Prevention of Parenteral Nutrition-associated Cholestasis Using Reduced Dose Soybean Lipid Emulsion: A Multicenter Randomized Trial.

Introduction: Reducing soybean lipid emulsion (SLE) dose may prevent parenteral nutrition-associated cholestasis (PNAC) but effects on growth and neurodevelopment are unknown. The purpose of this study was to evaluate the effect of reduced dose SLE on growth and neurodevelopment.

Methods: Surgical neonates at 4 centers were randomized to standard SLE (3 g/kg/day) or reduced SLE (1 g/kg/day) over a 12-week period.

Autophagy protein ATG-16.2 and its WD40 domain mediate the beneficial effects of inhibiting early-acting autophagy genes in C. elegans neurons.

While autophagy genes are required for lifespan of long-lived animals, their tissue-specific roles in aging remain unclear. Here, we inhibited autophagy genes in Caenorhabditis elegans neurons, and found that knockdown of early-acting autophagy genes, except atg-16.2, increased lifespan, and decreased neuronal PolyQ aggregates, independently of autophagosomal degradation.

Silky Liquid Metal Electrodes for On-Skin Health Monitoring.

Next generation on-skin electrodes will require soft, flexible, and gentle materials to provide both high-fidelity sensing and wearer comfort. However, many commercially available on-skin electrodes lack these key properties due to their use of rigid hardware, harsh adhesives, uncomfortable support structures, and poor breathability. To address these challenges, this work presents a new device paradigm by joining biocompatible electrospun spider silk with printable liquid metal to yield an incredibly soft and scalable on-skin electrode that is strain-tolerant, conformable, and gentle on-skin.

Intermediate filaments associate with aggresome-like structures in proteostressed C. elegans neurons and influence large vesicle extrusions as exophers.

Toxic protein aggregates can spread among neurons to promote human neurodegenerative disease pathology. We found that in C. elegans touch neurons intermediate filament proteins IFD-1 and IFD-2 associate with aggresome-like organelles and are required cell-autonomously for efficient production of neuronal exophers, giant vesicles that can carry aggregates away from the neuron of origin.

Large vesicle extrusions from neurons are consumed and stimulated by glial-like phagocytosis activity of the neighboring cell.

neurons under stress can produce giant vesicles, several microns in diameter, called exophers. Current models suggest that exophers are neuroprotective, providing a mechanism for stressed neurons to eject toxic protein aggregates and organelles. However, little is known of the fate of the exopher once it leaves the neuron.

Neonatal pneumothorax in congenital diaphragmatic hernia: Be wary of high ventilatory pressures.

Background: Patients with congenital diaphragmatic hernia (CDH) require invasive respiratory support and higher ventilator pressures may be associated with barotrauma. We sought to evaluate the risk factors associated with pneumothorax in CDH neonates prior to repair.

Methods: We retrospectively reviewed newborns born with CDH between 2014 and 2019 who developed a pneumothorax, and we matched these cases to patients with CDH without pneumothorax.

Hepatic Function in Premature Lambs Supported by the Artificial Placenta and Total Parenteral Nutrition.

The artificial placenta (AP) promotes organ development and reduces organ injury in a lamb model of extreme prematurity. This study evaluates hepatic outcomes after AP support with total parenteral nutrition (TPN) administration. Premature lambs (116-121 days estimated gestational age; term = 145) were cannulated for 7 days of AP support.

Management of the undescended testis in children: An American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee Systematic Review.

Purpose: Management of undescended testes (UDT) has evolved over the last decade. While urologic societies in the United States and Europe have established some guidelines for care, management by North American pediatric surgeons remains variable. The aim of this systematic review is to evaluate the published evidence regarding the treatment of (UDT) in children.

Stress increases in exopher-mediated neuronal extrusion require lipid biosynthesis, FGF, and EGF RAS/MAPK signaling.

In human neurodegenerative diseases, neurons can transfer toxic protein aggregates to surrounding cells, promoting pathology via poorly understood mechanisms. In , proteostressed neurons can expel neurotoxic proteins in large, membrane-bound vesicles called exophers. We investigated how specific stresses impact neuronal trash expulsion to show that neuronal exopher production can be markedly elevated by oxidative and osmotic stress.

Quantitative Approaches for Scoring in vivo Neuronal Aggregate and Organelle Extrusion in Large Exopher Vesicles in C. elegans.

Toxicity of misfolded proteins and mitochondrial dysfunction are pivotal factors that promote age-associated functional neuronal decline and neurodegenerative disease across species. Although these neurotoxic challenges have long been considered to be cell-intrinsic, considerable evidence now supports that misfolded human disease proteins originating in one neuron can appear in neighboring cells, a phenomenon proposed to promote pathology spread in human neurodegenerative disease. C.

Interdisciplinary Care and a Focus on Fertility Preservation When Multi-cystic Ovaries Cause Ovarian Torsion: A Case of a 9-year-old Girl with Severe, Undiagnosed Hypothyroidism.

Background: Ovarian torsion can occur in Van Wyk Grumbach syndrome, a disorder characterized by severe primary hypothyroidism and ovarian enlargement. To date, all documented cases of torsion in this setting describe oophorectomy, which has significant hormonal and fertility implications.

Case: A 9-year-old pubertal girl presented to the emergency room with abdominal pain.

Safety of delayed decannulation of venoarterial cannulas in patients with congenital diaphragmatic hernia.

Background: The practice of "cutting-away" from venoarterial extracorporeal life support (ECLS) and leaving indwelling heparinized cannulas prior to decannulation is controversial. This study aims to determine the safety and efficacy of this strategy in patients with congenital diaphragmatic hernia (CDH) who require ECLS.

Methods: A single-center retrospective review of electronic health records was performed on all patients with CDH who underwent elective ECLS decannulation between January 2014 and September 2018.

A standardized guideline for antibiotic prophylaxis in surgical neonates.

Infection following surgical procedures leads to increased morbidity and mortality in all populations. Guidelines to aid providers in the proper use of prophylactic antibiotics exist for adults, but are rare in the neonatal surgical population. A recent emphasis on appropriate antibiotic stewardship had led to the development of more guidelines without a coincident increase in surgical site infection.

Ovarian masses in the child and adolescent: An American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee systematic review.

Background: The treatment of ovarian masses in pediatric patients should balance appropriate surgical management with the preservation of future reproductive capability. Preoperative estimation of malignant potential is essential to planning an optimal surgical strategy.

Methods: The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee drafted three consensus-based questions regarding the evaluation and treatment of ovarian masses in pediatric patients.

Q&A: Trash talk: disposal and remote degradation of neuronal garbage.

Caenorhabditis elegans neurons have recently been found to throw out cellular debris for remote degradation and/or storage, adding an "extracellular garbage elimination" option to known intracellular protein and organelle degradation pathways. This Q&A describes initial insights into the biology of seemingly selective protein and organelle elimination by challenged neurons, highlighting mysteries of how garbage is distinguished and sorted in the sending neuron, how the garbage-filled "exophers" appear to elicit degradative responses as they transit neighboring tissue, and how non-digestible materials get thrown out of cells again via processes that may be highly relevant to human neurodegenerative disease mechanisms.

Time to appendectomy for acute appendicitis: A systematic review.

Objective: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to develop recommendations regarding time to appendectomy for acute appendicitis in children within the context of preventing adverse events, reducing cost, and optimizing patient/parent satisfaction.

Methods: The committee selected three questions that were addressed by searching MEDLINE, Embase, and the Cochrane Library databases for English language articles published between January 1, 1970 and November 3, 2016. Consensus recommendations for each question were made based on the best available evidence for both children and adults.

Ovarian torsion in pediatric and adolescent patients: A systematic review.

Objective: Ovarian torsion in pediatric patients is a rare event and is primarily managed by pediatric general surgeons. Torsion can be treated with detorsion of the ovary or oopherectomy. Oopherectomy is the most common procedure performed by pediatric general surgeons for ovarian torsion.

Avalon catheters in pediatric patients requiring ECMO: Placement and migration problems.

Purpose: The Avalon dual-lumen venovenous catheter has several advantages, but placement techniques and management have not been adequately addressed in the pediatric population. We assessed our institutional outcomes and complications using the Avalon catheter in children.

Methods: We reviewed all pediatric patients who had Avalon catheters placed for respiratory failure at our institution, excluding congenital heart disease patients, from April 2009 to March 2016.

Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee.

Purpose: Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients.

Methods: Questions regarding the management of a pediatric patient with a CPAM were generated.

C. elegans neurons jettison protein aggregates and mitochondria under neurotoxic stress.

The toxicity of misfolded proteins and mitochondrial dysfunction are pivotal factors that promote age-associated functional neuronal decline and neurodegenerative disease. Accordingly, neurons invest considerable cellular resources in chaperones, protein degradation, autophagy and mitophagy to maintain proteostasis and mitochondrial quality. Complicating the challenges of neuroprotection, misfolded human disease proteins and mitochondria can move into neighbouring cells via unknown mechanisms, which may promote pathological spread.

Taking a STEP back: Assessing the outcomes of multiple STEP procedures.

Purpose: Short bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)-associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described.

Surgical management of gastroesophageal reflux disease (GERD) in children: A systematic review.

Objective: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the surgical treatment of pediatric gastroesophageal reflux disease (GERD).

Methods: Five questions were addressed by searching the MEDLINE, Cochrane, Embase, Central, and National Guideline Clearinghouse databases using relevant search terms. Consensus recommendations were derived for each question based on the best available evidence.

Lipid management in pediatric intestinal failure.

Purpose Of Review: Lipids are a crucial component in parenteral nutrition but their prolonged use can be associated with parenteral nutrition-associated liver disease (PNALD), which may lead to significant morbidity and mortality. The purpose of this review is to discuss the current state of lipid management in pediatric intestinal failure, especially as it relates to the prevention of PNALD.

Recent Findings: Lipid restriction has emerged as a successful strategy in both the treatment and prevention of PNALD in parenteral nutrition-dependent children.