Children with eosinophilic esophagitis non-responsive to combination therapy have distinct esophageal transcriptomic and microbiome profile.

Background: A combination of proton-pump inhibitors (PPI) and topical steroids (TS) is used to treat children with eosinophilic esophagitis (EoE). However, a subset of children do not respond to this combination therapy. We aimed to identify the esophageal transcriptional, cell composition, and microbial differences between the non-responders (EoE-PPI-TSnr; n = 7) and responders (EoE-PPI-TSr; n = 7) to the combination therapy for EoE and controls (n = 9) using metatranscriptomics.

Clinical and Histopathologic Characteristics of Acute Severe Hepatitis Associated With Human Herpesvirus 6 Infection.

Acute severe hepatitis associated with active human herpesvirus 6 (HHV-6) infection is a rare life-threatening condition with unclear clinical course and histopathology. In this study, we retrospectively analyzed 5 patients with indeterminate acute severe hepatitis found to have active hepatic HHV-6 infection during care. All patients were previously healthy children presenting with a nonspecific prodrome.

Characterization of lamina propria remodeling in pediatric eosinophilic esophagitis using second harmonic generation microscopy.

Eosinophilic esophagitis (EoE) is a chronic inflammatory condition characterized by an intense infiltration of eosinophils into the esophageal epithelium. When not adequately controlled, eosinophilic inflammation can lead to changes in components of the extracellular matrix (ECM) of the lamina propria. Particularly, alterations to the collagen fiber matrix can lead to lamina propria fibrosis (LPF), which plays an important role in the fibrostenotic complications of EoE.

Proton Pump Inhibitors Modulate Gene Expression Profile in Esophageal Mucosa and Microbiome.

Objective: Proton pump inhibitors (PPIs) are commonly used to manage children with upper gastrointestinal symptoms and without a formal diagnosis. We investigated the effect of PPIs on esophageal mucosal transcriptome and active microbiota in children with normal esophagi. Furthermore, we examined whether the differences in host esophageal mucosal gene expression were driven by an underlying esophageal epithelial cell type composition.

In Vivo Raman Spectroscopy Reveals Biochemical Composition of the Esophageal Tissue in Pediatric Eosinophilic Esophagitis.

Introduction: Biochemical alterations in the esophagus of patients with eosinophilic esophagitis (EoE) are poorly understood. We used Raman spectroscopy through a pediatric endoscope to identify key Raman features reflective of the esophageal biochemical composition to differentiate between children with EoE from non-EoE controls and between children with active (aEoE) and inactive EoE (iEoE).

Methods: Spectral measurements were obtained using a customized pediatric endoscope-compatible fiber-optic Raman probe in real time during an esophagogastroduodenoscopy.

Hashimoto's Thyroiditis and the Risk of Papillary Thyroid Cancer in Children.

The association between Hashimoto's thyroiditis (HT) and pediatric thyroid cancer is controversial. Most studies examining this connection have been based on adults, and larger studies in children are lacking. We performed a retrospective study of all sequential pediatric patients who underwent a thyroidectomy for a neoplasm at our institution over a twenty-year period in order to explore the link between HT and pediatric thyroid cancer.

Clinicopathologic Features of Severe Acute Hepatitis Associated With Adenovirus Infection in Children.

A recent increase in reports of severe acute hepatitis of unknown etiology in children is under investigation. Although adenovirus has been frequently detected, its role remains unclear, and systematic histopathologic analysis is lacking. We conducted a retrospective study of 11 children hospitalized between October 2021 and May 2022 with unexplained acute hepatitis and concurrent adenovirus infection.

Outcomes and Histological Variations of Neuroblastoma and Ganglioneuroblastoma with Paraneoplastic Syndromes.

Background: Neuroblastomas are the most common extracranial solid malignancy in children with variable manifestations and complications depending on the presence of paraneoplastic syndromes.

Materials And Methods: We performed a single institution retrospective cohort study of all patients less than 18 years old diagnosed with neuroblastoma or ganglioneuroblastoma between January 2002 and July 2022. Patients were identified through the pathology and cancer registry and cross-referenced with pediatric records.

Exploiting embryonic niche conditions to grow Wilms tumor blastema in culture.

Introduction: Wilms Tumor (WT), or nephroblastoma, is the most common pediatric kidney cancer. Most WTs display a "favorable" triphasic histology, in which the tumor is comprised of blastemal, stromal, and epithelial cell types. Blastemal predominance after neoadjuvant chemotherapy or diffuse anaplasia ("unfavorable" histology; 5-8%) portend a worse prognosis.

Minimally invasive adnexa-sparing surgery for benign ovarian and paratubal masses in children.

Background: The precision of minimally invasive surgery (MIS) to resect benign ovarian and paratubal masses while preserving adnexa in children is unclear. This study evaluated the integrity of laparoscopy to spare adnexa while resecting benign pathologies in children.

Methods: The institutional pathology database was queried to identify patients aged 18 years and younger having any ovarian or tubal lesion resected at a comprehensive children's hospital between 2006 and 2021.

Comorbidities in Chilean patients with psoriasis: a Global Healthcare Study on Psoriasis.

Background: Psoriasis is a chronic inflammatory skin disease associated with several important medical comorbidities. There are scant data available on the comorbidities of patients with psoriasis in South America.

Aim: To examine the comorbidity profile of adult patients with psoriasis in Chile and its association with severity of psoriasis.

Intraoperative Ultrasound Guided Laparoscopic Spleen-Preserving Distal Pancreatectomy for Primitive Neuroendocrine Tumors in a Pediatric Patient With MEN-1.

This case details the presentation and surgical management of a 15-year-old male patient with multiple endocrine neoplasia syndrome type 1 (MEN1) who required distal pancreatectomy for multiple nonfunctional pancreatic tumors. An intraoperative ultrasound was utilized to allow for proper location of the distal pancreatectomy, as well as visualization of the splenic vessel relationships and to ensure all lesions were contained within the specimen. Pathology demonstrated 5 well-differentiated neuroendocrine tumors with no evidence of malignancy.

Civatte Bodies in Pediatric Esophageal Biopsies: Does Lichen Esophagitis Pattern Occur in Children?

Purpose And Context: Civatte bodies (CB) are associated with cutaneous and mucosal lichen planus in adults. They are a distinct feature of Lichen Esophagitis Pattern, which is not well described in children. We characterized clinicopathologic associations of archival esophageal CB at our Children's Hospital to determine whether lichen planus or Lichen Esophagitis Pattern occurs in children.

Elective choledochal cyst excision is associated with improved postoperative outcomes in children.

Purpose: The majority of pediatric patients with choledochal cysts (CDC) are symptomatic prior to undergoing CDC excision. This study investigated the impact of surgical timing of CDC excision on postoperative outcomes among children.

Methods: We performed a retrospective review of 59 patients undergoing open CDC excision with Roux-Y hepaticojejunostomy between 2000 and 2020.

Persistent mass after treatment for orbital rhabdomyosarcoma.

Purpose: To report a case of orbital rhabdomyosarcoma and highlight the treatment approach to the dilemma of a residual mass.

Observations: An eleven-year-old boy was diagnosed with Stage 1, Group III embryonal rhabdomyosarcoma in the orbit. After completing a 24-week treatment regimen of chemotherapy and radiation, imaging showed a large persistent mass with erosion through the medial wall.

Development and Validation of Web-Based Tool to Predict Lamina Propria Fibrosis in Eosinophilic Esophagitis.

Introduction: Approximately half of esophageal biopsies from patients with eosinophilic esophagitis (EoE) contain inadequate lamina propria, making it impossible to determine the lamina propria fibrosis (LPF). This study aimed to develop and validate a web-based tool to predict LPF in esophageal biopsies with inadequate lamina propria.

Methods: Prospectively collected demographic and clinical data and scores for 7 relevant EoE histology scoring system epithelial features from patients with EoE participating in the Consortium of Eosinophilic Gastrointestinal Disease Researchers observational study were used to build the models.

Laparoscopic Resection of Pediatric Solid Pseudopapillary Tumors of the Pancreas.

Background: Solid pseudopapillary tumors (SPTs) of the pancreas arise rarely in children, are often large, and can associate intimately with splenic vessels. Splenic preservation is a fundamental consideration when resecting distal SPT. Occasionally, the main splenic vessels must be divided to resect the SPT with negative margins, but the spleen can be preserved if the short gastric vessels remain intact (ie, Warshaw procedure).

Second Report of Fusion in Pediatric Spindle Cell Sarcoma With Infantile Fibrosarcoma-Like Morphology Suggesting Fusion Is a Recurrent Event.

Infantile/congenital fibrosarcoma (IFS) is the most common soft tissue tumor in children less than one year of age. The most common anatomic site of IFS is in the extremities or trunk, and rarely in the abdomen or retroperitoneum. Approximately 70-90% of cases are characterized by a distinct t(12;15)(p13;q25) translocation resulting in an gene fusion.