Minimally invasive approach to non-communicating pyloric duplication

Pyloric duplication is a rare subset of alimentary tract duplications that can present in a manner almost identical to hypertrophic pyloric stenosis. Diagnosis can typically be made with an upper gastrointestinal series and ultrasound, though axial imaging is occasionally required. Because most of these malformations do not communicate with the lumen, they can be effectively managed with partial excision and ablation of the common wall mucosa. Here we present an 11-day-old female with pyloric duplication that was successfully managed laparoscopically

Evolving understanding of neonatal necrotizing enterocolitis

Necrotizing enterocolitis (NEC) is a devastating disease that predominately affects premature neonates. The pathogenesis of NEC is multifactorial and poorly understood. Risk factors include low birth weight, formula-feeding, hypoxic/ischemic insults, and microbial dysbiosis. This review focuses on our current understanding of the diagnosis, management, and pathogenesis of NEC. Recent findings identify specific mucosal cell types as potential therapeutic targets in NEC. Despite a broadly accepted view that bacterial colonization plays a key role in NEC, characteristics of bacterial populations associated with this disease remain elusive. The use of probiotics such as lactobacilli and bifidobacteria has been studied in numerous trials, but there is a lack of consensus regarding specific strains and dosing. Although growth factors found in breast milk such as epidermal growth factor and heparin-binding epidermal growth factor may be useful in disease prevention, developing new therapeutic interventions in NEC critically depends on better understanding of its pathogenesis. NEC is a leading cause of morbidity and mortality in premature neonates. Recent data confirm that growth factors and certain bacteria may offer protection against NEC. Further studies are needed to better understand the complex pathogenesis of NEC

The C-terminal non-catalytic domain of Mkp-1 phosphatase harbors a complex signal for rapid proteasome degradation in enterocytes

Mitogen-activated kinase phosphatase Mkp-1 is an essential negative regulator of innate immune responses. In enterocytes, Mkp-1, which is transiently expressed in response to Toll-like receptor (TLR) ligands, plays a role as an initial step in establishing tolerance to bacteria and their pro-inflammatory molecules. Mkp-1 has a very short half-life (about 30 min) in IEC-6 enterocytes. Here we examined the mechanism of Mkp-1 rapid degradation in IEC-6 cells. Immunoprecipitation and proteolysis inhibitor analysis indicated that Mkp-1 is monoubiquitinated and degraded by proteasome. Dominant negative ubiquitin increased Mkp-1 half-life, indicating involvement of ubiquitination in Mkp-1 degradation. Stability of Mkp-1 is not affected by LPS treatment, consistent with constitutive degradation. U0126, a potent and selective inhibitor of extracellular response kinase (ERK) had negligible effect on the stability of both intrinsic and ectopically expressed Mkp-1, therefore enterocytes, unlike other cell types, do not regulate Mkp-1 degradation via ERK-dependent phosphorylation. C-Truncations of the C-terminal non-catalytic domain (at amino acids 306, 331, and 357), the 306-330 deletion obliterating the potential PEST domains, as well as S296A mutation, but not S323A, S358A, or S363A mutations in potential ERK phosphorylation sites dramatically stabilized Mkp-1. C-terminal fusion of the non-catalytic C-terminal domain of Mkp-1 conferred accelerated degradation on the intrinsically stable green fluorescent protein. According to these results, rapid degradation of Mkp-1 in IEC-6 enterocytes is ubiquitin-proteasome-dependent and ERK-independent. Multiple elements of the C-terminal non-catalytic domain play essential roles in the formation of the complex rapid degradation signal

Nonaccidental Trauma Is an Independent Risk Factor for Mortality Among Injured Infants

ObjectivesThe Centers for Disease Control disclosed over 600,000 cases of child abuse or neglect in 2016. Single-institution studies have shown that nonaccidental trauma (NAT) has higher complication rates than accidental trauma (AT). Nonaccidental trauma is disproportionately represented in infants. We hypothesized that NAT would increase the risk of mortality in infants. This study aims to provide a contemporary descriptive analysis for infant trauma patients and determine the association between NAT and mortality.MethodsInfants (<1 year of age) within the Pediatric Trauma Quality Improvement Program database (2014-2016) were identified. Descriptive statistics (χ2 and t test) were used to compare NAT infants to AT infants. A multivariable logistic regression was used to determine the risk of mortality associated with select variables including NAT.ResultsFrom 14,965 infant traumas, most presented to a level I pediatric trauma center (53.5%) with a median injury severity score of 9. The most common mechanism was falls (48.6%), followed by NAT (14.5%). Overall mortality was 2.1%. Although most NAT infants were white (60.2%), black infants were overrepresented (23.6% vs 18.3%; P < 0.0001) compared with AT infants. The incidence of mortality was higher in NAT infants (41.6% vs 13.9%; P < 0.0001), and they were more likely to have traumatic brain injury (TBI) (63.1% vs 50.6%; P < 0.001). Nonaccidental trauma [odds ratio (OR), 2.48; P < 0.001], hypotension within 24 hours (OR, 8.93; P < 0.001), injury severity score (OR, 1.12; P < 0.001), and severe abbreviated injury scale-head (OR 1.62, P = 0.014) had the highest association with mortality.ConclusionsThis study confirms the incidence of TBI and NAT in infants. Although providers should be vigilant for NAT, suspicion of NAT should prompt close surveillance, as there is a 2-fold increased risk of mortality independent of injury or TBI

Cannulating the contraindicated: effect of low birth weight on mortality in neonates with congenital diaphragmatic hernia on extracorporeal membrane oxygenation

Background/purposeRestrictions for ECMO in neonates include birth weight less than 2kg (BW <2kg) and/or gestational age less than 34weeks (GA <34weeks). We sought to describe their relationship on mortality.MethodsNeonates with a primary diagnosis code of CDH were identified in the Extracorporeal Life Support Organization (ELSO) registry, and logistic regression models were used to examine the effect of BW <2kg and GA <34weeks on mortality.ResultsWe identified 7564 neonates with CDH. The overall mortality was 50%. There was a significantly higher risk of death with unadjusted odds ratio (OR) 2.39 (95% confidence interval [CI]: 1.53-3.74; P<0.01) for BW <2kg neonates. The adjusted OR of death for BW <2kg neonates remained significantly high with over two-fold increase in the odds of mortality when adjusted for potential confounding variables (OR 2.11, 95% CI: 1.30-3.43; P<0.01). However, no difference in mortality was observed in neonates with GA <34weeks.ConclusionsWhile mortality among CDH neonates with a BW <2kg was substantially increased, GA <34weeks was not significantly associated with mortality. Effort should be made to identify the best candidates for ECMO in this high-risk group and develop treatment strategies to optimize their survival.Type of studyCase-Control Study, Retrospective Comparative Study.Level of evidenceLevel III

Management of choledocholithiasis in an infant

Choledocholithiasis is a rare diagnosis in the infant population with poorly defined optimal management. Endoscopic and operative interventions are both technically challenging. Endoscopic retrograde cholangiopancreatography (ERCP), common bile duct exploration and laparoscopic cholecystectomy are typically safe and successful when performed. However, choledocholithiasis has been reported to spontaneously resolve in up to 60% of cases in neonates and infants. We present five cases of choledocholithiasis in infants and a comprehensive review of the literature. Based on our review and experience, we make a recommendation that clinicians should proceed with a selective approach in the management of this disease based on severity of symptoms, co-morbidities, and the available surgical and endoscopic capabilities of each institution

Effects of artificially introduced Enterococcus faecalis strains in experimental necrotizing enterocolitis

Abstract Enterococcus faecalis is a ubiquitous intestinal symbiont and common early colonizer of the neonatal gut. Although colonization with E. faecalis has been previously associated with decreased NEC pathology, these bacteria have been also implicated as opportunistic pathogens. Here we characterized 21 strains of E. faecalis, naturally occurring in 4-day-old rats, for potentially pathogenic properties and ability to colonize the neonatal gut. The strains differed in hemolysis, gelatin liquefaction, antibiotic resistance, biofilm formation, and ability to activate the pro-inflammatory transcription factor NF-κB in cultured enterocytes. Only 3 strains appreciably colonized the neonatal intestine on day 4 after artificial introduction with the first feeding. The best colonizer, strain BB70, effectively displaced maternal E. faecalis and significantly increased NEC pathology. Our results show that colonization with E. faecalis may predispose neonates to NEC