Optimizing Post-Kasai Management in Biliary Atresia: Balancing Native Liver Survival and Transplant Timing.

Background Kasai procedure (KPE) is a palliative intervention in infants with biliary atresia (BA) aiming to restore biliary drainage. While the measure of success in BA is the post-Kasai native liver survival, BA remains the most frequent indication for liver transplantation in children. While a considerable amount of children fail to clear their jaundice following KPE, resulting in early liver failure and transplantation, some children become jaundice-free after "successful" KPE.

Dissecting the dynamics of cell death pathways in Hirschsprung's disease: a comparative analysis of viable and non-viable cells under proinflammatory conditions.

Purpose: The present study explores the dynamics of cell death in Hirschsprung's disease (HSCR) and control (CO) groups under inflammatory stress conditions.

Methods: Using flow cytometry, we analyzed intestinal colonic organoid cultures derived from the ganglionic segment of the HSCR and CO groups. Our analysis focused on the quantification of RIPK1-independent and RIPK1-dependent apoptosis, as well as necroptosis in both viable and non-viable cells under acute and chronic inflammatory stress.

Biliatresone induces cholangiopathy in C57BL/6J neonates.

Exposure to plant toxins or microbiota that are able to digest common food ingredients to toxic structures might be responsible for biliary atresia (BA). An isoflavonoid, biliatresone is known to effectively alter the extrahepatic bile duct (EHBD) development in BALB/c mice. Biliatresone causes a reduction of Glutathione (GSH) levels, SOX17 downregulation and is effectively countered with N-Acetyl-L-cysteine treatment in vitro.

Expanded ILC2s in human infant intestines promote tissue growth.

Early life is characterized by extraordinary challenges, including rapid tissue growth and immune adaptation to foreign antigens after birth. During this developmental stage, infants have an increased risk of immune-mediated diseases. Here, we demonstrate that tissue-resident, interleukin (IL)-13- and IL-4-producing group 2 innate lymphoid cells (ILC2s) are enriched in human infant intestines compared to adult intestines.

Appendicitis and Peritonitis in Children with a Ventriculo-Peritoneal Shunt.

The purpose of this study was to outline the management of patients with appendicitis and ventriculoperitoneal shunt (VPS) in the largest pediatric surgery department in Germany. Patients with VPS presenting with an acute abdomen between 2012 and 2022 at a tertiary-care pediatric facility were the subject of a retrospective descriptive analysis. Patients were divided into two groups based on their diagnoses: group A (appendicitis) and group B (primary peritonitis).

CXCR5PD-1 CD4 T cells colonize infant intestines early in life and promote B cell maturation.

Gastrointestinal infections are a major cause for serious clinical complications in infants. The induction of antibody responses by B cells is critical for protective immunity against infections and requires CXCR5PD-1 CD4 T cells (T cells). We investigated the ontogeny of CXCR5PD-1 CD4 T cells in human intestines.

Extrahepatic Bile Duct and Gall Bladder Dissection in Nine-Day-Old Mouse Neonates.

The dissection of murine neonatal bile ducts has been described as difficult. The main aim of the described standard operating procedure is the isolation of the extrahepatic bile duct (EBD) in mouse neonates without damaging the bile duct during preparation. Because of its exceptionally close preparation compared to the cholangiocytes cell line and harvesting of the entire extrahepatic bile duct system (EBDS), the described approach is extremely useful in researching animal models of newborn bile duct disorders, such as biliary atresia.

Limits in Laparoscopic Partial Splenectomy in Children.

The aim of this paper is to assess the effectiveness and perioperative complications of splenic surgeries in children. In 41 splenectomies, an anterior abdominal laparoscopic approach was used, with 35 including a partial laparoscopic splenectomy. Of these, three needed a conversion to open.

Systematic review: The quality of life of patients with biliary atresia.

Background: The quality of life of patients with Biliary Atresia (BA) have not been systematically examined. The goal of this meta-analysis is to determine patients' postoperative health-related Quality of life (HrQoL) with native or transplanted livers.

Methods: From 2000 to August 2021, a literature-based search for relevant cohorts was conducted using Pubmed/Medline, the Cochrane Library, and Embase.

The Length of the Transition Zone in Patients with Rectosigmoid Hirschsprung Disease.

The transition zone (TZ) is defined by specific histological findings in patients with Hirschsprung Disease (HSCR). HSCR treatment includes surgical removal of the aganglionic zone (AZ). During the pull-through procedure, it is critical to resect the TZ.

Colectomy Followed by J-Pouch Reconstruction to Correct Total Colonic Aganglionosis.

Background: Patients suffering from complete colonic aganglionosis (TCA) require the best surgical care possible. Only a few studies reported J-Pouch repair as the primary reconstructive surgery in TCA patients. This study adds to the current literature a thorough clinical and functional outcomes group.

Increased protease activated receptors in the colon of patients with Hirschsprung's disease.

Purpose: The pathophysiology of Hirschsprung's associated enterocolitis (HAEC) is not understood. Abnormal intestinal motility and altered intestinal epithelial barrier function have been suggested to play a key role in the causation of HAEC. Protease-activated receptors (PARs) 1 and 2, have been implicated in inflammatory reactions, intestinal permeability and modulation of motility in the gut.

Altered expression of caveolin-1 in the colon of patients with Hirschsprung's disease.

Background/purpose: The pathogenesis of Hirschsprung's disease-associated enterocolitis (HAEC) is unclear. Caveolin-1 (Cav-1) regulates the functions of different nitric oxide synthase (NOS) isoforms, which play critical roles in inflammation and intestinal epithelial barrier function. We designed this study to investigate the hypothesis that Cav-1 expression is altered in the bowel of patients with Hirschsprung's disease (HSCR).

The Extent of the Transition Zone in Hirschsprung Disease.

Background: Retained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease.

Objective: We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease.

Design: We performed an observational study.

Altered expression of inflammasomes in Hirschsprung's disease.

Aim Of The Study: The pathogenesis of Hirschsprung's disease-associated enterocolitis (HAEC) is poorly understood. Inflammasomes are a large family of multiprotein complexes that act to mediate host immune responses to microbial infection and have a regulatory or conditioning influence on the composition of the microbiota. Inflammasomes and the apoptosis-associated speck-like protein (ASC) lead to caspase-1 activation.

Altered expression of KCNG3 and KCNG4 in Hirschsprung's disease.

Purpose: Voltage-gated potassium ion channels have long been implicated in gastrointestinal motility. Recent studies have highlighted the role of voltage-gated channel subfamily G member 3 (KCNG3) and 4 (KCNG4) genes in the electrical functioning of interstitial cells of Cajal and PDGFRα cells of the mouse colon. We designed this study to investigate KCNG3 and KCNG4 expression in the normal human colon and in Hirschsprung's disease (HSCR).

Abnormal Scn1b and Fxyd1 gene expression in the pulled-through ganglionic colon may influence functional outcome in patients with Hirschsprung's disease.

Purpose: Smooth muscle cells are electrically coupled to ICC and PDGFRα cells, to regulate smooth muscle contraction. Recent studies have reported that the voltage-gated sodium channel type 1β (Scn1b), and the chloride channel subunit, Fxyd1, are highly expressed by both ICC and PDGFRα cells in the mouse colon. We designed this study to investigate the expression of the Scn1b and Fxyd1 genes in the normal human colon and in HSCR.

Reduced expression of the NLRP6 inflammasome in the colon of patients with Hirschsprung's disease.

Purpose: Hirschsprung's associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's Disease (HSCR). The pathogenesis of HAEC remains unsatisfactorily understood. Mounting evidence of an altered microbiome in patients with HSCR adds a new angle to the pathogenesis of HAEC.

Altered goblet cell function in Hirschsprung's disease.

Aims And Objectives: Hirschsprung's disease-associated enterocolitis (HAEC) is the most serious complication of Hirschsprung's disease (HSCR). HAEC occurs in 17-50% of patients with HSCR and may occur before or after a properly performed pull-through operation. The pathogenesis of HAEC is poorly understood.

Increased population of immature enteric glial cells in the resected proximal ganglionic bowel of Hirschsprung's disease patients.

Background: Enteric glial cells are essential for normal gastrointestinal function. Abnormalities in glial structure, development, or function lead to disturbances in gastrointestinal physiology. Fatty acid-binding protein 7 (FABP7) is a marker of immature enteric glial cells, whereas S100 is expressed only by mature glial cells.

Reduction of hydrogen sulfide synthesis enzymes cystathionine-β-synthase and cystathionine-γ-lyase in the colon of patients with Hirschsprungs disease.

Purpose: Hirschsprung associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung Disease (HSCR). The pathogenesis of HAEC is poorly understood. In recent years, there is increasing evidence that a compromised intestinal barrier function plays a major role in the pathogenesis of HAEC.

NOS-interacting protein (NOSIP) is increased in the colon of patients with Hirschsprungs's disease.

Purpose: Hirschsprung's associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Nitric oxide (NO) mediates intestinal homoeostasis and is inhibited by NOSIP, a modulator of NO production. We designed this study to investigate the expression of NOSIP in the colon of patients with HSCR.