Scott S. Short

Hirschsprung-associated enterocolitis: prevention and therapy

Hirschsprung-associated enterocolitis remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications, such as stricture formation, prophylaxis with rectal washouts, and identification of high-risk individuals. The chapter also details approaches to diagnose Hirschsprung-associated enterocolitis as well as to exclude other etiologies.

Percutaneous liver biopsy: pathologic diagnosis and complications in children.

Objective: The aim of this study was to determine patient factors that predict diagnostic failure or increased risk of bleeding complications following percutaneous liver biopsy in children. Methods: A retrospective review of all children undergoing percutaneous liver biopsy at a single institution between July 2008 and July 2011 was performed. Demographics, comorbid conditions, preprocedural diagnoses/indications, procedural details, laboratory data, pathologic diagnosis, and complications were recorded. Continuous data were analyzed by Wilcoxon test and categorical data by Fisher exact test to determine statistical significance. Results: Two hundred thirteen children (104 girls) with a median age of 7 years (range 1 week–22 years) underwent 328 percutaneous liver biopsies. Nine (4.2%) experienced a decrease in hemoglobin >2 g/dL, 7 required transfusion (3.3%), and 1 patient died (0.5%). Younger age (1.8 vs 84 months, P = 0.05) and lower preprocedural hematocrit (29.3 vs 34.3, P = 0.05) predicted bleeding complications, whereas the number of biopsies, comorbid conditions, and coagulopathy did not. Sixty-three (19.2%) biopsies were insufficient for definitive histologic evaluation on initial biopsy in 57 patients. Twenty-one of 57 patients (37%) underwent repeat percutaneous biopsy and 3 of 57 (8%) underwent surgical biopsy. Biopsy provided definitive diagnosis in 86% of cases when repeat biopsy was performed. Shorter specimen length (1.4 vs 1.7 cm, P < 0.01) and biopsies performed for unexplained elevation of liver function tests (34.9% vs 16.7%, P < 0.01) were predictive of nondiagnosis. Conclusions: Percutaneous liver biopsy is safe with a low rate of bleeding-related complications. Ensuring adequate sample length and careful patient selection may further increase the diagnostic yield.

Advances in Hirschsprung Disease Genetics and Treatment Strategies: An Update for the Primary Care Pediatrician

Hirschsprung disease (HSCR) is a multigenic condition with variable presentation. Most commonly, it presents in the neonatal period as a functional intestinal obstruction secondary to failure of caudal migration of the enteric nervous system. Classically, this manifests as dilated proximal bowel and constricted distal bowel with absent ganglia and hypertrophic nerve trunks. When recognized early, medical and surgical therapies can be instituted to minimize associated morbidity and mortality. This article reviews current understanding of the etiology of HSCR, its multigenic associations, the historical evolution of HSCR diagnosis and treatment, and current HSCR therapies.

Gender impacts mortality after traumatic brain injury in teenagers

BACKGROUND Gender may influence outcomes following traumatic brain injury (TBI) although the mechanism is unknown. Animal TBI studies suggest that gender differences in endogenous hormone production may be the source. Limited retrospective clinical studies on gender present varied conclusions. Pediatric patients represent a unique population as pubescent children experience up-regulation of endogenous hormones that varies dramatically by gender. Younger children do not have these hormonal differences. The aim of this study was to compare pubescent and prepubescent females with males after isolated TBI to identify independent predictors of mortality. METHODS We performed a retrospective review of the National Trauma Data Bank Research Data Sets from 2007 and 2008 looking at all blunt trauma patients 18 years or younger who required hospital admission after isolated, moderate-to-severe TBI, defined as head Abbreviated Injury Scale (AIS) score 3 or greater. We excluded all individuals with AIS score of 3 or greater for any other region to limit the confounding effect of comorbidities. Based on the median age of menarche, we defined two age groups as follows: prepubescent (0–12 years) and pubescent (>12 years). Analysis was performed to compare trauma profiles and outcomes between groups. Our primary outcome measure was in-hospital mortality. RESULTS A total of 20,280 patients met inclusion criteria; 10,135 were prepubescent, and 10,145 were pubescent. Overall mortality was 6.9%, and lower mortality was noted among prepubescent patients compared with pubescent (5.2% vs. 8.6%, p < 0.0001). Although female gender did not predict reduced mortality in the prepubescent cohort (adjusted odds ratio, 1.05; 95% confidence interval, 0.85–1.30; p = 0.63), female gender was associated with reduced mortality in the pubescent (adjusted odds ratio, 0.78; 95% confidence interval, 0.65–0.93; p = 0.007). CONCLUSION In contrast to prepubescent female gender, pubescent female gender predicts reduced mortality following isolated, moderate-to-severe TBI. Endogenous hormonal differences may be a contributing factor and require further investigation. LEVEL OF EVIDENCE Prognostic study, level III.

Smartphones, trainees, and mobile education: implications for graduate medical education.

Smartphones are one of the fastest-growing sectors in the technology industry, and they continue to evolve to combine faster processors, better memory, and more efficient operating systems into a compact handheld device. Smartphones also offer a dynamic tool for use in personal and professional environments.1 The role of smartphones in medicine continues to expand as additional uses and applications emerge. An estimated 80% of physicians, trainees, and medical students use smartphones, and this percentage is expected to increase.2–,4 Smartphones provide a multifaceted platform for mobile health care, allowing users to access a vast amount of information and interact with resources conveniently and quickly.5 Applications range from patient monitoring to use as a tool for diagnosis, to communication and medical education. Recent advances in smartphone technology have led many educators to extend their teaching methods into the mobile learning environment, providing an “anytime, anywhere” approach to learning. Mobile learning has been shown to have efficacy within the traditional classroom environment, and brief communications via short message service (SMS) supplement interactive classroom sessions, resulting in enhanced interest in and attention to classroom activity.6 Given the nature of graduate medical education (GME), where trainees are expected to assimilate a vast amount of information that is constantly evolving, and often are away from traditional classroom settings, the benefits of mobile learning with its uninterrupted access to educational resources can be particularly advantageous. In this perspective, we characterize the current and potential uses of smartphone technology in GME and provide recommendations for future studies on incorporating smartphone technology as an educational platform.

Low doses of Celecoxib attenuate gut barrier failure during experimental peritonitis

The intestinal barrier becomes compromised during systemic inflammation, leading to the entry of luminal bacteria into the host and gut origin sepsis. Pathogenesis and treatment of inflammatory gut barrier failure is an important problem in critical care. In this study, we examined the role of cyclooxygenase-2 (COX-2), a key enzyme in the production of inflammatory prostanoids, in gut barrier failure during experimental peritonitis in mice. I.p. injection of LPS or cecal ligation and puncture (CLP) increased the levels of COX-2 and its product prostaglandin E2 (PGE2) in the ileal mucosa, caused pathologic sloughing of the intestinal epithelium, increased passage of FITC-dextran and bacterial translocation across the barrier, and increased internalization of the tight junction (TJ)-associated proteins junction-associated molecule-A and zonula occludens-1. Luminal instillation of PGE2 in an isolated ileal loop increased transepithelial passage of FITC-dextran. Low doses (0.5–1 mg/kg), but not a higher dose (5 mg/kg) of the specific COX-2 inhibitor Celecoxib partially ameliorated the inflammatory gut barrier failure. These results demonstrate that high levels of COX-2-derived PGE2 seen in the mucosa during peritonitis contribute to gut barrier failure, presumably by compromising TJs. Low doses of specific COX-2 inhibitors may blunt this effect while preserving the homeostatic function of COX-2-derived prostanoids. Low doses of COX-2 inhibitors may find use as an adjunct barrier-protecting therapy in critically ill patients.

Laparoscopic Versus Open Treatment of Congenital Duodenal Obstruction: Multicenter Short-Term Outcomes Analysis

BACKGROUND Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair. PATIENTS AND METHODS Medical records for all cases of congenital duodenal obstruction from 2005 to 2011 at three academic teaching hospitals were retrospectively reviewed. Patients were excluded from the analysis if they had confounding surgical diseases, did not have duodenoduodenostomy during the first hospital admission, had the repair performed before transfer from a referring hospital, or weighed less than 1.7  kg at the time of surgery. Analysis was performed as intention to treat, with laparoscopic converted to open cases included in the laparoscopic group. RESULTS Sixty-four cases were included in the analysis (44 open, 20 laparoscopic). Baseline characteristics were similar between the two groups with the exception that the open group, on average, underwent repair later than the laparoscopic group (6 days versus 4 days, respectively). Seven laparoscopic cases were converted to an open procedure (35%), most commonly for difficulty in exposing the decompressed distal duodenum. Laparoscopic repair did take significantly longer than open repair (145 minutes versus 96 minutes, respectively), but clinical outcomes were similar. Complications were rare and were similar between methods of repair. Two patients in the laparoscopic group required subsequent open revision. CONCLUSIONS Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.

Distinct phenotypes of children with perianal perforating Crohn's disease ☆

PURPOSE Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohns disease (CD). It is unknown whether children who present with PF at the time of diagnosis have a different course than those that develop PF while on therapy. METHODS From a prospective, single institution observational registry of children diagnosed with CD, we identified children with perianal perforating CD, defined as perianal abscesses and/or fistulae. Patients who presented with perianal perforating CD (PF-CD0) were compared to those who developed perianal perforating CD (PF-CD1) after initial diagnosis. RESULTS Thirty-eight of 215 (18%) children with CD had PF-CD during a median follow up of 4.5 years. Patients with PF-CD0 (n=26) tended to be more likely male (81% vs. 50%, p=0.07) and younger (9.3 yrs vs. 12.5 yrs, p=0.02). PF-CD1 (n=12) patients were more likely to require diverting ileostomy (42% vs. 8%, p=0.02) and colectomy (33% vs. 4%, p=0.03). Multivariable analysis predicted increased rate of diverting ileostomy in the PF-CD1 group (p=0.007, OR 19.1, 95% CI 1.6-234.8). CONCLUSION Pediatric CD patients who develop PF while on therapy for CD have a more severe phenotype and are more likely to require diverting ileostomy or colectomy compared to those who present with PF-CD.

Adjuvant treatment of early colon cancer with micrometastases: Results of a national survey

Optimal adjuvant treatment for patients with Stage I/II colon cancer with micrometastases (MM) is unknown. Because there is no known adjuvant treatment‐related benefit, we evaluated whether MM influenced treatment decisions.

Support for blood alcohol screening in pediatric trauma

BACKGROUND Alcohol intoxication in pediatric trauma is underappreciated. The aim of this study was to characterize alcohol screening rates in pediatric trauma. METHODS The Los Angeles County Trauma System Database was queried for all patients aged ≤ 18 years who required admission between 2003 and 2008. Patients were compared by age and gender. RESULTS A total of 18,598 patients met the inclusion criteria; 4,899 (26.3%) underwent blood alcohol screening, and 2,797 (57.1%) of those screened positive. Screening increased with age (3.3% for 0-9 years, 15.1% for 10-14 years, and 45.4% for 15-18 years; P < .01), as did alcohol intoxication (1.9% for 0-9 years, 5.8% 10-14 years, and 27.3% for 15-18 years; P < .01). Male gender predicted higher mortality in those aged 15 to 18 years (adjusted odds ratio, 1.7; P < .01), while alcohol intoxication did not (adjusted odds ratio, .97; P = .84). CONCLUSIONS Alcohol intoxication is common in adolescent trauma patients. Screening is encouraged for pediatric trauma patients aged ≥10 years who require admission.