Primary Ewing's Sarcoma affecting the Central Nervous System: A single-center experience and Narrative review.

Background And Objectives: Ewing's sarcoma (EWS) is a malignant round-cell tumor arising from the bone and soft tissue. It is a disease of children and young adults. EWS affecting the central nervous system (cranial and spinal column) is relatively rare with an annual incidence of approximately one case per million in the Western population.

Therapeutic Plasma Exchange in Hepatology: Indications, Techniques, and Practical Application.

It is sobering that many liver failure patients die in the absence of liver transplantation (LT), and reducing its morbidity and mortality urgently needs more non-transplant treatment options. Among the several artificial liver support devices available, therapeutic plasma exchange (TPE) is the only one that improves survival in acute liver failure (ALF) patients. In many other disorders, data on survival benefits and successful bridging to transplant is encouraging.

Improved outcomes of ABO-incompatible living donor liver transplant with biologically related donors.

Liver transplantation is considered to be the only curative treatment for decompensated liver disease. Shortage of liver allografts is a major impediment to the widespread application of this procedure. ABO-incompatible (ABO-I) grafts have been used successfully, thereby increasing the living donor liver transplantation (LDLT) donor pool.

Therapeutic and pharmacological efficacy of plant-derived bioactive compounds in targeting breast cancer.

Breast cancer (BC) ranks number one among cancers affecting women globally. Serious concerns include delayed diagnosis, poor prognosis, and adverse side effects of conventional treatment, leading to residual morbidity. Therefore, an alternative treatment approach that is safe and effective has become the need of the hour.

Youngest Living Donor Liver Transplant for End-Stage Liver Disease in a 6-Month-Old With a Novel Aggressive Mutation in KIF12 Gene.

Background: Kinesin family member 12 (KIF12) mutation-related cholestatic disorder represents a rare subtype of progressive familial intrahepatic cholestasis (PFIC), referred to as PFIC Type 8, with only 21 reported cases globally to date.

Methods: Here, we present a unique case of a 6-month-old boy diagnosed with homozygous KIF12 gene mutation, who successfully underwent a living donor liver transplant at our center for end-stage liver disease.

Results: This case marks the youngest patient of KIF12-related cholestatic disorder necessitating a liver transplant to date.

Successful Surgical Management of Giant Mucinous Cystic Neoplasm of Liver (MCN-L) Presenting With Peritoneal Rupture and Biliary Prolapse: Case Report and Review of Literature.

Mucinous cystic neoplasms of liver (MCN-L) are generally considered benign indolent cystic liver lesions, not associated with significant clinical symptoms in majority of patients. However, rarely these benign-appearing lesions may have a complicated clinical course, presenting with jaundice, acute abdomen, or malignant transformation. We report one such rare clinical presentation of MCN-L presenting with obstructive jaundice and abdominal pain due to prolapse of cystic component in biliary system and peritoneal rupture occurring simultaneously.

Hepatitis A Liver Failure in Children: Native Liver Survival Despite Poor Prognosis.

Background: Hepatitis A virus (HAV) infection is the commonest cause of pediatric acute liver failure (PALF) in developing countries. Literature has shown good outcomes of HAV-induced PALF as compared to other etiologies. The advanced critical care and use of extracorporeal liver assist devices (ELAD) have improved the survival with native liver in PALF and overall outcomes.

Basics and Art of Immunosuppression in Liver Transplantation.

Liver transplantation is one of the most challenging areas in the medical field. Despite that, it has already been established as a standard treatment option, especially in decompensated cirrhosis and selected cases of hepatocellular carcinoma and acute liver failure. Complications due to graft rejection, including mortality and morbidity, have greatly improved over time due to better immunosuppressive agents and management protocols.

Genotype correlates with clinical course and outcome of children with tight junction protein 2 (TJP2) deficiency-related cholestasis.

Background And Aims: The study aimed to describe the clinical course and outcomes, and analyze the genotype-phenotype correlation in patients with tight junction protein 2 (TJP2) deficiency.

Approach And Results: Data from all children with chronic cholestasis and either homozygous or compound heterozygous mutations in TJP2 were extracted and analyzed. The patients were categorized into 3 genotypes: TJP2-A (missense mutations on both alleles), TJP2-B (missense mutation on one allele and a predicted protein-truncating mutation [PPTM] on the other), and TJP2-C (PPTMs on both alleles).

MYCN immunohistochemistry as surrogate marker for MYCN-amplified spinal ependymomas.

MYCN (master regulator of cell cycle entry and proliferative metabolism) gene amplification defines a molecular subgroup of spinal cord ependymomas that show high-grade morphology and aggressive behavior. Demonstration of MYCN amplification by DNA methylation or fluorescence-in situ hybridization (FISH) is required for diagnosis. We aimed to (i) assess prevalence and clinicopathological features of MYCN-amplified spinal ependymomas and (ii) evaluate utility of immunohistochemistry (IHC) for MYCN protein as a surrogate for molecular testing.

ABO Incompatible Living Donor Liver Transplantation in Children: A Single Centre Experience from India.

Background: In recent years, paediatric ABO incompatible (ABOi) living donor liver transplant (LT) has shown promising outcomes and can potentially eliminate organ shortage. This study aims to report paediatric ABOi LT experience, including short- and long-term outcomes.

Methods: It is a single-centre retrospective study.

Metabolic imaging in recurrent gliomas: comparative performance of 18F-FDOPA, 18F-fluorocholine and 18F-FDG PET/CT.

Purpose: The aim of this study was to directly evaluate glucose, amino-acid and membrane metabolism in tumor cells for diagnosis and prognostication of recurrent gliomas.

Methods: Fifty-five patients (median age = 36 years; 33 men) with histologically proven gliomas and suspected recurrence were prospectively recruited and underwent 18F-FDG (Fluorodeoxyglucose), 18F-FDOPA (fluorodopa) and 18F-Fluorocholine-PET/CT. Images were evaluated by two physicians visually and quantitatively [lesion-SUVmax, tumor (T) to gray-matter (G) and metabolically-active tumor volumes (MTV)].

Delayed diagnosis of bronchobiliary fistula complicating a pediatric living donor liver transplantation: a case report.

Bronchobiliary fistula (BBF) is a very rare condition in children. Only a few pediatric BBF cases have been reported, in the context of a ruptured hydatid cyst or liver abscess. BBF after living donor liver transplantation (LDLT) has not been reported in the pediatric literature.

Domino liver transplantation for maple syrup urine disease in children: A single-center case series.

Background: Domino liver transplant (DLT) represents another type of liver donor to expand the donor pool. Recent reports of successful DLT in children with maple syrup urine disease (MSUD) show promising long-term outcomes.

Methods: It was a retrospective study.

Living donor liver transplant in acute on chronic liver failure grade 3: Who not to transplant.

Background And Aim: Liver transplantation(LT)offers definitive treatment for acute on chronic liver failure(ACLF) patients. This study was done to analyze and compare the outcomes of living donor LT(LDLT) in patients with ACLF versus Chronic liver disease(CLD) and within the grades of ACLF. Factors affecting mortality in patients with ACLF and ACLF grade3 (ACLF3) following LDLT were also derived.

Hypo-fractionated accelerated radiotherapy with concurrent and maintenance temozolomide in newly diagnosed glioblastoma: updated results from phase II HART-GBM trial.

Background: Glioblastoma (GBM) patients have poor survival outcomes despite treatment advances and most recurrences occur within the radiation field. Survival outcomes after dose escalation through hypofractionated accelerated RT(HART) were evaluated in this study. We previously reported the study's initial results showing similar survival outcomes with acceptable toxicities.