Unusual association of cirrhosis and sickle cell anemia revealed by hemolytic anemia: A case report

Hepatobiliary complications of sickle cell disease are rare, cirrhosis remains very exceptional, especially in heterozygous forms of the disease. We report the case of a 19-year-old patient whose etiologic investigation of hemolytic anemia revealed heterozygous sickle cell disease complicated by hepatic cirrhosis. The diagnosis of cirrhosis was made due to the presence of signs of hepato-cellular insufficiency, portal hypertension syndrome and hepatic dysmorphia on imaging. The etiological assessment was negative. The liver biopsy was not performed due to the risk of bleeding. The interest of this observation is to evoke hepato-biliary complications (in particular cirrhosis) in patients with sickle cell anemia, in order to avoid a pejorative evolution burdened with serious complications.

Review of ERCP Techniques in Roux-en-Y Gastric Bypass Patients: Highlight on the Novel EUS-Directed Transgastric ERCP (EGDE) Technique

Purpose of Review Hepatobiliary complications are common in Roux-en-Y gastric bypass (RYGB) patients. Despite development of multiple surgical and endoscopic access techniques over the years, ERCP using standard duodenoscope remains challenging in these patients due to the altered anatomy. Recent Findings Limited success with enteroscope-assisted and laparoscope-assisted ERCP led to the evolution of the novel EUS-directed transgastric ERCP (EDGE) procedure, with variations of this technique termed as Gastric Access Temporary for Endoscopy (GATE), EUS-guided TransGastric ERCP (EUS-TG-ERCP), EUS-guided GastroGastrostomy-assisted ERCP (EUS-GG-ERCP), and EUS-directed transgastric intervention (EDGI). EDGE has high technical (100%) and clinical success rates (60–100%), lower adverse event rate (1.5–7.6%), and up to 20% access stent migration rate; without any significant weight changes. EDGE has significantly shorter procedure time (73vs184min), post-procedural hospital stays (0.8vs2.65 days) and is more cost effective compared to other modalities. Summary EDGE technique addresses the challenges of RYGB anatomy as a minimally invasive, clinically successful, fully endoscopic, and cost-effective option. We present a literature review of the EDGE technique from its inception to current, in addition to reviewing other access techniques, their advantages, disadvantages and outcomes.

The Overactivation of NADPH Oxidase during Clonorchis sinensis Infection and the Exposure to N-Nitroso Compounds Promote Periductal Fibrosis

Clonorchis sinensis, a high-risk pathogenic human liver fluke, provokes various hepatobiliary complications, including epithelial hyperplasia, inflammation, periductal fibrosis, and even cholangiocarcinogenesis via direct contact with worms and their excretory–secretory products (ESPs). These pathological changes are strongly associated with persistent increases in free radical accumulation, leading to oxidative stress-mediated lesions. The present study investigated C. sinensis infection- and/or carcinogen N-nitrosodimethylamine (NDMA)-associated fibrosis in cell culture and animal models. The treatment of human cholangiocytes (H69 cells) with ESPs or/and NDMA increased reactive oxidative species (ROS) generation via the activation of NADPH oxidase (NOX), resulting in augmented expression of fibrosis-related proteins. These increased expressions were markedly attenuated by preincubation with a NOX inhibitor (diphenyleneiodonium chloride) or an antioxidant (N-acetylcysteine), indicating the involvement of excessive NOX-dependent ROS formation in periductal fibrosis. The immunoreactive NOX subunits, p47phox and p67phox, were observed in the livers of mice infected with C. sinensis and both infection plus NDMA, concomitant with collagen deposition and immunoreactive fibronectin elevation. Staining intensities are proportional to lesion severity and infection duration or/and NDMA administration. Thus, excessive ROS formation via NOX overactivation is a detrimental factor for fibrogenesis during liver fluke infection and exposure to N-nitroso compounds.

A case of HIV associated cholangiopathy (CAS) - A case report

Diseases of the liver and biliary tree have been described with significant frequency among patients with human immunodeficiency virus (HIV), and its advanced state, acquired immunodeficiency syndrome (AIDS). Through a variety of mechanisms, HIV/AIDS has been shown to affect hepatic parenchyma and biliary tree, leading to liver inflammation and biliary strictures. One of the potential hepatobiliary complications of this viral infection is AIDS cholangiopathy, a syndrome of biliary obstruction and liver damage due to infection-related strictures of the biliary tract. AIDS cholangiopathy is highly associated with opportunistic infections and advanced immunosuppression in AIDS patients, and due to the increased availability of highly active antiretroviral therapy, is now primarily seen in instances of poor access to anti-retroviral therapy and medication non-compliance. Current published literature describes well the clinical, biochemical, and endoscopic management of AIDS-related cholangiopathy. We present a case of 23 year male, presented with the complains of jaundice and severe abdominal pain associated with vomiting which on further investigations and imaging work up turned out to be AIDS cholangiopathy.

A study of hepatobiliary involvement in adult patients with sickle cell disease

Background: Sickle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by a single amino acid substitution in the ß-globin chain. Hepatobiliary complications are frequent among sickle cell disease patients. Sickle cell disease has been extensively studied. However, data about hepatobiliary abnormalities among the adult age group are limited. Aim of our study aims to find the prevalence of hepatic-biliary involvement in Sickle cell disease in adult patients admitted to our hospital.Methods: A prospective study was done for a period of two years from October 2017 to October 2019. Subjects of both sexes above the age of 18 years with SCD admitted to our hospital were enrolled. Thorough history taking, full clinical examination, hematological and biochemical parameters assessment, and abdominal ultrasonographic studies were performed to all patients.Results: The results obtained showed that 59% of patients had hepatobiliary involvement. The most common symptom among these patients was bone pain, and the pallor was the most common sign. Biochemical tests revealed reduced hemoglobin concentration, elevated bilirubin, and compromised liver function. The most common ultrasound finding in this study was hepatomegaly, hepatosplenomegaly, cholelithiasis, and gall bladder sludge. The incidence of viral hepatitis was low compared to previous studies. Other hepatobiliary complications were cholelithiasis (14%), benign hyperbilirubinemia (14%), cholecystitis (8%) hepatic crisis (9%), hepatic sequestration (1%) , liver cirrhosis (1%), choledocholithiasis (1%) and cholangitis (1%).Conclusions:Hepatobiliary complications, particularly gallbladder diseases, are frequent among SCD patients. The early detection of hepatobiliary complications by repeated ultrasound screening and liver function tests is significant as their frequency and intensity are related to the patient's age and the duration.

Cholangioscopy and its Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterised by chronic inflammation and fibro-obliteration of the intrahepatic and/or extrahepatic bile ducts. It is associated with numerous hepatobiliary complications including an increased risk of malignancy (in particular, cholangiocarcinoma) and biliary tract stone formation. The evaluation of biliary strictures in patients with PSC is especially challenging, with imaging and endoscopic methods having only modest sensitivity for the diagnosis of cholangiocarcinoma, and treatment of biliary strictures poses a similarly significant clinical challenge. In recent years, peroral cholangioscopy has evolved technologically and increased in popularity as an endoscopic tool that can provide direct intraductal visualisation and facilitate therapeutic manipulation of the biliary tract. However, the indications for and effectiveness of its use in patients with PSC remain uncertain, with only a few studies performed on this small but important subset of patients. In this review, the authors discuss the available data regarding the use of peroral cholangioscopy in patients with PSC, with a focus on its use in the evaluation and management of biliary strictures and stones.

Liver injury in COVID-19: The current evidence

Patients with novel coronavirus disease 2019 (COVID-19) experience various degrees of liver function abnormalities. Liver injury requires extensive work-up and continuous surveillance and can be multifactorial and heterogeneous in nature. In the context of COVID-19, clinicians will have to determine whether liver injury is related to an underlying liver disease, drugs used for the treatment of COVID-19, direct effect of the virus, or a complicated disease course. Recent studies proposed several theories on potential mechanisms of liver injury in these patients. This review summarizes current evidence related to hepatobiliary complications in COVID-19, provides an overview of the available case series and critically elucidates the proposed mechanisms and provides recommendations for clinicians.