the IC group, the NDJS group had significantly higher levels of total bilirubin (TB), but a significantly lower level of alanine transaminase and a ratio of direct bilirubin (DB) to TB. There is no significance in total bile acid, gamma-glutamyl-transpeptidase, albumin, or international normalized ratio between the two groups. NDJS should be considered in prolonged neonatal intrahepatic cholestasis, especially in infants with normal or slightly elevated transaminase levels. Explore the biochemical parameters, characteristics, and genetic profile of NDJS. By summarizing the characteristics of biochemical indicators, seven new mutation types of the ABCC2 gene were detected, which expanded the mutation spectrum of the ABCC2 gene. NDJS should be considered in prolonged neonatal intrahepatic cholestasis, especially in infants with normal or slightly elevated transaminase levels.

The new era of endoscopic ultrasound in biliary disorders.

Biliary obstruction is one of challenging biliary disorders in gastroenterology field, where this long-standing condition can also lead to portal hypertension and multi-disciplinary teamwork is usually needed to manage this problem. Biliary drainage is the primary management to prevent prolonged cholestasis. Biliary system with its thin-walled and tubular structure sometimes makes the diagnosis and therapeutic not easy to approach. Over the past 3 decades, numerous new and modern diagnostic and therapeutic modalities have been developed to manage the complex biliary problems. It is well known that endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic biliary drainage (PTBD), and surgical procedure are common procedures in managing biliary disorders. However, surgical therapy and ERCP are not always easy to perform due to several contraindications. Because of difficulty in performing these procedures or unavailability of these procedures in the facility, PTBD, as the primary non-surgical procedure of choice, has been popular due to its easy technique. Endoscopic ultrasound (EUS) has evolved significantly not only as a diagnostic tool for identification and staging, but also for interventional approaches, especially in management of biliary malignancy. Recently, EUS-guided biliary drainage (EUS-BD) and EUS-guided gallbladder drainage (EUS-GBD) are developed for managing biliary disorders. Whether EUS can be useful for managing biliary obstruction as a primary procedure is still controversial. Hence, a large number of further studies are required to validate.