Portal hypertension in doublecortin domain-containing protein 2 (DCDC2) related neonatal sclerosing cholangitis.

Mutations in doublecortin domain-containing protein 2 (DCDC2) lead to neonatal sclerosing cholangitis (NSC), and portal hypertension (PHTN). The objective of the study was to systematically evaluate PHTN, variceal bleeding, and outcomes of patients with DCDC2-related NSC. The study included children with homozygous or compound heterozygous variants in DCDC2.

Injectable Hydrogels for Liver: Potential for Clinical Translation.

Injectable hydrogels are a sub-type of hydrogels which can be delivered into the host in a minimally invasive manner. They can act as carriers to encapsulate and deliver cells, drugs or active biomolecules across several disease conditions. Polymers, either synthetic or natural, or even a combination of the two, can be used to create injectable hydrogels.

The histological grading of fibrosis in Budd-Chiari syndrome: A chronic liver disease, different from others.

Introduction: Budd-Chiari syndrome (BCS) is an uncommon disease caused by hepatic venous outflow obstruction. They can result in centrilobular fibrosis, nodular regenerative hyperplasia and cirrhosis. Assessing liver fibrosis is crucial for determining the stage of BCS, predicting disease progression and guiding treatment decisions.

Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management.

Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy.

Pre-transplant Serum Procalcitonin as a Predictor of Early Post-transplant Sepsis and Mortality After Living Donor Liver Transplantation: A Prospective Observational Study.

Purpose: The early post-transplant period after liver transplantation is critical, as recipients are highly susceptible to sepsis due to their immune-compromised state. This study aimed to identify the association between preoperative procalcitonin and early post-transplant sepsis and mortality at one month after living donor liver transplantation (LDLT).

Methodology: All patients who underwent LDLT from July 2021 to December 2021 were recruited prospectively.

Diffusion weighted MRI and neutrophil lymphocyte ratio non-invasively predict infection in pancreatic necrosis: a pilot study.

Background: Infected pancreatic necrosis (IPN) is a major determinant of mortality in acute pancreatitis (AP). Non-invasive diagnosis of IPN could guide the intervention in AP. We aimed to investigate the role of non-invasive methods like diffusion weighted magnetic resonance imaging (DW-MRI) and clinico-laboratory parameters as predictors of IPN.

Clinico-Pathological Spectrum of Hepatitis A Virus-Induced Autoimmune-Like Hepatitis in Children.

There is limited evidence that hepatitis A virus (HAV) infection can trigger hepatic autoimmunity, but this area remains largely unexplored. This study was thus planned with the aim to compare HAV-induced autoimmune-like hepatitis (HAV-ALH) with HAV-related liver dysfunction (HAV-acute viral hepatitis or HAV-AVH) and classical autoimmune hepatitis (AIH). This was a retrospective review of 46 patients with HAV infection who underwent liver biopsy (including 17 cases of HAV-ALH: diagnosis based on histopathology), and they were compared to 46 cases of age- and gender-matched classical AIH.

Non-alcoholic fatty liver disease (NAFLD) integration into India's NCD program - Obstacles and solutions for the implementation of guidelines at the national level.

Individuals who have non-alcoholic fatty liver disease (NAFLD) are at high risk of metabolic comorbidities and can put a significant strain on healthcare systems. If not managed in a timely manner, NAFLD can lead to sustained healthcare costs, economic losses, and reduced health-related quality of life. In India, the Ministry of Health and Family Welfare recognized the need for a comprehensive approach to prevent NAFLD and integrated public health measures into the existing National Program for Prevention and Control of Non-Communicable Diseases (NP NCD).

Re-emerging : A cause of concern for global health.

 (CHPV) is an emerging neurotropic virus primarily affecting children and causing acute encephalitis syndrome (AES) in India. The virus, transmitted mainly by sand flies, has led to multiple outbreaks with high mortality rates, particularly in rural and resource-limited settings. CHPV infection is characterized by rapid disease progression, with symptoms ranging from fever and seizures to coma and death, often within 24 to 48 h of onset.

Natural course and outcomes of children with ubiquitin-specific protease 53 (USP53)-related genetic chronic cholestasis.

Ubiquitin-specific protease 53 (USP53) is essential for formation of cellular tight junctions and variations in this gene disrupt the tight junctions, resulting in cholestasis. We describe the clinical manifestations and outcomes of patients with USP53 mutations from the Indian progressive familial intrahepatic cholestasis registry. All 29 patients who harbored mutations in the USP53 gene either in the homozygous, compound heterozygous, or heterozygous state and presented with cholestasis were included.

Early Prediction of Acute Kidney Injury in Living Donor Liver Transplantation by Serum Cystatin C Concentration at the End of the Surgery.

Introduction: Acute kidney injury (AKI) is a prevalent complication of liver transplantation, leading to prolonged hospital or intensive care unit stay and significant morbidity. Recently, biomarkers such as neutrophil gelatinase-associated lipocalin (NGAL) and cystatin C have been investigated for their potential role in the early detection of AKI in liver transplantation patients.

Method: Our study comprised 60 patients with end-stage liver disease undergoing living donor liver transplantation.

A Novel Approach to Identifying Hibernating Myocardium Using Radiomics-Based Machine Learning.

Background To assess the feasibility of a machine learning (ML) approach using radiomics features of perfusion defects on rest myocardial perfusion imaging (MPI) to detect the presence of hibernating myocardium. Methodology Data of patients who underwent 99mTc-sestamibi MPI and 18F-FDG PET/CT for myocardial viability assessment were retrieved. Rest MPI data were processed on ECToolbox, and polar maps were saved using the NFile PMap tool.

Safety and Efficacy of Dapagliflozin in Recurrent Ascites: A Pilot Study.

Background: In cirrhosis, activation of renin-angiotensin-aldosterone system leads to sodium and water retention causing ascites. Dapagliflozin, a sodium glucose linked transporter-2 inhibitor, induces natriuresis in patients with heart failure. A similar natriuretic effect may improve ascites in patients with cirrhosis.

Management of Portal vein Thrombosis in Cirrhosis.

Portal vein thrombosis (PVT) is one of the common complications of cirrhosis. The incidence of PVT correlates with liver disease severity-higher incidence in patients with Child-Turcotte-Pugh (CTP) C, large spontaneous portosystemic shunts, hepatofugal portal flow, and in the presence of hepatocellular carcinoma. PVT may worsen ascites, increase the risk and poor control of variceal bleeding.