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Extracorporeal life support for neonatal respiratory failure has decreased, but utilization and outcome of cardiac extracorporeal life support are not well characterized. Among neonates born 1996-2000, our objects were to evaluate changes in utilization and outcome of cardiac extracorporeal life support and characterize correlates of survival.Retrospective analysis of Extracorporeal Life Support Organization Registry data.Intensive care units participating in the ELSO registry.Patients placed on extracorporeal life support for center-specified "cardiac support" at/=30 days of age from 1996 to 2000. Patients with hypoplastic left heart syndrome were also analyzed separately.None.Patient characteristics and correlates of survival to discharge or transfer were analyzed by chi-square, Student's t-test, and logistic regression analysis. Neonates placed on cardiac extracorporeal life support increased from 112 in 1996 to 200 in 2000 (total n = 740). Overall survival was 34.2%: 28% for hypoplastic left heart syndrome and 35.4% for nonhypoplastic left heart syndrome. For the overall group, no significant correlations were found between survival and year on extracorporeal life support, multiple runs, or diagnosis of hypoplastic left heart syndrome. Diagnoses of transposition of the great arteries (p = .03) or persistent pulmonary hypertension of the neonate (p = .004) and extracorporeal life support at3 days (p = .003) were associated with higher survival. Survivors had fewer mean extracorporeal life support hours (125.5 +/- 121.4 vs. 159.0 +/- 127.6, p = .0006). Logistic regression confirmed significant bivariate findings. A total of 118 hypoplastic left heart syndrome patients were reported from 1996 to 2000. Extracorporeal life support at15 days was associated with improved survival among hypoplastic left heart syndrome patients (p = .03), and survivors had fewer mean extracorporeal life support hours (89.3 +/- 52.3 vs. 147.5 +/- 129.7, p = .015). Logistic regression showed that only greater number of hours on extracorporeal life support was independently associated with nonsurvival.Neonatal cardiac extracorporeal life support use increased substantially from 1996 to 2000, with survival to discharge or transfer in more than one third of patients. Hypoplastic left heart syndrome was not associated with nonsurvival. Fewer hours on extracorporeal life support, diagnoses of persistent pulmonary hypertension of the neonate and transposition of the great arteries, and extracorporeal life support at3 days were associated with survival.

BACKGROUND: Fungal infection can be a significant complication for the critically ill neonate. However, the usefulness of extensive radiologic and ophthalmologic investigations in this population has not been thoroughly elucidated. OBJECTIVE: To report the incidence of organ fungal involvement diagnosed by ancillary testing (echocardiogram, ophthalmologic examination, brain imaging, and renal ultrasound (RUS)) among neonatal intensive care unit (NICU) patients with Candida infection. METHODS: This was a single center review of all NICU patients with Candida-positive cultures of blood, urine, peritoneal fluid, endotracheal tube aspirate, or cerebrospinal fluid from January 1, 1997 to June 1 2002. Data regarding the number of positive cultures, species isolated, and presence of specific risk factors and clinical symptoms were recorded for each case, as well as occurrence, timing and results of ancillary testing. RESULTS: In all, 66 patients had at least one positive culture for Candida. The majority (71%) were

The diagnosis of necrotizing enterocolitis (NEC) is made from a combination of clinical and radiographic findings. There are no useful screening biochemical markers of intestinal injury. The serum concentration of cytosolic beta-glucosidase (CBG), an enzyme found primarily in enterocytes, is markedly elevated in animal models of ischemia and bowel obstruction. We hypothesized that in a rat model of NEC, serum CBG activity would significantly increase before microscopic evidence of severe intestinal injury. Cohorts of 2-wk-old Sprague-Dawley rats (n = 10/cohort) were anesthetized and underwent laparotomy with occlusion of the superior mesenteric artery (SMA). Platelet-activating factor (200 microg/animal) was injected in the proximal duodenum. Serum and intestinal samples were obtained at time 0 (control) and 30, 60, and 90 min of ischemia (I) and after 90 min of I followed by 60 min of reperfusion (I/R). Histopathologic injury was categorized as either no or minimal injury or mural necrosis by two masked investigators and CBG activity was measured by ELISA. Data were analyzed with Fisher's exact test and ANOVA. Only the I/R group had significantly greater mural necrosis compared with the control group (90% versus 0%, respectively, p < 0.001). In contrast, CBG activity was significantly elevated after only 90 min of I and after I/R (15.1 +/- 5.6 and 16.4 +/- 4.3 units/mL, respectively, p < 0.05). We conclude that serum CBG is elevated before transmural intestinal injury in this model and may have utility as an early marker of ischemia in patients at risk for NEC.

Background/Purpose: More than 97% of the clinical research in pediatric surgery consists of retrospective data. Although these studies have significant limitations, they fundamentally shape clinical practice within the field. In this report, the authors describe the development and potential applications of a standardized quality assessment scale designed for retrospective studies in pediatric surgery. Methods: The authors developed a comprehensive quality assessment instrument incorporating 30 items within 3 subscales. These subscales were designed to assess 3 integral components of study design: clinical relevance, reporting methodology, and the strength of conclusions. Global quality ratings (poor, fair, or good) were derived by combining scores from each subscale. To examine inter-rater reliability, 10 retrospective studies from the pediatric surgery literature were assessed with the instrument by 6 independent reviewers. Results: Inter-rater reliability was excellent as assessed by the level of agreement for all items within the instrument (84.6% concordance, n=1,573 total item-choices) and for individual subscales (range, 73.3% to 85.8%, n=60 to 1,258). The extent of agreement among reviewers was 82% for classifying reports into qualitative categories by global quality scores (n = 60). The ranking of papers by individual reviewers was highly predictive of overall ranking by mean quality scores (n = 60 rank pairs, r=0.83; P Conclusions: The authors have developed a standardized and reliable quality assessment scale for the analysis of retrospective data in pediatric surgery. Potential applications include: (1) providing the practicing surgeon with a knowledge base to critically evaluate published retrospective data; (2) providing a standardized methodology for the systematic review of existing retrospective data; and (3) developing standardized reporting guidelines for use in peer-reviewed journals. J Pediatr Surg 38:390-396. Copyright 2003, Elsevier Science (USA). All rights reserved.

Tandem mass spectrometry (MS/MS) has been introduced in several newborn screening programs for the detection of a large number of inborn errors of metabolism, including fatty acid oxidation disorders (FAOD). Early identification and treatment of FAOD have the potential to improve outcome and may be life-saving in some cases; an estimated 5% of sudden infant deaths are attributable to undiagnosed disorders of fatty acid oxidation. We report very early neonatal presentations of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein (TFP) deficiencies confirmed by molecular analysis. Both patients had cardiorespiratory collapse and hypoglycemia, without a history of maternal pregnancy complications. Retrospective MS/MS analysis of the original newborn screening blood spots revealed characteristic acylcarnitine profiles. These cases are among the earliest reported presentations of LCHAD and TFP deficiencies and further illustrate the potential of MS/MS as a valuable tool for newborn screening of FAOD. However, timely analysis and reporting of results to clinicians are essential, because these disorders can manifest in the first few days of life.

Aim To determine if a real-time audiovisual link with a neonatologist, termed video-assisted resuscitation or VAR, improves provider performance during a simulated neonatal resuscitation scenario. Methods Using high-fidelity simulation, 46 study participants were presented with a neonatal resuscitation scenario. The control group performed independently, while the intervention group utilized VAR. Time to effective ventilation was compared using Wilcoxon rank sum tests. Providers' use of the corrective steps for ineffective ventilation per the NRP algorithm was compared using Cochran–Armitage trend tests. Results The time needed to establish effective ventilation was significantly reduced in the intervention group when compared to the control group (mean time 2min 42s versus 4min 11s, p In the setting of ineffective ventilation, only 35% of control subjects used three or more of the first five corrective steps and none of them used all five steps. Providers in the control group most frequently neglected to open the mouth and increase positive pressure. In contrast, all of those in the intervention group used all of the first five corrective steps, p All participants in the control group decided to intubate the infant to establish effective ventilation, compared to none in the intervention group, p Conclusion Using VAR during a simulated neonatal resuscitation scenario significantly reduces the time to establish effective ventilation and improves provider adherence to NRP guidelines. This technology may be a means for regional centers to support local providers during a neonatal emergency to improve patient safety and improve neonatal outcomes.

Prematurity is associated with poor neurodevelopmental outcomes, and laws mandate the provision of early intervention services to those infants with disability. However, it is often difficult to identify early which infants would benefit most from these services. The Neurobiologic Risk Score (NBRS) and the Neurodevelopmental Risk Exam (NRE) are instruments used to assess infants at near-term corrected age. These instruments have been shown to correlate with later developmental outcomes. However, the environment of the neonatal intensive care unit (NICU) has changed since the NBRS and NRE were first validated, and it is not known whether they are still able to accurately predict future developmental outcomes. The objective of this study was therefore to examine the ability of the NBRS and NRE, both alone and in combination with socio-economic variables, to predict future developmental outcomes in the contemporary NICU. The subjects were 219 neonates of less than 32 weeks' gestational age discharged from the NICU between November 2001 and December 2006 who had undergone both the NBRS and NRE. Infants were assessed at chronological age 6, 12 and 24 months, with developmental quotients being assigned at these ages. Parental socio-economic data were also collected and analysed. The hypothesis was that the NBRS and NRE would be less effective at predicting neurodevelopmental outcomes in the contemporary NICU. The best measure of future developmental outcome is likely to need to include both neurobiological and socio-economic risk factors.

The objective of this study was to identify risk factors of significance for acquisition of Pseudomonas aeruginosa by children with cystic fibrosis (CF). Our working hypothesis is that exposure of infants and young children with CF to older, infected patients increases their risk for acquiring this organism. A special opportunity arose to study this question in detail, as we have been performing a randomized clinical trial of neonatal screening for CF throughout the state of Wisconsin during the period of 1985-1994. Patients were selected for this study based on either early identification through screening or diagnosis by standard methods. A longitudinal protocol employed at Wisconsin's two CF Centers includes routine cultures of respiratory secretions and collection of clinical, demographic, and activity information on patients and their families. Previous observations in our trial revealed that one center at an old hospital in an urban location showed a significantly shorter time to acquisition of P. aeruginosa for CF patients followed there. To study the center effect further, we performed statistical analyses using survival curves and stepwise regression analysis of all life history covariates available. The results of these analyses showed that the statistically significant correlations involve the following risk factors: 1) center and old hospital (r=0.42); 2) center and original physician (r=0.61); 3) center and exposure to pseudomonas-positive patients (r=0.29); and 4) population density and urban location (r=0.49). The final statistical model demonstrated that increased risk due to aerosol use (odds ratio=3.45, P=0.014) and a protective effect associated with education of the mother (odds ratio=0.81, P=0.024) were the most significant factors for acquisition of P. aeruginosa. The previously observed center effect was confined to the 1985-1990 interval at the old hospital (odds ratio=4.43, P < 0.001). We conclude that multiple factors are involved in increasing the risk of young children with CF to acquire P. aeruginosa, and that the observed center effect can best be explained by a combination of factors. These results suggest that facilities and methods used to care for young children with CF can significantly influence their likelihood of acquiring pseudomonas in the respiratory tract.

Prenatal History: ### Birth History: ### Hospital Course: The infant had respiratory distress syndrome that required mechanical ventilation and three doses of exogenous surfactant; he was extubated to nasal continuous positive airway pressure on the eighth postnatal day. Additionally, he received three doses of prophylactic indomethacin during the first 72 hours after birth. His ductus arteriosus was closed upon completion of indomethacin treatment. He was treated with cefotaxime and ampicillin for the first eight postnatal days for culture-negative presumed sepsis. Within the first 24 hours of birth, he received dopamine, an insulin infusion to treat hyperkalemia, and nitric oxide for 36 hours due to refractory hypoxemia. His nutritional requirements were delivered with total parenteral nutrition; enteral feedings had not been started. He did not receive postnatal steroids. Nine days after birth, he developed inguinal and scrotal discoloration (Fig. 1). These findings were associated with hypotension (mean arterial pressure, 20 mm Hg), decreased urine output (0.13 mL/kg per hour), hypothermia (core body temperature of 96.4°F [35.8°C]), and five episodes of …

Automated monitoring of the QT interval is increasingly common in a variety of clinical settings. A better understanding of how the heart-rate-corrected QT interval (QTc) evolves in early postnatal life is needed before its clinical utility in neonates can be determined. This study aimed to use real-time bedside monitoring as a tool to describe the QTc evolution of premature neonates during the first week of life. All neonates born at a gestation age (GA) of 31 weeks or later and admitted to the level 2 intensive care nursery of the authors’ institution between December 2012 and March 2013 were included in this study. The authors prospectively collected QTc values at 15-min intervals during the first week of life, then used two-way analysis of variance (ANOVA) to compare these data among three GA cohorts: 31 to

For more than a decade there has been considerable interest in the role of QT interval prolongation in the pathogenesis of sudden infant death syndrome. It has been proposed that the QT interval is a surrogate marker for autonomic instability and can be used to identify infants at risk for significant morbidity and mortality, including sudden infant death syndrome. We present the case of an infant that experienced a significant increase in his QTc, as detected by continuous QTc monitoring in the NICU after repositioning from a supine to prone position. This increase from a 413 ± 6 millisecond baseline average to 500 milliseconds was sustained for 2 hours and associated with clinically relevant apnea that ultimately required repositioning of the infant back to the supine position. Repositioning resulted in an immediate decrease of the QTc back to the previous baseline and termination of the apneic events. This case demonstrates an example of how the use of continuous QTc monitoring in the NICU setting may be used to detect QTc-accentuating factors in real time and identify situations that cause perturbations in an infant’s autonomic nervous system.

In 2005, therapeutic hypothermia (TH) was used in few American neonatal intensive care units (NICUs) with great variability in practices. We hypothesized that TH would be used with greater frequency and uniformity today.We surveyed directors of 797 NICUs queried in our prior study to determine attitudes toward and practices of TH.Of the 781 participants with valid addresses, we received completed surveys from 330 (42.3%). There was an increase in the number of respondents who believed that TH is effective (85% versus 31%, p 0.0001). More NICUs used TH (50% versus 6%, p 0.0001) and nearly all not offering TH transferred eligible neonates to centers that did (97% versus 29%, p 0.0001). There has been increased standardization of TH practices with regard to enrollment criteria, duration, and methods of monitoring.TH has become standard of care for the treatment of HIE in the United States. Most NICUs that use TH adhere to protocols, but variation still exists in TH practices.

The following guidelines are an interpretation of the evidence presented in the 2010 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations 1 ). They apply primarily to newly born infants undergoing transition from intrauterine to extrauterine life, but the recommendations are also applicable to neonates who have completed perinatal transition and require resuscitation during the first few weeks to months following birth. Practitioners who resuscitate infants at birth or at any time during the initial hospital admission should consider following these guidelines. For the purposes of these guidelines, the terms newborn and neonate are intended to apply to any infant during the initial hospitalization. The term newly born is intended to apply specifically to an infant at the time of birth. Approximately 10% of newborns require some assistance to begin breathing at birth. Less than 1% require extensive resuscitative measures. 2,3 Although the vast majority of newly born infants do not require intervention to make the transition from intrauterine to extrauterine life, because of the large total number of births, a sizable number will require some degree of resuscitation. Those newly born infants who do not require resuscitation can generally be identified by a rapid assessment of the following 3 characteristics: ● Term gestation? ● Crying or breathing? ● Good muscle tone? If the answer to all 3 of these questions is “yes,” the baby does not need resuscitation and should not be separated from the mother. The baby should be dried, placed skin-to-skin with the mother, and covered with dry linen to maintain temperature. Observation of breathing, activity, and color should be ongoing. If the answer to any of these assessment questions is “no,” the infant should receive one or more of the following 4 categories of action in sequence

Evidence suggests that hypothermia for hypoxic ischemic encephalopathy in the term neonate may decrease the risk of death or neurodevelopmental impairment. The objective of this study was to determine how hypothermia has been incorporated into practice. An anonymous survey was sent to medical directors of United States neonatal intensive care units (NICUs) in October 2005. We received completed surveys from 441 (54.5%) of 809 of NICUs. Only 6.4% of respondents used hypothermia. The most common method was total body cooling (64.3%) compared with head cooling (25%) or both (10.7%). At centers that did not offer hypothermia, 29% transferred infants to an institution that did. Centers that offered hypothermia were more likely at academic institutions (76.9%) compared with private practices (11.5%; p < 0.001). Hypothermia was more likely offered at institutions that offered extracorporeal membrane oxygenation (ECMO; 57%) than centers where ECMO was not offered (43%; p < 0.001). There has not been widespread use of hypothermia. There are a variety of protocols used. As results of further outcome studies become available, educational efforts and national practice guidelines will be essential.

Background purpose Respiratory failure in neonates with congenital diaphragmatic hernia (CDH) may in part be caused by a primary or secondary surfactant deficiency. Knowledge of the optimal approach to surfactant replacement in neonates with CDH and respiratory failure is limited. The aim of this study was to determine if surfactant replacement on extracorporeal membrane oxygenation (ECMO) results in improved outcomes in neonates ≥35 weeks' gestation with unrepaired CDH. Methods Using the CDH Study Group Registry, the authors identified 448 neonates with CDH who were ≥35 weeks' gestation, had no major anomalies, were treated with ECMO within the first 7 days of life, and underwent repair on or after ECMO therapy. Patients in 2 groups were compared: group 1 (− Surf, n=334) consisted of patients who received no surfactant and group 2 (+ Surf, n=114) consisted of patients who received at least 1 dose of surfactant while on ECMO. An analysis of all patients in both groups was performed. Additionally, subgroup analyses stratified by gestational age were performed for patients 351/7 to 366/7 weeks' gestation and for patients ≥37 weeks' gestation. Primary end-points for the study were survival and length of ECMO run. Secondary end-points were length of intubation, need for supplemental oxygen at 30 days of life, and at discharge to home. Demographic, clinical, and outcome variables were examined using Fisher's Exact tests for categorical variables and using unpaired t tests for continuous variables. Odds ratios were calculated for categorical end-point variables. Results Demographic and clinical variables were similar between groups. Analyses of aggregate data showed no significant differences between groups in length of ECMO run, survival, number of days intubated, and percent of patients requiring supplemental oxygen at 30 days or discharge. Subgroup stratification by gestational age did not show significant differences between groups in any of the outcome variables. Conclusions The data from this study suggest that surfactant replacement on ECMO for neonates with congenital diaphragmatic hernia does not provide significant benefit in the infant's clinical course with respect to survival, length of ECMO course, length of intubation, or subsequent need for supplemental oxygen.

Neu-Laxova syndrome (NLS) is a lethal, autosomal recessive multiple malformation syndrome with many features resulting from severe skin restriction and decreased fetal movement. It is characterized by ichthyosis, marked intrauterine growth restriction (IUGR), microcephaly, short neck, central nervous system (CNS) anomalies, limb deformities, hypoplastic lungs, edema, and abnormal facial features including severe proptosis with ectropion, hypertelorism, micrognathia, flattened nose, and malformed ears. We present two new patients with NLS with striking prenatal diagnostic findings and detailed post-mortem examinations and review the previously described cases in the literature. Data from these patients suggest that the NLS represents a heterogeneous phenotype. Prenatal ultrasound findings of marked ocular proptosis in a growth restricted, edematous fetus should prompt consideration of a diagnosis of the NLS.

Extracorporeal membrane oxygenation (ECMO) therapy requires that patients be anticoagulated to prevent clotting and thrombotic complications. There are several bedside whole blood microcoagulation systems available to determine activated clotting time (ACT) levels. Many ECMO centers use Hemochron (International Technidyne, Edison, NJ) products to determine ACT levels. During the study period, we used the Hemochron 400 and then changed to the Hemochron Junior Low Range. There were two specific aims of this study. First, to determine if there was a difference in ACT levels measured by these two distinct Hemochron products both marketed for the use in ECMO therapy. Second, to determine if the differing ACT levels produced by these two devices affected clinical outcomes. We compared ACT levels between two devices on 70 paired blood specimens obtained from four neonatal ECMO patients receiving heparin. A retrospective review of 77 ECMO patients was performed to analyze frequency of circuit emergencies and length of ECMO circuit life while using the two products. In lower ACT ranges, the Hemochron Jr. LR consistently yielded higher ACT values than the Hemochron 400. In higher ACT ranges, the Hemochron Jr. LR consistently yielded lower ACT values than the Hemochron 400. Without calibration, after changing devices, this discrepancy led to shorter circuit life and more circuit clotting complications. After calibration and adjustment in target ACT values, there was a trend toward longer circuit life, and there were fewer clotting complications. There is a difference in the ACT values produced by Hemochron 400 and Hemochron Jr. LR. Failure to calibrate target ACT levels after changing machines may lead to shorter circuit life and more clotting complications.

Introduction In 1999, the Accreditation Council for Graduate Medical Education identified 6 general competencies in which all residents must receive training. In the decade since these requirements went into effect, practice-based learning and improvement (PBLI) and systems-based practice (SBP) have proven to be the most challenging competencies to teach and assess. Because PBLI and SBP both are related to quality improvement (QI) principles and processes, we developed a QI-based curriculum to teach these competencies to our fellows. Methods This experiential curriculum engaged our fellows in our neonatal intensive care unit's (NICU's) structured QI process. After identifying specific patient outcomes in need of improvement, our fellows applied validated QI methods to develop evidence-based treatment protocols for our neonatal intensive care unit. Results These projects led to immediate and meaningful improvements in patient care and also afforded our fellows various means by which to demonstrate their competence in PBLI and SBP. Our use of portfolios enabled us to document our fellows' performance in these competencies quite easily and comprehensively. Discussion Given the clinical and educational structures common to most intensive care unit–based training programs, we believe that a QI-based curriculum such as ours could be adapted by others to teach and assess PBLI and SBP.

In this report, we review the case of a candidal lens abscess in a premature infant girl who was 28 weeks’ gestational age at birth. The culture obtained from the lens abscess grew Candida albicans sensitive to amphotericin B but resistant to flucytosine. This case is unique in that the infant developed a fungal lens cataract at 34 weeks’ postconceptional age during the last week of a 30-day course of amphotericin B. The embryonic hyaloid artery system, which perfuses the developing lens, regresses between 29 and 32 weeks of gestation; thus, the mechanism for an infection of the lens may be inoculation of the lens by Candida before hyaloid artery system regression, followed by developmental loss of this blood supply, which makes the lens inaccessible to antimicrobial penetration. Candidal endophthalmitis with lens abscess is an uncommon morbidity that requires prompt recognition and surgical intervention for effective management.

Objective. This study was pursued as an extension of a randomized clinical investigation of neonatal screening for cystic fibrosis (CF). The project included assessment of respiratory secretion cultures for pathogens associated with CF. The objective was to determine whether patients diagnosed through neonatal screening and treated in early infancy were more likely to become colonized with Pseudomonas aeruginosa compared with those identified by standard diagnostic methods. Methodology. The design involved prospective cultures of respiratory secretions obtained generally by oropharyngeal swabs at least every 6 months and more often if clinically indicated. Patients were managed with a standardized evaluation and treatment protocol at the two Wisconsin certified CF centers; however, there were community and environmental variations associated with the follow-up period as described below. Results. Overall, there were no differences in acquisition of respiratory pathogens between the screened and the control (standard diagnosis) groups. Evaluation of the data between and within the two centers, however, revealed significant differences with earlier acquisition of P aeruginosa in the center with the following distinguishing characteristics: urban location; following patients with the standard US approach in which newly diagnosed, young children were interspersed with older CF patients; and where there were more opportunities for social interactions with other CF patients. The differences were confined to the screened group followed in the urban center in which the median pseudomonas-free survival period was 52 weeks contrasted with 289 weeks in the other center. In addition, assessment of data for the entire CF populations followed at the two centers revealed that the urban center showed a significantly higher prevalence of P aeruginosa colonization in patients between the ages of 3 and 9 years. Conclusions. These results present questions and generate hypotheses on risk factors for acquisition of P aeruginosain CF and suggest that clinic exposures and/or social interactions may predispose such patients to pseudomonas infections.