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.

Diagnosis and management of pediatric acute liver failure: consensus recommendations of the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ISPGHAN).

Timely diagnosis and management of pediatric acute liver failure (PALF) is of paramount importance to improve survival. The Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition invited national and international experts to identify and review important management and research questions. These covered the definition, age appropriate stepwise workup for the etiology, non-invasive diagnosis and management of cerebral edema, prognostic scores, criteria for listing for liver transplantation (LT) and bridging therapies in PALF.

Data from the Indian drug regulator and from Clinical Trials Registry-India does not always match.

Introduction: In India, regulatory trials, which require the drug regulator's permission, must be registered with the Clinical Trials Registry-India (CTRI) as of 19 March 2019. In this study, for about 300 trials, we aimed to identify the CTRI record that matched the trial for which the regulator had given permission. After identifying 'true pairs', our goal was to determine whether the sites and Principal Investigators mentioned in the permission letter were the same as those mentioned in the CTRI record.

An Approach to Investigations of Chronic Liver Disease.

Chronic liver disease (CLD) in children is more diverse compared to adults with respect to the etiology, progression and response to therapy. After history and clinical examination, the first step is to confirm the presence of CLD with basic blood investigations and ultrasonography. Markers of portal hypertension are splenomegaly, increased portal vein diameter, thrombocytopenia and presence of varices on endoscopy.

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.

CAR-T cell therapy in India requires a paradigm shift in training, education and health care processes.

Chimeric antigen receptor (CAR)-T cell therapy has revolutionized the treatment of some kinds of cancers. Hundreds of companies and academic institutions are collaborating to develop gene-modified cell therapies using novel targets, different cell types, and manufacturing processes of autologous and allogenic cell therapies. The individualized, custom-made autologous CAR-T cell production platform remains a significant limiting factor for its large-scale clinical application.

The Increasing Economic Burden with Additional Steps of Pharmacotherapy in Major Depressive Disorder.

Objectives: Major depressive disorder (MDD) is a common and serious disorder with significant impact on patients and families. The goal of this retrospective cohort study was to determine the economic burden among patients with MDD stratified by number of treatment lines needed for episode resolution.

Methods: Truven Health Analytics MarketScan claims data were used to identify US patients (≥ 18 years) who were diagnosed with MDD and started on an antidepressant between 2013 and 2017.

Portal Cavernoma Cholangiopathy in Children and the Management Dilemmas.

Portal cavernoma cholangiopathy (PCC) is one of the most harrowing complications of extrahepatic portal venous obstruction, as it determines the long-term hepatobiliary outcome. Although symptomatic PCC is rare in children, asymptomatic PCC is as common as that in adults. However, there are major gaps in the literature with regard to the best imaging strategy and management modality in children.

Childhood pancreatic trauma: Clinical presentation, natural history and outcome.

Objectives: To study the presentation, management strategies and long-term natural history of children with pancreatic trauma.

Methods: Children admitted with pancreatic trauma were analyzed for their presentation, management and outcome. Management included nasojejunal feeds, total parenteral nutrition (TPN), octreotide, drainage (radiological and endoscopic), endoscopic retrograde cholangiopancreatography (ERCP) and surgery.

Outcome of portosystemic shunt surgery on pre-existing cholangiopathy in children with extrahepatic portal vein obstruction.

Background: This study was performed to assess the effect of portosystemic shunt surgery (PSS) on portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal vein obstruction (EHPVO).

Methods: Children with EHPVO and PCC (unfit for Meso-Rex shunt) underwent magnetic resonance cholangiogram (MRC) and magnetic resonance portovenogram (MRPV) before non-selective PSS. PCC was graded by modified Llop classification.

Peritoneal Lymphomatosis Masquerading as Pyoperitoneum in a Teenage Boy.

A 16-year-old boy presented with 1 month of fever, abdominal pain, and distension. The ascitic tap drained pus-like fluid, and ultrasonography showed diffuse thickening of the omentum and mesentery with echogenic ascites. A diagnosis of pyoperitoneum due to peritoneal tuberculosis with secondary infection was suspected, and antitubercular therapy was started elsewhere, but there was no improvement.