Effect of Early vs Late Inguinal Hernia Repair on Serious Adverse Event Rates in Preterm Infants: A Randomized Clinical Trial.

Importance: Inguinal hernia repair in preterm infants is common and is associated with considerable morbidity. Whether the inguinal hernia should be repaired prior to or after discharge from the neonatal intensive care unit is controversial.

Objective: To evaluate the safety of early vs late surgical repair for preterm infants with an inguinal hernia.

Congenital intrathoracic accessory spleen is a very rare trick of nature: a case report.

Background: Congenital intrathoracic accessory spleen (CIAS) refers to a developmental anomaly resulting in the presence of splenic tissue within the chest. The differential diagnoses for the resulting mass are pulmonary malformations, or lesions with malignant potential. To our knowledge, only four cases of presumed CIAS have been described in literature to date, and no cases were reported in the United States.

Laparoscopic Splenectomy: Has It Become the Standard Surgical Approach in Pediatric Patients?

Background: Splenectomy is often required in the pediatric population as part of the treatment of hematologic disorders and can be performed laparoscopically or open. We evaluated the comparative effectiveness of laparoscopic (LS) and open (OS) splenectomies using the American College of Surgeons National Surgical Quality Improvement Program Pediatric (NSQIP-P) data set.

Methods: The NSQIP-P data set was used to identify children who underwent elective splenectomy between January 2012 and December 2016.

Pediatric trauma center verification improves quality of care and reduces resource utilization in blunt splenic injury.

Purpose: We sought to evaluate value impact of transition from an adult trauma center treating children (ATC) to a verified pediatric trauma center (PTC) in children with blunt splenic injury (BSI).

Methods: Children with BSI from FY 2005 to FY 2017 were extracted from the hospital trauma registry. February 2009 distinguished "ATC" treated children from "PTC" treated children.

The Thoracic Inlet Index in patients with tracheal obstruction caused by chest wall deformity: Validation in patients and age-matched controls.

Aim: We present a novel index for evaluating severity of airway-threatening thoracic inlet compromise in childhood. Two indices were validated in three cases and sixty asymptomatic controls.

Methods: We developed an index to determine severity of thoracic inlet narrowing.

Peritoneal dialysis in an extremely low-birth-weight infant with acute kidney injury.

Critically ill neonates are at high risk for acute kidney injury (AKI). Renal supportive therapy (RST) can be an important tool for supporting critically ill neonates with AKI, particularly in cases of oliguria and fluid overload. There are few reports of RST for management of oligo-anuric AKI in the extremely low-birth-weight infant weighing <1000 g.

Segmental resection for the treatment of congenital pulmonary malformations.

Purpose: The purpose of this study was to compare clinical outcomes of segmental resection to lobectomy as increasing antenatal diagnosis of congenital pulmonary malformations has led to a shift in surgical management.

Methods: A retrospective institutional review for patients undergoing surgical excision of congenital pulmonary malformations was performed.

Results: Sixty-two patients with congenital pulmonary malformations were reviewed between 2001 and 2012.

Outcome of antegrade continent enema (ACE) procedures in children and young adults.

Objective: Intractable incontinence affects a large number of children and young adults in the US. The goal of this study is to evaluate the long-term outcomes of surgical access for administration of antegrade continence enemas (ACE) in affected children and young adults.

Methods: Patients who underwent surgical procedure to enable administration of ACE from 1994 to 2011 were retrospectively reviewed.

A pediatric cervical spine clearance protocol to reduce radiation exposure in children.

Background: To minimize radiation exposure in children and reduce resource use, we implemented an age-specific algorithm to evaluate cervical spine injuries at a Level 1 trauma center. The effects of protocol implementation on computed tomography (CT) use in children (≤ 10 y) were determined.

Methods: With institutional review board approval, we conducted a retrospective review using the institutional trauma registry.

Invasion in follicular thyroid cancer cell lines is mediated by EphA2 and pAkt.

Background: EphA2 is a tyrosine kinase receptor that is overexpressed in many cancers and is associated with poor prognosis and increased metastasis. Phosphorylated Akt (pAkt) plays a role in the regulation of thyroid cancer invasion and metastasis. We investigated the role of EphA2 and Akt in FTC-133 and FTC-238, 2 closely related human cell lines with differing invasive phenotypes.

Annexin-V promotes anti-tumor immunity and inhibits neuroblastoma growth in vivo.

The goal of the current study is to determine the effects of blocking phosphatidylserine (PS) on the growth of neuroblastoma in mice. PS, an anionic phospholipid restricted to the cytoplasmic surface of plasma membranes in most cells, is externalized to the surface of apoptotic cells. PS has been shown to induce immune tolerance to self-antigens.

Outcomes of plastic closure in gastroschisis.

Background: Gastroschisis is a congenital abdominal wall defect in which the intestines develop outside the abdomen and are exposed to amniotic fluid. When the defect is small, lymphatic, venous, and intestinal obstruction may occur and contribute to the formation of intestinal edema, atresia, ischemia, and a thick inflammatory peel. Treatment requires early coverage of abdominal contents either by primary closure or by the placement of temporary Silastic silo followed by abdominal wall closure.

Phosphatidylserine inhibits NFκB and p38 MAPK activation in human monocyte derived dendritic cells.

Phosphatidylserine (PS) is an anionic phospholipid restricted to the inner surface of the plasma membrane. PS translocates to the cell surface during early apoptosis where it serves as a marker for rapid uptake by phagocytes. PS is also thought to regulate immune responses.

The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs.

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. The most common CF-associated mutation is ΔF508, which deletes a phenylalanine in position 508. In vitro studies indicate that the resultant protein, CFTR-ΔF508, is misprocessed, although the in vivo consequences of this mutation remain uncertain.

Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth.

Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). Understanding the pathogenesis of this disease has been hindered, however, by the lack of an animal model with characteristic features of CF. To overcome this problem, we recently generated pigs with mutated CFTR genes.

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.

Almost two decades after CFTR was identified as the gene responsible for cystic fibrosis (CF), we still lack answers to many questions about the pathogenesis of the disease, and it remains incurable. Mice with a disrupted CFTR gene have greatly facilitated CF studies, but the mutant mice do not develop the characteristic manifestations of human CF, including abnormalities of the pancreas, lung, intestine, liver, and other organs. Because pigs share many anatomical and physiological features with humans, we generated pigs with a targeted disruption of both CFTR alleles.

Induction of cytolytic T lymphocytes against pediatric solid tumors in vitro using autologous dendritic cells pulsed with necrotic primary tumor.

Purpose: Effective and generally applicable methods for generating cancer vaccines in children have not been defined. Dendritic cells (DCs) are the most potent professional antigen-presenting cells capable of activating primary cytolytic T cells. We tested the ability of DCs generated from pediatric patients' peripheral blood monocytes and pulsed with a necrotic tumor to activate autologous tumor-specific cytolytic T cells.

CD40 ligation restores cytolytic T lymphocyte response and eliminates fibrosarcoma in the peritoneum of mice lacking CD4+ T cells.

Absence of CD4(+) T cell help has been suggested as a mechanism for failed anti-tumor cytotoxic T lymphocytes (CTL) response. We examined the requirement for CD4(+) T cells to eliminate an immunogenic murine fibrosarcoma (6132A) inoculated into the peritoneal cavity. Immunocompetent C3H mice eliminated both single and repeat intraperitoneal (IP) inoculums, and developed high frequency of 6132A-specific interferon-gamma (IFNgamma)-producing CTL in the peritoneal cavity.

Use of vacuum-assisted closure system in the management of complex wounds in the neonate.

The vacuum-assisted closure (VAC) system has become an accepted treatment modality for acute and chronic wounds in adults. The use of negative-pressure dressing has been documented in adults and, to some extent, in children. However, its use in premature infants has not been reported in the literature.

Phosphatidylserine regulates the maturation of human dendritic cells.

Phosphatidylserine (PS), which is exposed on the surface of apoptotic cells, has been implicated in immune regulation. However, the effects of PS on the maturation and function of dendritic cells (DCs), which play a central role in both immune activation and regulation, have not been described. Large unilamellar liposomes containing PS or phosphatidylcholine were used to model the plasma membrane phospholipid composition of apoptotic and live cells, respectively.

Inhibition of hepatic gluconeogenesis and enhanced glucose uptake contribute to the development of hypoglycemia in mice bearing interleukin-1beta- secreting tumor.

Mice bearing IL-1beta-secreting tumor were used to study the chronic effect of IL-1beta on glucose metabolism. Mice were injected with syngeneic tumor cells transduced with the human IL-1beta gene. Serum IL-1beta levels increased exponentially with time.

Restored immune response to an MHC-II-Restricted antigen in tumor-bearing hosts after elimination of regulatory T cells.

Background/purpose: Pediatric sarcomas have a poor prognosis, recur frequently, and are not effectively treated by currently available therapy. Immunotherapy is a promising treatment modality; however, to be successful, immune tolerance must be overcome. CD4+CD25+ T cells are immunosuppressive.

Choledochal cysts in western adults: complexities compared to children.

Choledochal cysts occur most frequently in East Asian children and rarely in Western adults. Over the past two decades, pediatric treatment has been standardized, but relatively little information is available on the management of Western adults with choledochal cysts. Therefore the aims of this analysis were to compare the presentation, management, and late results of Western adults and children with choledochal cysts.