ABSTRACT
This perspective work by academic neonatal providers is written specifically for the audience of newborn care providers and neonatologists involved in neonatal hypoglycemia screening. Herein, we propose adding a screen for congenital hyperinsulinism (CHI) by measuring glucose and ketone (i.e., ß-hydroxybutyrate (BOHB)) concentrations just prior to newborn hospital discharge and as close to 48 h after birth as possible, at the same time that the mandated state Newborn Dried Blood Spot Screen is obtained. In the proposed protocol, we do not recommend specific metabolite cutoffs, as our primary objective is to simply highlight the concept of screening for CHI in newborns to newborn caregivers. The premise for our proposed screen is based on the known effect of hyperinsulinism in suppressing ketogenesis, thereby limiting ketone production. We will briefly discuss genetic CHI, other forms of neonatal hypoglycemia, and their shared mechanisms; the mechanism of insulin regulation by functional pancreatic islet cell membrane KATP channels; adverse neurodevelopmental sequelae and brain injury due to missing or delaying the CHI diagnosis; the principles of a good screening test; how current neonatal hypoglycemia screening programs do not fulfill the criteria for being effective screening tests; and our proposed algorithm for screening for CHI in newborns.
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BACKGROUND AND OBJECTIVES: Neonatal endotracheal tube (ETT) size recommendations are based on limited evidence. We sought to determine data-driven weight-based ETT sizes for infants undergoing tracheal intubation and to compare these with Neonatal Resuscitation Program (NRP) recommendations. METHODS: Retrospective multicenter cohort study from an international airway registry. We evaluated ETT size changes (downsizing to a smaller ETT during the procedure or upsizing to a larger ETT within 7 days) and risk of procedural adverse outcomes associated with first-attempt ETT size selection when stratifying the cohort into 200 g subgroups. RESULTS: Of 7293 intubations assessed, the initial ETT was downsized in 5.0% of encounters and upsized within 7 days in 1.5%. ETT downsizing was most common when NRP-recommended sizes were attempted in the following weight subgroups: 1000 to 1199 g with a 3.0 mm (12.6%) and 2000 to 2199 g with a 3.5 mm (17.1%). For infants in these 2 weight subgroups, selection of ETTs 0.5 mm smaller than NRP recommendations was independently associated with lower odds of adverse outcomes compared with NRP-recommended sizes. Among infants weighing 1000 to 1199 g: any tracheal intubation associated event, 20.8% with 2.5 mm versus 21.9% with 3.0 mm (adjusted OR [aOR] 0.62, 95% confidence interval [CI] 0.41-0.94); severe oxygen desaturation, 35.2% with 2.5 mm vs 52.9% with 3.0 mm (aOR 0.53, 95% CI 0.38-0.75). Among infants weighing 2000 to 2199 g: severe oxygen desaturation, 41% with 3.0 mm versus 56% with 3.5mm (aOR 0.55, 95% CI 0.34-0.89). CONCLUSIONS: For infants weighing 1000 to 1199 g and 2000 to 2199 g, the recommended ETT size was frequently downsized during the procedure, whereas 0.5 mm smaller ETT sizes were associated with fewer adverse events and were rarely upsized.
Subject(s)
Intubation, Intratracheal , Resuscitation , Humans , Infant, Newborn , Cohort Studies , Intubation, Intratracheal/methods , OxygenABSTRACT
OBJECTIVE: To determine the factors associated with second attempt success and the risk of adverse events following a failed first attempt at neonatal tracheal intubation. DESIGN: Retrospective analysis of prospectively collected data on intubations performed in the neonatal intensive care unit (NICU) and delivery room from the National Emergency Airway Registry for Neonates (NEAR4NEOS). SETTING: Eighteen academic NICUs in NEAR4NEOS. PATIENTS: Neonates requiring two or more attempts at intubation between October 2014 and December 2021. MAIN OUTCOME MEASURES: The primary outcome was successful intubation on the second attempt, with severe tracheal intubation-associated events (TIAEs) or severe desaturation (≥20% decline in oxygen saturation) being secondary outcomes. Multivariate regression examined the associations between these outcomes and patient characteristics and changes in intubation practice. RESULTS: 5805 of 13 126 (44%) encounters required two or more intubation attempts, with 3156 (54%) successful on the second attempt. Second attempt success was more likely with changes in any of the following: intubator (OR 1.80, 95% CI 1.56 to 2.07), stylet use (OR 1.65, 95% CI 1.36 to 2.01) or endotracheal tube (ETT) size (OR 2.11, 95% CI 1.74 to 2.56). Changes in stylet use were associated with a reduced chance of severe desaturation (OR 0.74, 95% CI 0.61 to 0.90), but changes in intubator, laryngoscope type or ETT size were not; no changes in intubator or equipment were associated with severe TIAEs. CONCLUSIONS: Successful neonatal intubation on a second attempt was more likely with a change in intubator, stylet use or ETT size.
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OBJECTIVE: To determine the relationship between number of attempts and adverse events during neonatal intubation. STUDY DESIGN: A retrospective study of prospectively collected data of intubations in the delivery room and NICU from the National Emergency Airway Registry for Neonates (NEAR4NEOS) in 17 academic centers from 1/2016 to 12/2019. We examined the association between tracheal intubation attempts [1, 2, and ≥3 (multiple attempts)] and clinical adverse outcomes (any tracheal intubation associated events (TIAE), severe TIAE, and severe oxygen desaturation). RESULTS: Of 7708 intubations, 1474 (22%) required ≥3 attempts. Patient, provider, and practice factors were associated with higher TI attempts. Increasing intubation attempts was independently associated with a higher risk for TIAE. The adjusted odds ratio for TIAE and severe oxygen desaturation were significantly higher in TIs with 2 and ≥3 attempts than with one attempt. CONCLUSION: The risk of adverse safety events during intubation increases with the number of intubation attempts.
Subject(s)
Intubation, Intratracheal , Oxygen , Humans , Infant, Newborn , Intubation, Intratracheal/adverse effects , Registries , Retrospective StudiesABSTRACT
BACKGROUND: To compare validity evidence for dichotomous and trichotomous versions of a neonatal intubation (NI) procedural skills checklist. METHODS: NI skills checklists were developed utilizing an existing framework. Experts were trained on scoring using dichotomous and trichotomous checklists, and rated recordings of 23 providers performing simulated NI. Videolaryngoscope recordings of glottic exposure were evaluated using Cormack-Lehane (CL) and Percent of Glottic Opening scales. Internal consistency and reliability of both checklists were analyzed, and correlations between checklist scores, airway visualization, entrustable professional activities (EPA), and global skills assessment (GSA) were calculated. RESULTS: During rater training, raters gave significantly higher scores on better provider performance in standardized videos (both p < 0.001). When utilized to evaluate study participants' simulated NI attempts, both dichotomous and trichotomous checklist scores demonstrated very good internal consistency (Cronbach's alpha 0.868 and 0.840, respectively). Inter-rater reliability was higher for dichotomous than trichotomous checklists [Fleiss kappa of 0.642 and 0.576, respectively (p < 0.001)]. Sum checklist scores were significantly different among providers in different disciplines (p < 0.001, dichotomous and trichotomous). Sum dichotomous checklist scores correlated more strongly than trichotomous scores with GSA and CL grades. Sum dichotomous and trichotomous checklist scores correlated similarly well with EPA. CONCLUSIONS: Neither dichotomous or trichotomous checklist was superior in discriminating provider NI skill when compared to GSA, EPA, or airway visualization assessment. Sum scores from dichotomous checklists may provide sufficient information to assess procedural competence, but trichotomous checklists may permit more granular feedback to learners and educators. The checklist selected may vary with assessment needs.
Subject(s)
Checklist , Clinical Competence , Feedback , Humans , Infant, Newborn , Intubation, Intratracheal , Reproducibility of ResultsABSTRACT
OBJECTIVE: We hypothesized that videolaryngoscope use for tracheal intubations would differ across NICUs, be associated with higher first attempt success and lower adverse events. STUDY DESIGN: Data from the National Emergency Airway Registry for Neonates (01/2015 to 12/2017) included intubation with direct laryngoscope or videolaryngoscope. Primary outcome was first attempt success. Secondary outcomes were adverse tracheal intubation associated events and severe desaturation. RESULTS: Of 2730 encounters (13 NICUs), 626 (23%) utilized a videolaryngoscope (3% to 64% per site). Videolaryngoscope use was associated with higher first attempt success (p < 0.001), lower adverse tracheal intubation associated events (p < 0.001), but no difference in severe desaturation. After adjustment, videolaryngoscope use was not associated with higher first attempt success (OR:1.18, p = 0.136), but was associated with lower tracheal intubation associated events (OR:0.45, p < 0.001). CONCLUSION: Videolaryngoscope use is variable, not independently associated with higher first attempt success but associated with fewer tracheal intubation associated events.
Subject(s)
Laryngoscopes , Humans , Infant, Newborn , Intensive Care Units, Neonatal , Intubation, Intratracheal/adverse effects , Laryngoscopy , RegistriesABSTRACT
Polymerization of deoxygenated hemoglobin S underlies the pathophysiology of sickle cell disease (SCD). In activating red blood cell pyruvate kinase and glycolysis, mitapivat (AG-348) increases adenosine triphosphate (ATP) levels and decreases the 2,3-diphosphoglycerate (2,3-DPG) concentration, an upstream precursor in glycolysis. Both changes have therapeutic potential for patients with SCD. Here, we evaluated the safety and tolerability of multiple ascending doses of mitapivat in adults with SCD with no recent blood transfusions or changes in hydroxyurea or l-glutamine therapy. Seventeen subjects were enrolled; 1 subject was withdrawn shortly after starting the study. Sixteen subjects completed 3 ascending dose levels of mitapivat (5, 20, and 50 mg, twice daily [BID]) for 2 weeks each; following a protocol amendment, the dose was escalated to 100 mg BID in 9 subjects. Mitapivat was well tolerated at all dose levels, with the most common treatment-emergent adverse events (AEs) being insomnia, headache, and hypertension. Six serious AEs (SAEs) included 4 vaso-occlusive crises (VOCs), non-VOC-related shoulder pain, and a preexisting pulmonary embolism. Two VOCs occurred during drug taper and were possibly drug related; no other SAEs were drug related. Mean hemoglobin increase at the 50 mg BID dose level was 1.2 g/dL, with 9 of 16 (56.3%) patients achieving a hemoglobin response of a ≥1 g/dL increase compared with baseline. Mean reductions in hemolytic markers and dose-dependent decreases in 2,3-DPG and increases in ATP were also observed. This study provides proof of concept that mitapivat has disease-modifying potential in patients with SCD. This trial was registered at www.clinicaltrials.gov as #NCT04000165.
Subject(s)
Anemia, Sickle Cell , Pyruvate Kinase , Adult , Humans , Pyruvic Acid , 2,3-Diphosphoglycerate , Anemia, Sickle Cell/drug therapy , Hemoglobins , Adenosine TriphosphateABSTRACT
OBJECTIVE: To develop a comprehensive competency assessment tool for pediatric bag-mask ventilation (pBMV) and demonstrate multidimensional validity evidence for this tool. STUDY DESIGN: A novel pBMV assessment tool was developed consisting of 3 components: a 22-item-based checklist (trichotomized response), global rating scale (GRS, 5-point), and entrustment assessment (4-point). Participants' performance in a realistic simulation scenario was video-recorded and assessed by blinded raters. Multidimensional validity evidence for procedural assessment, including evidence for content, response-process, internal structure, and relation to other variables, was assessed. The scores of each scale were compared with training level. Item-based checklist scores also were correlated with GRS and entrustment scores. RESULTS: Fifty-eight participants (9 medical students, 10 pediatric residents, 18 critical care/neonatology fellows, 21 critical care/neonatology attendings) were evaluated. The pBMV tool was supported by high internal consistency (Cronbach α = 0.867). Inter-rater reliability for the item-based checklist component was acceptable (r = 0.65, P < .0001). The item-based checklist scores differentiated between medical students and other providers (P < .0001), but not by other trainee level. GRS and entrustment scores significantly differentiated between training levels (P < .001). Correlation between skill item-based checklist and GRS was r = 0.489 (P = .0001) and between item-based checklist and entrustment score was r = 0.52 (P < .001). This moderate correlation suggested each component measures pBMV skills differently. The GRS and entrustment scores demonstrated moderate inter-rater reliability (0.42 and 0.46). CONCLUSIONS: We established evidence of multidimensional validity for a novel entrustment-based pBMV competence assessment tool, incorporating global and entrustment-based assessments. This comprehensive tool can provide learner feedback and aid in entrustment decisions as learners progress through training.
Subject(s)
Internship and Residency , Students, Medical , Checklist/methods , Child , Clinical Competence , Critical Care , Educational Measurement , Humans , Reproducibility of ResultsABSTRACT
The issue of treating sickle cell disease with drugs that increase hemoglobin oxygen affinity has come to the fore with the US Food and Drug Administration approval in 2019 of voxelotor, the only antisickling drug approved since hydroxyurea in 1998. Voxelotor reduces sickling by increasing the concentration of the nonpolymerizing, high oxygen affinity R (oxy) conformation of hemoglobin S (HbS). Treatment of sickle cell patients with voxelotor increases Hb levels and decreases indicators of hemolysis, but with no indication as yet that it reduces the frequency of pain episodes. In this study, we used the allosteric model of Monod, Wyman, and Changeux to simulate whole-blood oxygen dissociation curves and red cell sickling in the absence and presence of voxelotor under the in vivo conditions of rapid oxygen pressure decreases. Our modeling agrees with results of experiments using a new robust assay, which shows the large, expected decrease in sickling from the drug. The modeling indicates, however, that the increase in oxygen delivery from reduced sickling is largely offset by the increase in oxygen affinity. The net result is that the drug increases overall oxygen delivery only at the very lowest oxygen pressures. However, reduction of sickling mitigates red cell damage and explains the observed decrease in hemolysis. More importantly, our modeling of in vivo oxygen dissociation, sickling, and oxygen delivery suggests that drugs that increase fetal Hb or decrease mean corpuscular hemoglobin concentration (MCHC) should be more therapeutically effective than drugs that increase oxygen affinity.
Subject(s)
Anemia, Sickle Cell/drug therapy , Antisickling Agents/therapeutic use , Benzaldehydes/therapeutic use , Hemoglobin, Sickle/metabolism , Oxygen/metabolism , Pyrazines/therapeutic use , Pyrazoles/therapeutic use , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/metabolism , Antisickling Agents/pharmacology , Benzaldehydes/pharmacology , Erythrocytes/drug effects , Erythrocytes/metabolism , Hemoglobin, Sickle/chemistry , Humans , Models, Molecular , Oxygen/blood , Pyrazines/pharmacology , Pyrazoles/pharmacologyABSTRACT
OBJECTIVES: To characterize neonatal-perinatal medicine fellows' progression toward neonatal intubation procedural competence during fellowship training. METHODS: Multi-center cohort study of neonatal intubation encounters performed by neonatal-perinatal medicine fellows between 2014 through 2018 at North American academic centers in the National Emergency Airway Registry for Neonates. Cumulative sum analysis was used to characterize progression of individual fellows' intubation competence, defined by an 80% overall success rate within 2 intubation attempts. We employed multivariable analysis to assess the independent impact of advancing quarter of fellowship training on intubation success. RESULTS: There were 2297 intubation encounters performed by 92 fellows in 8 hospitals. Of these, 1766 (77%) were successful within 2 attempts. Of the 40 fellows assessed from the start of training, 18 (45%) achieved procedural competence, and 12 (30%) exceeded the deficiency threshold. Among fellows who achieved competence, the number of intubations to meet this threshold was variable, with an absolute range of 8 to 46 procedures. After adjusting for patient and practice characteristics, advancing quarter of training was independently associated with an increased odds of successful intubation (adjusted odds ratio: 1.10; 95% confidence interval 1.07-1.14). CONCLUSIONS: The number of neonatal intubations required to achieve procedural competence is variable, and overall intubation competence rates are modest. Although repetition leads to skill acquisition for many trainees, some learners may require adjunctive educational strategies. An individualized approach to assess trainees' progression toward intubation competence is warranted.
Subject(s)
Clinical Competence , Fellowships and Scholarships , Intubation, Intratracheal , Canada , Humans , Infant, Newborn , Multivariate Analysis , Registries , Retrospective Studies , United StatesABSTRACT
INTRODUCTION: Neonatal tracheal intubation (TI) outcomes have been assessed by role, but training level may impact TI success and safety. Effect of physician training level (PTL) on the first-attempt success, adverse TI-associated events (TIAEs), and oxygen desaturation was assessed. METHODS: Prospective cohort study in 11 international NEAR4NEOS sites between October 2014 and December 2017. Primary TIs performed by pediatric/neonatal physicians were included. Univariable analysis evaluated association between PTL, patient/practice characteristics, and outcomes. Multivariable analysis with generalized estimating equation assessed for independent association between PTL and outcomes (first-attempt success, TIAEs, and oxygen desaturation ≥20%; attending as reference). RESULTS: Of 2,608 primary TIs, 1,298 were first attempted by pediatric/neonatal physicians. PTL was associated with patient age, weight, comorbidities, TI indication, difficult airway history, premedication, and device. First-attempt success rate differed across PTL (resident 23%, fellow 53%, and attending 60%; p < 0.001). There was no statistically significant difference in TIAEs (resident 22%, fellow 20%, and attending 25%; p = 0.34). Desaturation occurred more frequently with residents (60%), compared to fellows and attendings (46 and 53%; p < 0.001). In multivariable analysis, adjusted odds ratio of the first-attempt success was 0.18 (95% CI: 0.11-0.30) for residents and 0.80 (95% CI: 0.51-1.24) for fellows. PTL was not independently associated with adjusted odds of TIAEs or severe oxygen desaturation. CONCLUSION: Higher PTL was associated with increased first-attempt success but not TIAE/oxygen desaturation. Identifying strategies to decrease adverse events during neonatal TI remains critical.
Subject(s)
Intubation, Intratracheal , Physicians , Child , Educational Status , Humans , Infant, Newborn , Intubation, Intratracheal/adverse effects , Prospective Studies , RegistriesABSTRACT
INTRODUCTION: Intubations are frequently performed procedures in neonatal intensive care units (NICU) and delivery rooms (DR). Unsuccessful first attempts are common as are tracheal intubation-associated events (TIAEs) and severe desaturations. Stylets are often used during intubation, but their association with intubation outcomes is unclear. OBJECTIVE: To compare intubation success, rate of relevant TIAEs, and severe desaturations in neonates intubated with and without stylets. METHODS: Tracheal intubations of neonates in the NICU or DR from 16 centers between October 2014 and December 2018, performed by neonatology or pediatric providers, were collected from the NEAR4NEOs international registry. Primary oral intubations with a laryngoscope were included in the analysis. First-attempt success, the occurrence of relevant TIAEs, and severe oxygen desaturation (≥20% saturation drop from baseline) were compared between intubations performed with versus without a stylet. Logistic regression with generalized estimate equations was used to control for covariates and clustering by sites. RESULTS: Out of 5,292 primary oral intubations, 3,877 (73%) utilized stylets. Stylet use varied considerably across the centers with a range between 0.5 and 100%. Stylet use was not associated with first-attempt intubation success, esophageal intubation, mainstem intubation, or severe desaturations after controlling for confounders. Patient size was associated with these outcomes and much more predictive of success. CONCLUSIONS: Stylet use during neonatal intubation was not associated with higher first-attempt intubation success, fewer relevant TIAEs, or less severe desaturations. These data suggest that stylets can be used based on individual preference, but stylet use may not be associated with better intubation outcomes.
Subject(s)
Intensive Care Units, Neonatal , Intubation, Intratracheal , Child , Humans , Infant, Newborn , Intubation, Intratracheal/adverse effects , Logistic Models , RegistriesABSTRACT
An oxygen-affinity-modifying drug, voxelotor, has very recently been approved by the FDA for treatment of sickle cell disease. The proposed mechanism of action is by preferential binding of the drug to the R quaternary conformation, which cannot copolymerize with the T conformation to form sickle fibers. Here, we report widely different oxygen dissociation and oxygen association curves for normal blood in the presence of voxelotor and interpret the results in terms of the allosteric model of Monod, Wyman, and Changeux with the addition of drug binding. The model does remarkably well in quantitatively explaining a complex data set with just the addition of drug binding and dissociation rates for the R and T conformations. Whereas slow dissociation of the drug from R results in time-independent dissociation curves, the changing association curves result from slow dissociation of the drug from T, as well as extremely slow binding of the drug to T. By calculating true equilibrium curves from the model parameters, we show that there would be a smaller decrease in oxygen delivery from the left shift in the dissociation curve caused by drug binding if drug binding and dissociation for both R and T were rapid. Our application of the Monod, Wyman, and Changeux model demonstrates once more its enormous power in explaining many different kinds of experimental results for hemoglobin. It should also be helpful in analyzing oxygen binding and in vivo delivery in future investigations of oxygen-affinity-modifying drugs for sickle cell disease.
Subject(s)
Anemia, Sickle Cell , Pharmaceutical Preparations , Allosteric Regulation , Anemia, Sickle Cell/drug therapy , Hemoglobins/metabolism , Humans , Kinetics , Oxygen , Protein BindingABSTRACT
OBJECTIVE: Describe the current practice of family presence during neonatal tracheal intubations (TIs) across neonatal intensive care units (NICUs) and examine the association with outcomes. DESIGN: Retrospective analysis of TIs performed in NICUs participating in the National Emergency Airway Registry for Neonates (NEAR4NEOS). SETTING: Thirteen academic NICUs. PATIENTS: Infants undergoing TI between October 2014 and December 2017. MAIN OUTCOME MEASURES: Association of family presence with TI processes and outcomes including first attempt success (primary outcome), success within two attempts, adverse TI-associated events (TIAEs) and severe oxygen desaturation ≥20% from baseline. RESULTS: Of the 2570 TIs, 242 (9.4%) had family presence, which varied by site (median 3.6%, range 0%-33%; p<0.01). Family member was more often present for older infants and those with chronic respiratory failure. Fewer TIs were performed by residents when family was present (FP 10% vs no FP 18%, p=0.041). Among TIs with family presence versus without family presence, the first attempt success rate was 55% vs 49% (p=0.062), success within two attempts was 74% vs 66% (p=0.014), adverse TIAEs were 18% vs 20% (p=0.62) and severe oxygen desaturation was 49% vs 52%, (p=0.40). In multivariate analyses, there was no independent association between family presence and intubation success, adverse TIAEs or severe oxygen desaturation. CONCLUSION: Family are present in less than 10% of TIs, with variation across NICUs. Even after controlling for important patient, provider and site factors, there were no significant associations between family presence and intubation success, adverse TIAEs or severe oxygen desaturation.
Subject(s)
Family/psychology , Intensive Care Units, Neonatal/statistics & numerical data , Intubation, Intratracheal/statistics & numerical data , Academic Medical Centers , Female , Gestational Age , Humans , Infant , Infant, Newborn , Intubation, Intratracheal/adverse effects , Intubation, Intratracheal/methods , Male , Oximetry , Oxygen/blood , Prospective Studies , Registries , Retrospective StudiesABSTRACT
There is limited information about newborns with confirmed or suspected COVID-19. Particularly in the hospital after delivery, clinicians have refined practices in order to prevent secondary infection. While guidance from international associations is continuously being updated, all facets of care of neonates born to women with confirmed or suspected COVID-19 are center-specific, given local customs, building infrastructure constraints, and availability of protective equipment. Based on anecdotal reports from institutions in the epicenter of the COVID-19 pandemic close to our hospital, together with our limited experience, in anticipation of increasing numbers of exposed newborns, we have developed a triage algorithm at the Penn State Hospital at Milton S. Hershey Medical Center that may be useful for other centers anticipating a similar surge. We discuss several care practices that have changed in the COVID-19 era including the use of antenatal steroids, delayed cord clamping (DCC), mother-newborn separation, and breastfeeding. Moreover, this paper provides comprehensive guidance on the most suitable respiratory support for newborns during the COVID-19 pandemic. We also present detailed recommendations about the discharge process and beyond, including providing scales and home phototherapy to families, parental teaching via telehealth and in-person education at the doors of the hospital, and telehealth newborn follow-up.
Subject(s)
Coronavirus Infections , Infant Care/methods , Pandemics , Pneumonia, Viral , Postnatal Care/organization & administration , Pregnancy Complications, Infectious , Betacoronavirus/isolation & purification , COVID-19 , Coronavirus Infections/diagnosis , Coronavirus Infections/epidemiology , Coronavirus Infections/therapy , Evidence-Based Practice , Female , Humans , Infant Care/organization & administration , Infant, Newborn , Infectious Disease Transmission, Vertical , Pneumonia, Viral/diagnosis , Pneumonia, Viral/epidemiology , Pneumonia, Viral/therapy , Practice Guidelines as Topic , Pregnancy , Pregnancy Complications, Infectious/epidemiology , Pregnancy Complications, Infectious/virology , SARS-CoV-2 , Triage/methods , Triage/organization & administrationABSTRACT
Mucolipidosis II α/beta (MLII) is an autosomal recessive disease in which a gene mutation leads to improper targeting of lysosomal enzymes with an end result of accumulation of lysosomes in the mitochondria resulting in a dysfunctional mitochondria. 1 Leigh syndrome (LS) is a rare progressive neurodegenerative disorder associated with dysfunctional mitochondria and oxidative phosphorylation. 4 Both disease processes typically present in infancy. 3 7 Herein, we present a case of an infant diagnosed with both mucolipidosis II and Leigh syndrome. Genetic analysis in this case revealed two mutations (NDUFA12 c.178C > T p.Arg60* and GNPTAB c.732_733delAA) on the long arm of chromosome 12 as the etiology of MLII and LS in this neonate, respectively. We are unaware of any previously published cases of the presence of these two diseases occurring in the same patient. The complex clinical presentation of this case led to a delay in the diagnosis, and we believe that the clinical phenotypes of these two conditions were likely worsened. The genetic alterations presented in this case occurred as a result of mutations on chromosome 12. We suggest further investigation into the potential overlap in the pathophysiology, specifically the inheritance pattern, linkage disequilibrium, mitochondrial-lysosomal interaction, or crosstalk contributing to both diseases.
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OBJECTIVE: This study aimed to examine the association between team stress level and adverse tracheal intubation (TI)-associated events during neonatal intubations. STUDY DESIGN: TIs from 10 academic neonatal intensive care units were analyzed. Team stress level was rated immediately after TI using a 7-point Likert scale (1 = high stress). Associations among team stress, adverse TI-associated events, and TI characteristics were evaluated. RESULT: In this study, 208 of 2,009 TIs (10%) had high stress levels (score < 4). Oxygenation failure, hemodynamic instability, and family presence were associated with high stress level. Video laryngoscopy and premedication were associated with lower stress levels. High stress level TIs were associated with adverse TI-associated event rates (31 vs. 16%, p < 0.001), which remained significant after adjusting for potential confounders including patient, provider, and practice factors associated with high stress (odds ratio: 1.90, 96% confidence interval: 1.36-2.67, p < 0.001). CONCLUSION: High team stress levels during TI were more frequently reported among TIs with adverse events.
Subject(s)
Intensive Care Units, Neonatal , Intubation, Intratracheal/methods , Patient Care Team , Stress, Psychological , Clinical Competence/standards , Female , Humans , Infant, Newborn , Intubation, Intratracheal/adverse effects , Laryngoscopy , Male , Premedication , Retrospective Studies , Task Performance and Analysis , United StatesABSTRACT
BACKGROUND: Characteristics of neonatal tracheal intubations (TI) may vary between the neonatal intensive care unit (NICU) and delivery room (DR). The impact of the setting on TI outcomes is not well characterized. OBJECTIVE: The aim of this study was to define variation in neonatal TI practice between settings, and identify the association between setting and TI success and safety outcomes. DESIGN: This was a retrospective cohort study of TIs in the National Emergency Airway Registry for Neonates from October 2014 to September 2017. The setting (NICU vs. DR) was the exposure of interest. The outcomes were first attempt success, course success, success within 4 attempts, adverse TI-associated events, severe desaturation, and bradycardia. We compared TI characteristics and outcomes between settings in univariable analysis. Factors significant in univariable analysis (p < 0.1) were included in a logistic regression model, with adjustment for clustering by center, to identify the independent impact of the setting on TI outcomes. RESULTS: There were 3,145 TI encounters (2279 NICU, 866 DR) in 9 centers. Almost all baseline characteristics significantly varied between settings. First attempt success rates were 48% (NICU) and 46% (DR). In multivariable analysis, the setting was not associated with first attempt success. DR was associated with a higher adjusted OR (aOR) of success within 4 attempts (1.48, 95% CI 1.06-2.08) and a lower aOR for bradycardia (0.43, 95% CI 0.26-0.71). CONCLUSION: Significant differences in patient, provider, and practice characteristics exist between NICU and DR TIs. There is substantial room for improvement in first attempt success rates. These results suggest interventions to improve safety and success need to be targeted to the distinct setting.
Subject(s)
Delivery Rooms/statistics & numerical data , Emergency Medical Services/methods , Intensive Care Units, Neonatal/statistics & numerical data , Intubation, Intratracheal/methods , Registries , Emergency Medical Services/trends , Female , Humans , Infant, Newborn , Intubation, Intratracheal/trends , Logistic Models , Male , Philadelphia , Retrospective Studies , Treatment OutcomeABSTRACT
The objective of this study was to describe a single-center experience with neonatal and pediatric extracorporeal life support (ECLS) and compare patient-related outcomes with those of the Extracorporeal Life Support Organization (ELSO) Registry. A retrospective review of subject characteristics, outcomes, and complications of patients who received the ECLS at Penn State Health Children's Hospital (PSHCH) from 2000 to 2016 was performed. Fisher's exact test was used to compare the PSHCH outcomes and complications to the ELSO Registry report. Data from 118 patients were included. Survival to discontinuation of the ECLS was 70.3% and 65.2% to discharge/transfer. Following circuitry equipment changes, the survival to discharge/transfer improved for both neonatal (<29 days) and pediatric (29 days to <18 years) patients. The most common complications associated with ECLS were clinical seizures, intracranial hemorrhage, and culture-proven infection. ECLS for pulmonary support appeared to be associated with a higher risk of circuit thrombus and cannula problems. When compared to the ELSO Registry, low volume ECLS centers, like our institution, can have outcomes that are no different or statistically better as noted with neonatal and pediatric cardiac patients. Pediatric patients requiring pulmonary support appeared to experience more mechanical complications during ECLS suggesting the need for ongoing technological improvement.