Novel Dynamic Organ Storage System Enhances Liver Graft Function in a Porcine Donation After Circulatory Death Model.

Donation after circulatory death (DCD) livers face increased risks of critical complications when preserved with static cold storage (SCS). Although machine perfusion (MP) may mitigate these risks, its cost and logistical complexity limit widespread application. We developed the Dynamic Organ Storage System (DOSS), which delivers oxygenated perfusate at 10°C with minimal electrical power requirement and allows real-time effluent sampling in a portable cooler.

Survival Mechanisms and Retention Strategies in Large-Volume Fat Grafting: A Comprehensive Review and Future Perspectives.

Introduction: Large-volume fat grafting is emerging as a promising technique in plastic and reconstructive surgery. However, the unpredictable graft volume retention rate remains a critical challenge. To address this issue, we need a profound understanding of the survival mechanisms following large-volume fat transplantation.

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.

The transcription factor ZNF469 regulates collagen production in liver fibrosis.

Non-alcoholic fatty liver disease (NAFLD) - characterized by excess accumulation of fat in the liver - now affects one third of the world's population. As NAFLD progresses, extracellular matrix components including collagen accumulate in the liver causing tissue fibrosis, a major determinant of disease severity and mortality. To identify transcriptional regulators of fibrosis, we computationally inferred the activity of transcription factors (TFs) relevant to fibrosis by profiling the matched transcriptomes and epigenomes of 108 human liver biopsies from a deeply-characterized cohort of patients spanning the full histopathologic spectrum of NAFLD.

Survival outcomes in pediatric patients with metastatic Ewing sarcoma who achieve a rapid complete response of pulmonary metastases.

Purpose: Our objectives were to compare overall survival (OS) and pulmonary relapse between patients with metastatic Ewing sarcoma (EWS) at diagnosis who achieve rapid complete response (RCR) and those with residual pulmonary nodules after induction chemotherapy (non-RCR).

Patients And Methods: This retrospective cohort study included children under 20 years with metastatic EWS treated from 2007 to 2020 at 19 institutions in the Pediatric Surgical Oncology Research Collaborative. Chi-square tests were conducted for differences among groups.

Open Thermal Control System for Stable Polymerase Chain Reaction on a Digital Microfluidic Chip.

In this paper, a digital microfluidic thermal control system was introduced for the stable polymerase chain reaction (PCR). The system consists of a thermoelectric cooler unit, a thermal control board, and graphical-user-interface software capable of simultaneously achieving temperature control and on-chip droplet observation. A fuzzy proportional-integral-derivative (PID) method was developed for this system.

Characterization of pediatric non-hematopoietic tumor metastases to the central nervous system: A single institution review.

Central nervous system (CNS) metastases represent a small portion of pediatric CNS neoplasms and data surrounding this condition with high morbidity is scarce. Single institutional archival institutional pathology records between 1999 and 2022 were searched for patients over 21 years old and younger with CNS, dura, cranial nerve, CSF, or leptomeningeal metastases; 41 cases were identified. We documented primary tumor types and locations, metastasis locations, types of invasion (direct extension vs distant metastasis), times from imaging or pathologic diagnosis to CNS involvement, and outcomes.

Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor.

Intracranial mesenchymal tumor (IMT), FET::CREB fusion-positive is a provisional tumor type in the 2021 WHO classification of central nervous system tumors with limited information available. Herein, we describe five new IMT cases from four females and one male with three harboring an EWSR1::CREM fusion and two featuring an EWSR1::ATF1 fusion. Uniform manifold approximation and projection of DNA methylation array data placed two cases to the methylation class "IMT, subclass B", one to "meningioma-benign" and one to "meningioma-intermediate".

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.

New Insights and Advanced Strategies for Construction of Vascularized Tissue Engineering.

Inadequate vascularization is a significant barrier to clinical application of large-volume tissue engineered grafts. In contrast to vascularization, prevascularization shortens the time required for host vessels to grow into the graft core and minimizes necrosis in the core region of the graft. However, the challenge of prevascularization is to construct hierarchical perfusable vascular networks, increase graft volume, and form a vascular tip that can anastomose with host vessels.

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.

Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents.

Introduction: Ewing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS).

Pulmonary artery banding in sheep: a novel large animal model for congestive hepatopathy.

Congestive hepatopathy is becoming increasingly recognized among Fontan-palliated patients. Elevated central venous pressure is thought to drive the pathologic progression, characterized by sinusoidal dilatation, congestion, and fibrosis. A clinically relevant large animal model for congestive hepatopathy would provide a valuable platform for researching novel biomarkers, treatment, and prevention.

Early-Onset Dropped Head Syndrome and Person-in-the-Barrel Syndrome After Radiation Therapy: Clinical, Electromyographic, and MRI Findings.

Dropped head syndrome (DHS) involves severe weakness of the neck extensor muscles causing the mandible to drop to the chest wall. Isolated neck extensor weakness is a rare complication of radiotherapy. This condition may result within a few weeks or months following radiotherapy (early-onset) or several years after radiotherapy (late-onset), with the latter more commonly encountered.

Bilateral Ovarian Sertoli-Leydig Cell Tumors Harboring Germline and Distinct Somatic Mutations: Case Report and Literature Review.

tumor predisposition syndrome is characterized by an increased risk for development of pleuropulmonary blastoma, pituitary blastoma, multinodular thyroid goiter, thyroid carcinoma, sex cord stromal tumor, cystic nephroma, embryonal rhabdomyosarcoma, and tumors of the CNS, amongst others. Of this list, only pituitary blastoma is recognized as pathognomonic for the syndrome. We describe a 15-year-old female with bilateral, asynchronous Sertoli-Leydig cell tumors (SLCT).

The Green Placental Disk: Massive Placental Disk Bilirubin Deposition Due to Maternal Hyperbilirubinemia.

Background: Bile staining in the hepatobiliary tree is a well-known phenomenon, but bilirubin can also be deposited in and stain the umbilical cord, amniotic fluid, placental disk and placental membranes.

Case Report: We present a placenta with grossly greenish discoloration of the placental disk and microscopic villous bilirubin deposition due to maternal hyperbilirubinemia. Maternal hyperbilirubinemia causes massive bilirubin deposition in villous synctiotrophoblasts and Hofbauer cells leading to grossly green discoloration of the placental disk.

Nondiagnostic category of Milan System for Reporting Pediatric Salivary Gland Cytopathology: outcomes and root cause analysis.

Background: Fine-needle aspiration (FNA) results classified as the nondiagnostic category of the Milan system for reporting salivary gland cytopathology (MSRSGC) may be infrequently encountered in children. Clinical management may be challenging due to lack of data regarding outcomes and underlying causes.

Methods: We retrospectively analyzed 106 consecutive pediatric salivary gland FNAs (2000-2020; 45% performed under image guidance).

Fine-Needle Aspiration Biopsy of Pediatric Salivary Gland Tumors: Analysis of Patient Tolerability, Sedation Requirement, and Procedural Complication.

Objective: Fine-needle aspiration (FNA) biopsy is the standard diagnostic tool recommended by consensus management guidelines for preoperative evaluation of salivary gland tumors in adults. However, its utility in the pediatric population remains debated due to a paucity of data and inherited challenges of pediatric management (patient cooperation, the need for sedation, and procedural complications).

Methods: Consecutive series of 92 FNA biopsies of pediatric salivary gland lesions with available procedural data were included for retrospective analysis.

Pediatric Extra-Axial Chordoma: Case Report and Literature Review.

Extra-axial chordomas in the pediatric population are extremely rare and diagnostically challenging; only four cases have been previously reported with ages ranging from 13 to 20 years. We report a primary extra-axial chordoma involving the soft tissue directly dorsal and ulnar to proximal phalanx in the right thumb of a 12-year-old girl who presented with worsening right thumb pain for 1.5 years.

Mucinous Adenocarcinoma With Intrapulmonary Metastasis Harboring KRAS and GNAS Mutations Arising in Congenital Pulmonary Airway Malformation.

Objectives: Mucinous adenocarcinoma arising in unresected congenital pulmonary airway malformation (CPAM) is rare. Underlying driver mutations in addition to KRAS gain-of-function mutations in this setting and the long-term outcomes of these patients are unknown.

Methods: We report a case of metastatic mucinous adenocarcinoma harboring both KRAS and GNAS mutations arising in a type 1 CPAM of a 14-year-old male.

Application of the Milan System for Reporting Pediatric Salivary Gland Cytopathology: Analysis of histologic follow-up, risk of malignancy, and diagnostic accuracy.

Background: The diagnosis and management of salivary gland tumors in pediatric patients can be challenging. The utility of fine-needle aspiration (FNA) cytopathology and the performance of the Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) in this age group have not been systematically assessed. The paucity of data has contributed to the controversial role of FNA cytopathology in the presurgical management of these patients.