Adipocyte lipin 1 expression associates with human metabolic health and regulates systemic metabolism in mice.

Dysfunctional adipose tissue is believed to promote the development of hepatic steatosis and systemic insulin resistance, but many of the mechanisms involved are still unclear. Lipin 1 catalyzes the conversion of phosphatidic acid to diacylglycerol, the penultimate step of triglyceride synthesis, which is essential for lipid storage. Herein we found that adipose tissue LPIN1 expression is decreased in people with obesity compared with lean subjects, and low LPIN1 expression correlated with multi-tissue insulin resistance and increased rates of hepatic de novo lipogenesis.

Worse fibro-inflammatory activity on diagnostic liver biopsy adversely impacts biochemical remission in autoimmune hepatitis.

Background: Autoimmune hepatitis (AIH) patients can present with advanced fibrosis at diagnosis or may progress to the same if biochemical remission on treatment is not achieved.

Methods: We conducted a single-center retrospective analysis of 34 pediatrics and 39 adult AIH patients. Three pathologists, blinded to clinical information, reviewed the diagnostic liver biopsy (DLB) slides of AIH patients.

Immune checkpoint markers and tumour mutation burden in Wilms tumour: a study of 59 cases.

Wilms tumour (WT) is the most common renal tumour in children, and studies of immune checkpoint inhibitors (ICIs) treatment and markers are limited in number. In this study we investigated the ICIs' related immune landscape by examining the expression of PD-L1, PD-1, CD8 and DNA mismatch repair (MMR) proteins by immunohistochemistry (IHC), tumour mutation burden (TMB), and correlations with histology and clinical outcome. Positive PD-L1 (SP263) expression was defined as modified combined positive score (CPS) ≥1.

Noninvasive Markers of Inflammation and Protein Loss Augment Diagnosis of Pediatric Celiac Disease.

Introduction: Circulating tissue transglutaminase immunoglobulin A concentration is a sensitive and specific indicator of celiac disease, but discrepancies between serologic and histologic findings occur. We hypothesized that fecal markers of inflammation and protein loss would be greater in patients with untreated celiac disease than in healthy controls. Our study aims to evaluate multiple fecal and plasma markers in celiac disease and correlate these findings with serologic and histologic findings as noninvasive means of evaluating disease activity.

A Case of Congenital Nephrotic Syndrome with Crescents Caused by a Novel Compound Heterozygous Pairing of Genetic Variants.

Congenital nephrotic syndrome is an autosomal recessive inherited disorder that manifests as steroid-resistant massive proteinuria in the first three months of life. Defects in the glomerular filtration mechanism are the primary etiology. We present a child who developed severe nephrotic syndrome at two weeks of age and eventually required a bilateral nephrectomy.

Manual and Semi-Automated Measurement and Calculation of Osteosarcoma Treatment Effect Using Whole Slide Image and Qupath.

Introduction: In osteosarcoma, the most significant indicator of prognosis is the histologic changes related to tumor response to preoperative chemotherapy, such as necrosis. We have developed a method to measure the osteosarcoma treatment effect using whole slide image (WSI) with an open-source digital image analytical software Qupath.

Materials And Methods: In Qupath, each osteosarcoma case was treated as a project.

Complete Hydatidiform Mole with a Coexisting Viable Male Fetus Detected by Cell-Free DNA.

Complete hydatidiform mole with coexisting fetus (CHMCF) is rare, and diagnosis is challenging due to limited data. Here, we present the case of a patient with noninvasive prenatal test (NIPT) resulting in "likely molar pregnancy" in the second trimester. Subsequent ultrasound confirmed a cystic appearing portion of the placenta.

Observation and Modulation of High-Temperature Moiré-Locale Excitons in van der Waals Heterobilayers.

Transition metal dichalcogenide heterobilayers feature strong moiré potentials with multiple local minima, which can spatially trap interlayer excitons at different locations within one moiré unit cell (dubbed moiré locales). However, current studies mainly focus on moiré excitons trapped at a single moiré locale. Exploring interlayer excitons trapped at different moiré locales is highly desirable for building polarized light-emitter arrays and studying multiorbital correlated and topological physics.

Non-Invasive markers of inflammation and protein loss augment diagnosis of celiac disease.

Background: Circulating tissue transglutaminase IgA (TTG IgA) concentrations are sensitive and specific indicators of celiac disease, but discrepancies between serologic and histologic findings still occur. We hypothesized that fecal markers of inflammation and protein loss would be greater in patients with untreated celiac disease than in healthy controls. Our study aims to evaluate multiple fecal and plasma markers in celiac disease and correlate these findings with serologic and histologic findings as non-invasive means of evaluating disease activity.

The utility of TLE1 and BCOR as immunohistochemical markers for angiomatoid fibrous histiocytoma.

Objectives: Diagnosis of angiomatoid fibrous histiocytoma (AFH) can be challenging due to its variable histologic features and a lack of highly sensitive and/or specific immunohistochemical markers. The utility of TLE1 and BCOR as immunohistochemical markers for AFH is not known.

Methods: We examined the spectrum of histologic features of 36 AFHs, and studied the expression of both TLE1 and BCOR in AFH and its mimics by immunohistochemical staining.

A Broad Characterization of Glycogen Storage Disease IV Patients: A Clinical, Genetic, and Histopathological Study.

Glycogen storage disease type IV (GSD IV) is an ultra-rare autosomal recessive disease caused by variants in the gene, which encodes the glycogen branching enzyme (GBE). GSD IV accounts for approximately 3% of all GSD. The phenotype of GSD IV ranges from neonatal death to mild adult-onset disease with variable hepatic, muscular, neurologic, dermatologic, and cardiac involvement.

Mitochondrial Pyruvate Carrier Inhibition Attenuates Hepatic Stellate Cell Activation and Liver Injury in a Mouse Model of Metabolic Dysfunction-associated Steatotic Liver Disease.

Hepatic stellate cells (HSC) are non-parenchymal liver cells that produce extracellular matrix comprising fibrotic lesions in chronic liver diseases. Prior work demonstrated that mitochondrial pyruvate carrier (MPC) inhibitors suppress HSC activation and fibrosis in a mouse model of metabolic dysfunction-associated steatohepatitis (MASH). In the present study, pharmacologic or genetic inhibition of the MPC in HSC decreased expression of markers of activation .

Adipocyte lipin 1 is positively associated with metabolic health in humans and regulates systemic metabolism in mice.

Dysfunctional adipose tissue is believed to promote the development of hepatic steatosis and systemic insulin resistance, but many of the mechanisms involved are still unclear. Lipin 1 catalyzes the conversion of phosphatidic acid to diacylglycerol (DAG), the penultimate step of triglyceride synthesis, which is essential for lipid storage. Herein we found that adipose tissue expression is decreased in people with obesity compared to lean subjects and low expression correlated with multi-tissue insulin resistance and increased rates of hepatic de novo lipogenesis.

Sclerosing Epithelioid Fibrosarcoma of the Liver in a Pediatric Patient.

Sclerosing epithelioid fibrosarcoma (SEF) is a rare but aggressive sarcoma. We report the first case of hepatic SEF in pediatric patient, which is also the second case in literature. A 17-year-old previously healthy female presented with a liver mass measuring 13.

Protocol for doping of an Sn-based two-dimensional perovskite semiconductor by incorporating SnI for field-effect transistors and thermoelectric devices.

Doping is an important technique for semiconductor materials, yet effective and controllable doping of organic-inorganic halide perovskites is still a challenge. Here, we present a protocol to dope 2D perovskite (PEA)SnI by incorporating SnI in the precursor solutions. We detail steps for preparation of field-effect transistors (FETs) and thermoelectric devices (TEs) based on SnI-doped (PEA)SnI films.

The effects of preconception and early gestation SARS-CoV-2 infection on pregnancy outcomes and placental pathology.

Objective: To evaluate if peri-pregnancy timing of a PCR+ test for SARS-CoV-2 RNA affects pregnancy outcomes and placental pathology.

Methods: This is a retrospective cohort study conducted in a tertiary center. Pregnancy outcomes and placental pathology were compiled for women who tested positive for SARS-CoV-2 RNA from a nasopharyngeal swab assessed by RT-PCR.

Calretinin Staining in Anorectal Line Biopsies Accurately Distinguished Hirschsprung Disease in a Retrospective Study.

Introduction: The absence of submucosal ganglion cells does not reliably distinguish Hirschsprung disease from non Hirschsprung disease in anorectal line biopsies. Calretinin staining might be helpful in these biopsies. To determine its value, we analyzed calretinin positive mucosal neurites in anorectal line biopsies.

Placental delayed villous maturation is associated with fetal congenital heart disease.

Background: The placenta is crucial for the overall development and lifelong health of the fetus. Abnormal placental development and function occur in pregnancies with fetal congenital heart disease. However, studies that use standardized diagnostic criteria and incorporate control populations are lacking.

Very-Early Onset Chronic Active Colitis with Heterozygous Variants in and a Case of "Immune TOR-Opathies".

Background: A small subset of cases of inflammatory bowel disease (IBD) occurs as a result of single gene defects, and typically occurs in young or very young pediatric patients, referred to as "monogenic very-early onset IBD (VEO-IBD)". The gene variants leading to monogenic VEO-IBD are often associated with primary immunodeficiency syndromes.

Case Report: A six year-old girl presented to our gastroenterology clinic with LRBA deficiency with a heterozygous mutation at c.

Automated Tumor Count for Mitosis-Karyorrhexis Index Determination in Neuroblastoma Using Whole Slide Image and Qupath, an Image Analytic Software.

Introduction: Mitosis-karyorrhexis index (MKI) is important for risk stratification workup of neuroblastic tumors. MKI is calculated by estimating the denominator (5000 tumor cells). We hypothesized that whole slide image (WSI) with appropriate digital image analytical software could provide an objective aid to pathologist's MKI workup.

Rhamnose Is Superior to Mannitol as a Monosaccharide in the Dual Sugar Absorption Test: A Prospective Randomized Study in Children With Treatment-Naïve Celiac Disease.

Background And Aim: We sought to correlate two different measures of gut permeability [lactulose:mannitol (L:M) and lactulose:rhamnose (L:R)] to the severity of duodenal histopathology in children with and without elevated antibodies to tissue transglutaminase (tTG). A secondary objective was to correlate gut permeability with celiac disease (CD) serology and indices of inflammation and bacterial product translocation.

Methods: We prospectively randomized children undergoing endoscopy with abnormal ( = 54) and normal ( = 10) concentrations of circulating antibodies to tTG, to either L:M or L:R.

Case Report: "Primary Immunodeficiency"-Severe Autoimmune Enteropathy in a Pediatric Heart Transplant Recipient Treated With Abatacept and Alemtuzumab.

Disorders of immune dysregulation following heart transplantation in children have been reported; however, the management of such disorders remains uncertain and challenging. In this case report, we describe a clinical course of a child with severe autoimmune enteropathy after a heart transplant in infancy and detail a treatment approach with abatacept and alemtuzumab. A 21-month-old girl with a medical history of congenital dilated cardiomyopathy and heart transplantation at 2 months was evaluated for chronic hematochezia.

Doping of Sn-based two-dimensional perovskite semiconductor for high-performance field-effect transistors and thermoelectric devices.

Doping is an important technique for semiconductor materials and devices, yet effective and controllable doping of organic-inorganic halide perovskites is still a challenge. Here, we demonstrate a facile way to dope two-dimensional Sn-based perovskite (PEA)SnI by incorporating SnI in the precursor solutions. It is observed that Sn produces p-doping effect on the perovskite, which increases the electrical conductivity by 10 times.