Mercy Children's Hospital

Mutations in SCN2A, a gene encoding the voltage-gated sodium channel Nav1.2, have been associated with a spectrum of epilepsies and neurodevelopmental disorders. Here, we report the phenotypes of 71 patients and review 130 previously reported patients. We found that (i) encephalopathies with infantile/childhood onset epilepsies (≥3 months of age) occur almost as often as those with an early infantile onset (<3 months), and are thus more frequent than previously reported; (ii) distinct phenotypes can be seen within the late onset group, including myoclonic-atonic epilepsy (two patients), Lennox-Gastaut not emerging from West syndrome (two patients), and focal epilepsies with an electrical status epilepticus during slow sleep-like EEG pattern (six patients); and (iii) West syndrome constitutes a common phenotype with a major recurring mutation (p.Arg853Gln: two new and four previously reported children). Other known phenotypes include Ohtahara syndrome, epilepsy of infancy with migrating focal seizures, and intellectual disability or autism without epilepsy. To assess the response to antiepileptic therapy, we retrospectively reviewed the treatment regimen and the course of the epilepsy in 66 patients for which well-documented medical information was available. We find that the use of sodium channel blockers was often associated with clinically relevant seizure reduction or seizure freedom in children with early infantile epilepsies (<3 months), whereas other antiepileptic drugs were less effective. In contrast, sodium channel blockers were rarely effective in epilepsies with later onset (≥3 months) and sometimes induced seizure worsening. Regarding the genetic findings, truncating mutations were exclusively seen in patients with late onset epilepsies and lack of response to sodium channel blockers. Functional characterization of four selected missense mutations using whole cell patch-clamping in tsA201 cells-together with data from the literature-suggest that mutations associated with early infantile epilepsy result in increased sodium channel activity with gain-of-function, characterized by slowing of fast inactivation, acceleration of its recovery or increased persistent sodium current. Further, a good response to sodium channel blockers clinically was found to be associated with a relatively small gain-of-function. In contrast, mutations in patients with late-onset forms and an insufficient response to sodium channel blockers were associated with loss-of-function effects, including a depolarizing shift of voltage-dependent activation or a hyperpolarizing shift of channel availability (steady-state inactivation). Our clinical and experimental data suggest a correlation between age at disease onset, response to sodium channel blockers and the functional properties of mutations in children with SCN2A-related epilepsy.

In Brief Objective: To determine predictors of survival and of weaning off parenteral nutrition (PN) in pediatric short bowel syndrome (SBS) patients. Summary Background Data: Pediatric SBS carries extensive morbidity and high mortality, but factors believed to predict survival or weaning from PN have been based on limited studies. This study reviews outcomes of a large number of SBS infants and identifies predictors of success. Methods: Multivariate Cox proportional hazards analysis was conducted on 80 pediatric SBS patients. Primary outcome was survival; secondary outcome was ability to wean off PN. Nonsignificant covariates were eliminated. P < 0.05 was considered significant. Results: Over a mean of 5.1 years of follow-up, survival was 58 of 80 (72.5%) and 51 weaned off PN (63.8%). Cholestasis (conjugated bilirubin ≥2.5 mg/dL) was the strongest predictor of mortality (relative risk [RR] 22.7, P = 0.005). Although absolute small bowel length was only slightly predictive, percentage of normal bowel length (for a given infant's gestational age) was strongly predictive of mortality (if <10% of normal length, RR of death was 5.7, P = 0.003) and of weaning PN (if ≥10% of normal, RR of weaning PN was 11.8, P = 0.001). Presence of the ileocecal valve (ICV) also strongly predicted weaning PN (RR 3.9, P < 0.0005); however, ICV was not predictive of survival. Conclusions: Cholestasis and age-adjusted small bowel length are the major predictors of mortality in pediatric SBS. Age-adjusted small bowel length and ICV are the major predictors of weaning from PN. These data permit better prediction of outcomes of pediatric SBS, which may help to direct future management of these challenging patients. A review of 80 pediatric short bowel syndrome patients demonstrated that cholestasis and age-adjusted bowel length, but not the ileocecal valve, are the main predictors of mortality. The combination of bowel length <10% of predicted and cholestasis results in such high mortality that early referral for intestinal transplantation should be considered.

The growth and evolution of telehealth are opening new avenues for efficient, effective, and affordable pediatric health care services in the United States and around the world. However, there remain several barriers to the integration of telehealth into current practice. Establishing the necessary technical, administrative, and operational infrastructure can be challenging, and there is a relative lack of rigorous research data to demonstrate that telehealth is indeed delivering on its promise. That being said, a knowledge of the current state of pediatric telehealth can overcome many of these barriers, and programs are beginning to collaborate through a new pediatric telehealth research network called Supporting Pediatric Research on Outcomes and Utilization of Telehealth (SPROUT). In this report, we provide an update on the landscape of pediatric telehealth and summarize the findings of a recent SPROUT study in which researchers assessed pediatric telehealth programs across the United States. There were >50 programs representing 30 states that provided data on their implementation barriers, staffing resources, operational processes, technology, and funding sources to establish a base understanding of pediatric telehealth infrastructure on a national level. Moving forward, the database created from the SPROUT study will also serve as a foundation on which multicenter studies will be developed and facilitated in an ongoing effort to firmly establish the value of telehealth in pediatric health care.

The coronavirus disease 2019 (COVID-19) pandemic led to an unprecedented demand for health care at a distance, and telehealth (the delivery of patient care using telecommunications technology) became more widespread. Since our 2018 state-of-the-art review assessing the pediatric telehealth landscape, there have been many changes in technology, policy, payment, and physician and patient acceptance of this care model. Clinical best practices in telehealth, on the other hand, have remained unchanged during this time, with the primary difference being the need to implement them at scale.Because of the pandemic, underlying health system weaknesses that have previously challenged telehealth adoption (including inequitable access to care, unsustainable costs in a fee-for-service system, and a lack of quality metrics for novel care delivery modalities) were simultaneously exacerbated. Higher volume use has provided a new appreciation of how patients from underrepresented backgrounds can benefit from or be disadvantaged by the shift toward virtual care. Moving forward, it will be critical to assess which COVID-19 telehealth changes should remain in place or be developed further to ensure children have equitable access to high-quality care.With this review, we aim to (1) depict today's pediatric telehealth practice in an era of digital disruption; (2) describe the people, training, processes, and tools needed for its successful implementation and sustainability; (3) examine health equity implications; and (4) critically review current telehealth policy as well as future policy needs. The American Academy of Pediatrics (AAP) is continuing to develop policy, specific practice tips, training modules, checklists, and other detailed resources, which will be available later in 2021.

OBJECTIVE: To determine whether lung volume changes and breathing pattern parameters differ among 3 devices for delivery of nasal continuous positive airway pressure (CPAP) in premature infants. METHODS: Thirty-two premature infants receiving nasal CPAP for apnea or mild respiratory distress were enrolled. Birth weight was (mean +/- standard deviation) 1081 +/- 316 g, gestational age 29 +/- 2 weeks, age at study 13 +/- 12 days, and fraction of inspired oxygen (FIO(2)) at study.29 +/-.1. Three devices, applied in random order, were studied in each infant: continuous flow nasal CPAP via CPAP prongs, continuous flow nasal CPAP via modified nasal cannula, and variable flow nasal CPAP. After lung recruitment to standardize volume history, changes in lung volume (DeltaV(L)) were assessed at nasal CPAP of 8, 6, 4, and 0 cm H(2)O using calibrated direct current-coupled respiratory inductance plethysmography. RESULTS: DeltaV(L) was significantly greater overall with the variable flow device compared with both the nasal cannula and CPAP prongs. However, DeltaV(L) was not different between the cannula and the prongs. Respiratory rate, tidal volume, thoraco-abdominal asynchrony, and FIO(2) were greater with the modified cannula than for either of the other 2 devices. CONCLUSION: Compared with 2 continuous flow devices, the variable flow nasal CPAP device leads to greater lung recruitment. Although a nasal cannula is able to recruit lung volume, it does so at the cost of increased respiratory effort and FIO(2).

OBJECTIVE: The desire for evidence-based clinical guidelines for nutritional support of the preterm infant has been identified. Published evidence has not yielded clear guidelines about the best method of delivery, substrate use, or appropriate outcome measure to evaluate nutrition support. In addition, reports on research of nutrition support often fail to give the most rudimentary process necessary to improve quality in various unit settings. METHODS: The Vermont Oxford Network "Got Milk" focus group developed eight potentially better practices for nutrition support, implementation strategies for these practices, and a comprehensive appraisal process to measure nutrition outcome in preterm infants. RESULTS: After implementation of the potentially better practices, all participating institutions showed earlier initiation of nutrition support, earlier attainment of adequate energy intakes, reduced delay in reaching full enteral feeds, more consistent nutrition support practice, decreased length of stay, cost savings, and improved growth at time of discharge. CONCLUSIONS: Development and implementation of evidence-based better nutrition support practices in neonates led to improved nutrient intake and growth with reduced length of stay and related costs. Consistent, comprehensive, multidisciplinary appraisal of practice is an integral component of improving nutrition outcomes in the neonatal population.

BACKGROUND: Constant-flow nasal continuous positive airway pressure (NCPAP) often is used in preterm neonates to recruit and maintain lung volume. Physical model studies indicate that a variable-flow NCPAP device provides more stable volume recruitment with less imposed work of breathing (WOB). Although superior lung recruitment with variable-flow NCPAP has been demonstrated in preterm neonates, corroborating WOB data are lacking. OBJECTIVE: To measure and compare WOB associated with the use of variable-flow versus constant-flow NCPAP in preterm neonates. METHODS: Twenty-four preterm infants who were receiving constant-flow NCPAP (means, SD) and had birth weight of 1024 +/- 253 g, gestational age of 28 +/- 1.7 weeks, age of 14 +/- 13 days, and FIO(2) of 0.3 +/- 0.1 were studied. Variable-flow and constant-flow NCPAP were applied in random order. We measured changes in lung volume and tidal ventilation (V(T)) by DC-coupled/calibrated respiratory inductance plethysmography as well as esophageal pressures at NCPAP of 8, 6, 4, and 0 cm H(2)O. Inspiratory WOB (WOB(I)) and lung compliance were calculated from the esophageal pressure and V(T) data using standard methods. WOB was divided by V(T) to standardize the results. RESULTS: WOB(I) decreased at all CPAP levels with variable-flow NCPAP, with a maximal decrease at 4 cm H(2)O. WOB(I) increased at all CPAP levels with constant-flow CPAP. Lung compliance increased at all NCPAP levels with variable-flow, with a relative decrease at 8 cm H(2)O, whereas it increased only at 8 cm H(2)O with constant-flow NCPAP. Compared with constant-flow NCPAP, WOB(I) was 13% to 29% lower with variable-flow NCPAP. CONCLUSION: WOB(I) is decreased with variable-flow NCPAP compared with constant-flow NCPAP. The increase in WOB(I) with constant-flow NCPAP indicates the presence of appreciable imposed WOB with this device. Our study, performed in neonates with little lung disease, indicates the possibility of lung overdistention at CPAP of 6 to 8 cm H(2)O with the variable-flow device. Further study is necessary to determine the efficacy of variable-flow NCPAP in neonates with significant lung disease and its use over extended periods of time.continuous-flow and variable-flow NCPAP, work of breathing, premature neonates, lung compliance.

Importance: Increasing evidence indicates that acute kidney injury (AKI) occurs frequently in children and young adults and is associated with poor short-term and long-term outcomes. Guidance is required to focus efforts related to expansion of pediatric AKI knowledge. Objective: To develop expert-driven pediatric specific recommendations on needed AKI research, education, practice, and advocacy. Evidence Review: At the 26th Acute Disease Quality Initiative meeting conducted in November 2021 by 47 multiprofessional international experts in general pediatrics, nephrology, and critical care, the panel focused on 6 areas: (1) epidemiology; (2) diagnostics; (3) fluid overload; (4) kidney support therapies; (5) biology, pharmacology, and nutrition; and (6) education and advocacy. An objective scientific review and distillation of literature through September 2021 was performed of (1) epidemiology, (2) risk assessment and diagnosis, (3) fluid assessment, (4) kidney support and extracorporeal therapies, (5) pathobiology, nutrition, and pharmacology, and (6) education and advocacy. Using an established modified Delphi process based on existing data, workgroups derived consensus statements with recommendations. Findings: The meeting developed 12 consensus statements and 29 research recommendations. Principal suggestions were to address gaps of knowledge by including data from varying socioeconomic groups, broadening definition of AKI phenotypes, adjudicating fluid balance by disease severity, integrating biopathology of child growth and development, and partnering with families and communities in AKI advocacy. Conclusions and Relevance: Existing evidence across observational study supports further efforts to increase knowledge related to AKI in childhood. Significant gaps of knowledge may be addressed by focused efforts.

Synchronized nasal intermittent positive pressure ventilation (SNIPPV) is non-invasive respiratory support that delivers ventilator breaths via the nasal prongs. We hypothesized that SNIPPV is more effective than nasal continuous positive airway pressure (NCPAP) in premature neonates due to decreased work of breathing (WOB). Fifteen infants (BW: 1,367 +/- 325 g, GA: 29.5 +/- 2.4 weeks) were studied on (a) NCPAP at 5 cmH(2)O (NCPAP5) and (b) three increasing SNIPPV settings achieved by NCPAP5 with additional delivered peak inspiratory pressures (PIP) of 10, 12, and 14 cmH(2)O. Tidal volumes and transpulmonary pressures were estimated via calibrated respiratory inductance plethysmography (RIP) and esophageal pressures, respectively. Inspiratory (WOB(insp)), resistive (RWOB), and elastic (WOB(E)) components of WOB were calculated using standard methods. Compared to NCPAP5, (a) WOB(insp) and RWOB were significantly lower with SNIPPV12, and were similarly lower with SNIPPV14 and (b) WOB(E) was significantly lower only with SNIPPV14. WOB components did not differ significantly for the three SNIPPV settings. Tidal volume, respiratory rate (RR), minute ventilation, compliance, and phase angle were similar for all four measurements. In conclusion, compared to NCPAP, the addition of ventilator-delivered PIP during SNIPPV decreases WOB in premature infants.

Growth hormone (GH) insensitivity syndrome (GHIS) is a rare clinical condition in which production of insulin-like growth factor 1 is blunted and, consequently, postnatal growth impaired. Autosomal-recessive mutations in signal transducer and activator of transcription (STAT5B), the key signal transducer for GH, cause severe GHIS with additional characteristics of immune and, often fatal, pulmonary complications. Here we report dominant-negative, inactivating STAT5B germline mutations in patients with growth failure, eczema, and elevated IgE but without severe immune and pulmonary problems. These STAT5B missense mutants are robustly tyrosine phosphorylated upon stimulation, but are unable to nuclear localize, or fail to bind canonical STAT5B DNA response elements. Importantly, each variant retains the ability to dimerize with wild-type STAT5B, disrupting the normal transcriptional functions of wild-type STAT5B. We conclude that these STAT5B variants exert dominant-negative effects through distinct pathomechanisms, manifesting in milder clinical GHIS with general sparing of the immune system.

OBJECTIVE: To provide a comprehensive, evidence-based review of helium-oxygen gas mixtures (heliox) in the management of pediatric respiratory diseases. DATA SOURCE: A thorough, computerized bibliographic search of the preclinical and clinical literature regarding the properties of helium and its application in pediatric respiratory disease states. DATA SYNTHESIS: After an overview of the potential benefits and technical aspects of helium-oxygen gas mixtures, the role of heliox is addressed for asthma, aerosolized medication delivery, upper airway obstruction, postextubation stridor, croup, bronchiolitis, and high-frequency ventilation. The available data are objectively classified based on the value of the therapy or intervention as determined by the study design from which the data are obtained. CONCLUSIONS: Heliox administration is most effective during conditions involving density-dependent increases in airway resistance, especially when used early in an acute disease process. Any beneficial effect of heliox should become evident in a relatively short period of time. The medical literature supports the use of heliox to relieve respiratory distress, decrease the work of breathing, and improve gas exchange. No adverse effects of heliox have been reported. However, heliox must be administered with vigilance and continuous monitoring to avoid technical complications.

BACKGROUND: The combined inhibition of insulin-growth factor type 1 receptor (IGF-1R) and the mammalian target of rapamycin (mTOR) has shown activity in preclinical models of pediatric sarcoma and in adult sarcoma patients. We evaluated the activity of the anti-IGF-1R antibody cixutumumab with the mTOR inhibitor temsirolimus in patients with relapsed or refractory Ewing sarcoma, osteosarcoma, rhabdomyosarcoma, and other soft tissue sarcoma, using the recommended dosages from a pediatric phase I trial. METHODS: Cixutumumab 6 mg/kg and temsirolimus 8 mg/m(2) were administered intravenously once weekly in 4-week cycles to patients <30 years. Temsirolimus was escalated to 10 mg/m(2) for subsequent cycles in patients who did not experience unacceptable first-cycle toxicity. A two-stage design was used to identify a response rate <10 or >35% for each tumor-specific cohort. Tumor tissue was analyzed by immunohistochemistry for potential biomarkers of response. RESULTS: Forty-three evaluable patients received a median of 2 cycles (range 1-7). No objective responses were observed, and 16% of patients were progression-free at 12 weeks. Dose-limiting toxicity was observed in 15 (16%) of 92 cycles. The most common toxicities were mucositis, electrolyte disturbances, and myelosuppression. The majority of patients receiving a second cycle were not eligible for temsirolimus escalation due to first-cycle toxicity. The lack of objective responses precluded correlation with tissue biomarkers. CONCLUSIONS: Despite encouraging preclinical data, the combination of cixutumumab and temsirolimus did not result in objective responses in this phase II trial of pediatric and young adults with recurrent or refractory sarcoma.

BACKGROUND: This article summarizes our 10-year multicenter experience with transplantation of 50 blood group A2 and A2B kidneys into B and O patients. METHODS: Since 1986, we have transplanted kidneys from 46 cadaver donors and 4 living donors who were blood group A2 (47 donors) or A2B (3 donors) into 19 B and 31 O patients. In 1991, we began allocating these kidneys preferentially to B and O recipients who were selected based on a history of low (< or =4) anti-A IgG isoagglutinin titers. Immunosuppression was no different from that used in ABO-compatible grafts. RESULTS: The 1-month function rate before thus selecting the patients was 68% (19/28), but is now 94% (17/18). Two-year cadaver-donor graft survival with this selection method is 94%, compared with 88% for 640 concurrent and consecutive ABO-compatible transplants (log-rank, 0.15). All four living-related transplants are still functioning, with a mean follow-up of 71 months. Since we began allocating A2 kidneys preferentially to B and O recipients, the percentage of the B patients who received A2 or A2B kidneys has increased from 29% (8/28) to 55% (10/18). CONCLUSIONS: Transplantation of A2 or A2B kidneys into B and O patients is clinically equivalent to that of ABO-compatible transplantation when recipients are selected by low pretransplant anti-A titer histories. This approach increases access of blood group B recipients to kidneys.

Bed sharing has been promoted as facilitating breastfeeding but also may increase risks for sudden, unexpected infant deaths. This prospective cohort study was performed to determine the prevalence of adult and infant bed sharing and its association with maternal and infant characteristics. Demographic data were collected from 10,355 infant-mother pairs at birth hospitals in Eastern Massachusetts and Northwest Ohio, and follow-up data were collected at 1, 3, and 6 months by questionnaire. Associations with bed sharing were estimated using odds ratios and 95% confidence intervals from multiple logistic regression models while adjusting for confounding variables. At 1, 3, and 6 months, 22%, 14%, and 13% of infant-mother pairs shared a bed, respectively. On multivariate analysis, race/ethnicity and breastfeeding seemed to have the strongest association with bed sharing. These factors need to be considered in any comprehensive risk to benefit analysis of bed sharing.

Febrile non-haemolytic or allergic reactions occur in 0.1-30% of transfusions; physicians often premedicate patients with acetaminophen or diphenhydramine to prevent these reactions. The effectiveness of this practice has not been demonstrated. In this retrospective review of all transfusions at our institution during 2002, 385 patients received 7900 evaluable leucoreduced, irradiated blood products (4280 single-donor apheresis platelets and 3620 packed red blood cells). Febrile reactions occurred in 0.95% of 4108 transfusions with, and 0.53% of 3792 transfusions without, acetaminophen premedication. Allergic reactions occurred in 0.90% of 4315 transfusions with, and 0.56% of 3585 transfusions without, diphenhydramine premedication. In a multivariate analysis that adjusted for age, patient category, transfusion location, product, transfusion history, and reaction history, premedication with acetaminophen was associated with a statistically non-significant increase in the odds of a febrile reaction (odds ratio 1.74; 95% confidence interval 0.71-4.23; P = 0.22), and diphenhydramine with a non-significant increase in allergic reactions (odds ratio 1.74; 95% confidence interval 0.99-3.06; P = 0.054). Reactions occurred in only 1.3% of the 518 transfusions to patients with a history of two or more prior reactions. Febrile and allergic transfusion reactions were rare in paediatric patients transfused with leucoreduced, irradiated blood products, whether premedication was used or not.

OBJECTIVES: To assess general pediatricians' screening practices for sleep-related issues and assess their knowledge on common sleep complaints in children, their perceived barriers to screening for sleep issues, and whether they have received training regarding sleep issues. METHODS: A national random sample (n = 700) of general pediatricians who were members of the American Academy of Pediatrics were sent a valid and reliable questionnaire on sleep problems in youth. RESULTS: A total of 346 pediatricians (49%) responded. The vast majority (96%) indicated that they believed it was their job to counsel patients/guardians regarding sleep hygiene, yet few pediatricians (18%) had ever received formal training on sleep disorders. Those who did not screen for sleep problems spent significantly less time with each patient and perceived significantly more barriers to screening. Pediatricians who had received training about sleep disorders had significantly higher knowledge scores on sleep problems, perceived significantly fewer barriers to screening, and reported significantly higher confidence scores regarding counseling patients/guardians on sleep problems. CONCLUSION: These findings support the need for formal education on sleep disorders for all pediatricians.

STUDY DESIGN: Cadaver study. OBJECTIVE: To evaluate with direct observation the errors made when novice resident surgeons place thoracic pedicle screws. To determine how many specimens need to be instrumented to assure an improvement in accuracy to currently published levels. SUMMARY OF BACKGROUND DATA: Thoracic pedicle screw instrumentation has been shown to provide numerous benefits in spinal deformity surgery including 3 column fixation of the spinal elements, decreased need for thoracoplasty or anterior thoracic release and decreased operative time and blood loss. METHODS: Three orthopaedic residents inexperienced in pedicle screw placement received an introductory teaching session. Intact thoracic vertebral body specimens were harvested from 15 cadaver spines. Each vertebral body was mounted on a clear Plexiglas frame with only the posterior surface anatomy visible to the surgeon. Each resident surgeon instrumented 5 thoracic spines verbalizing all perceived pedicle wall violations as they occurred. An observer recorded the accuracy of the gearshift probe, flexible probe, tap, and screw placement. Critically perforated screws were defined as a greater than 2 mm breach of the pedicle wall. RESULTS: Two hundred ninety-seven pedicles in 149 intact vertebral body specimens were instrumented. Eighty-five (29%) screws were not fully within the pedicle. Sixty-three (74%) were noncritical violations and 22 (26%) were critical violations. There were 18 (21%) screw violations not perceived by the surgeon to be outside the pedicle. There was a decrease in the proportion of total screw violations by the third cadaver (P < 0.001) and in critical screw violations by the fourth cadaver (P = 0.01). CONCLUSION: Novice resident surgeons placing thoracic pedicle screws in cadavers were able to significantly improve by the fourth cadaver to accuracy levels documented in the literature. Surgeons in training shouldpractice these skills in the laboratory before proceeding to the operating room.

BACKGROUND: Neonates receiving parenteral nutrition (PN) are at risk for PN-associated cholestasis (PNAC); however, no preventive factors for PNAC have been clearly identified. Despite reports suggesting that taurine may prevent PNAC in neonates, such an effect of taurine has not yet been definitively demonstrated. We determined whether taurine supplementation reduces the incidence of PNAC in premature or critically ill neonates. METHODS: This study was part of a prospective, randomized, multi-institutional trial designed to assess cholecystokinin vs placebo as a potential preventive therapy of PNAC. Taurine supplementation of PN varied between institutions. The presence or absence of taurine in PN was analyzed by multivariate analysis, with a primary outcome measure of serum conjugated bilirubin (CB) as a measure of PNAC. RESULTS: Taurine reduced PNAC in premature infants (estimated maximum CB [95% confidence interval] 0.50 mg/dL [-0.17 to 1.18] for those receiving taurine, vs 3.45 mg/dL [1.79-5.11] for neonates not receiving taurine, approaching significance, p = .07). Taurine significantly reduced PNAC in infants with necrotizing enterocolitis (NEC; estimated maximum CB 4.04 mg/dL [2.85-5.23], NEC infants receiving taurine, vs 8.29 mg/dL [5.61-10.96], NEC infants not receiving taurine, p < .01). There were too few neonates with surgical anomalies to evaluate the effect of taurine in this group. CONCLUSIONS: Within specific subgroups of neonatal patients, taurine supplementation does offer a very significant degree of protection against PNAC. Patients with NEC or severe prematurity are most likely to benefit substantially from taurine supplementation.

BACKGROUND: Nonoperative management (NOM) is standard of care for most pediatric blunt liver and spleen injuries (BLSI); only 5% of patients fail NOM in retrospective reports. No prospective studies examine failure of NOM of BLSI in children. The aim of this study was to determine the frequency and clinical characteristics of failure of NOM in pediatric BLSI patients. METHODS: A prospective observational study was conducted on patients 18 years or younger presenting to any of 10 Level I pediatric trauma centers April 2013 and January 2016 with BLSI on computed tomography. Management of BLSI was based on the Arizona-Texas-Oklahoma-Memphis-Arkansas Consortium pediatric guideline. Failure of NOM was defined as needing laparoscopy or laparotomy. RESULTS: A total of 1008 patients met inclusion; 499 (50%) had liver injury, 410 (41%) spleen injury, and 99 (10%) had both. Most patients were male (n = 624; 62%) with a median age of 10.3 years (interquartile range, 5.9, 14.2). A total of 69 (7%) underwent laparotomy or laparoscopy, but only 34 (3%) underwent surgery for spleen or liver bleeding. Other (nonexclusive) operations were for 21 intestinal injuries; 15 hematoma evacuations, washouts, or drain placements; 9 pancreatic injuries; 5 mesenteric injuries; 3 diaphragm injuries; and 2 bladder injuries. Patients who failed were more likely to receive blood (52 of 69 vs. 162 of 939; p < 0.001) and median time from injury to first blood transfusion was 2.3 hours for those who failed versus 5.9 hours for those who did not (p = 0.002). Overall mortality rate was 24% (8 of 34) in those who failed NOM due to bleeding. CONCLUSION: NOM fails in 7% of children with BLSI, but only 3% of patients failed for bleeding due to liver or spleen injury. For children failing NOM due to bleeding, the mortality was 24%. LEVEL OF EVIDENCE: Therapeutic study, level II.

Attention-Deficit/Hyperactivity Disorder (ADHD) is a neurodevelopmental disorder increasing in prevalence. Although there is limited evidence to support treating ADHD with mineral/vitamin supplements, research does exist showing that patients with ADHD may have reduced levels of vitamin D, zinc, ferritin, and magnesium. These nutrients have important roles in neurologic function, including involvement in neurotransmitter synthesis. The aim of this paper is to discuss the role of each of these nutrients in the brain, the possible altered levels of these nutrients in patients with ADHD, possible reasons for a differential level in children with ADHD, and safety and effect of supplementation. With this knowledge, clinicians may choose in certain patients at high risk of deficiency, to screen for possible deficiencies of magnesium, vitamin D, zinc, and iron by checking RBC-magnesium, 25-OH vitamin D, serum/plasma zinc, and ferritin. Although children with ADHD may be more likely to have lower levels of vitamin D, zinc, magnesium, and iron, it cannot be stated that these lower levels caused ADHD. However, supplementing areas of deficiency may be a safe and justified intervention.