Biomarkers Associated With Future Severe Liver Disease in Children With Alpha-1-Antitrypsin Deficiency.

Background and Aims: Children with alpha-1-antitrypsin deficiency (AATD) exhibit a wide range of liver disease outcomes from portal hypertension and transplant to asymptomatic without fibrosis. Individual outcomes cannot be predicted. Liver injury in AATD is caused by the accumulation in hepatocytes of the mutant Z alpha-1-antitrypsin (AAT) protein, especially the toxic, intracellular polymerized conformation. AATD patients have trace Z polymer detectable in serum with unknown significance. Methods: The Childhood Liver Disease Research Network is an NIH consortium for the study of pediatric liver diseases, including AATD. We obtained data and samples with the aim of identifying biomarkers predictive of severe AATD liver disease. Results: We analyzed prospective AATD Childhood Liver Disease Research Network data and serum samples in 251 subjects from 2007 to 2015 for outcomes and Z polymer levels. Fifty-eight of 251 had clinically evident portal hypertension (CEPH) at enrollment, and 10 developed CEPH during follow-up. Higher Z AAT polymer levels were associated with existing CEPH (P = .01). In infants without CEPH, higher polymer levels were associated with future CEPH later in childhood, but total AAT was not predictive. Higher gamma-glutamyl transferase (GGT) in the first few months of life was also significantly associated with future CEPH, and risk-threshold GGT levels can be identified. A model was constructed to identify subjects at high risk of future CEPH by combining clinical GGT and polymer levels (area under the curve of 0.83; 95% confidence interval: 0.656-1.00, P = .019). Conclusion: High circulating Z polymer levels and high GGT early in life are associated with future CEPH in AATD, and the use of predictive cutoffs may assist in future clinical trial design.

Impact of Pharmacist Intervention on Inappropriate Continuations of Antipsychotics upon ICU Discharge.

Background: Transitions of care (TOC) are important to best practices as they are at times prone to medication errors. The intensive care unit (ICU) is an essential location needing effective TOC due to many reasons, but an important one being that certain medications are only indicated there. One example is antipsychotics used for agitation, delirium, and sedation. Objective: To design, implement, and analyze the benefit of a pharmacist intervention on inappropriate antipsychotic continuation from the ICU to another point in care at a small community hospital. Secondary outcomes include patients discharged from the hospital on antipsychotics inappropriately and accepted pharmacist interventions. Methods: This standard of care, prospective with historical control study included adult patients who were ordered a formulary antipsychotic for delirium, agitation, or sedation during their ICU-level of care admission at SSM Health: St. Clare Hospital- Fenton. Results: There were 33 patients in the historical period and 24 in the intervention period. Those in the intervention period were less likely to have a continuation of antipsychotics beyond 72 hours compared to patients in the historical period (16.7% vs 57.6%, P = 0.002). In addition, patients in the intervention period were less likely to have continuation of antipsychotics when discharged to home (12.5% vs 36.4%, P = 0.04). Conclusions: A pharmacist-driven intervention led to a significant decrease in patients continuing antipsychotics upon ICU discharge. This decrease was seen at both 72 hours from patients leaving the ICU and at hospital discharge.

Oral sodium supplementation on growth and hypertension in preterm infants: an observational cohort study.

OBJECTIVE: To evaluate the association between enteral sodium supplementation on growth and hypertension (HTN) in preterm infants. STUDY DESIGN: A retrospective cohort study of infants born between 22-32 weeks and weighing 450-1500 grams (N = 821). Enteral sodium supplementation amounts, systolic blood pressures (SBP), weight gain, and other infant and maternal risk factors for HTN were electronically extracted. RESULTS: Infants receiving sodium supplementation were smaller and less mature. Sodium supplementation improved serum sodium levels, weight gain, and head circumference growth without causing hypernatremia. There was no correlation between urine and serum sodium or urine sodium and weight gain. Although infants receiving sodium had higher average SBP and rates of HTN, analysis demonstrated sodium supplementation did not increase odds of hypertension (ORADJ 1.02;0.64-1.64). Postnatal steroids were associated with HTN. CONCLUSIONS: In preterm infants with poor weight gain, enteral sodium supplementation improved growth without increasing hypertension or hypernatremia.

Incidence and survival characteristics of pediatric ganglioglioma from 2004 to 2018, with focus on infratentorial sites.

Background: Ganglioglioma (GG) is a slow-growing glioneuronal neoplasm, most frequently seen in the supratentorial location in older children and associated with epilepsy syndromes. GG is rare in the infratentorial location, hence we embarked upon analyzing the National Cancer Institute's (NCI) Survival, Epidemiology, and End Results (SEER) database to better evaluate GG outcomes by location in comparison to the broader pediatric low-grade glioma (pLGG) population. Methods: Pediatric patients diagnosed with GG and pLGG from 2004 to 2018 were included in the study. Their demographic, clinical, and survival characteristics were analyzed using SEER*Stat. Results: This study describes the largest cohort of pediatric GG, including 852 cases from year 2004 to 2018, with focus on infratentorial sites. Patients with brainstem GG or those with subtotally resected disease were identified as having higher risk of death. Conclusions: Our analysis highlights brainstem GG as a high-risk, poor-prognostic subgroup and elaborates on the incidence and survival characteristic of this lesser-known subgroup.

Crossbow Injuries: Predictors of Mortality.

INTRODUCTION: Crossbow injuries are rare but carry significant morbidity and mortality, and there is limited evidence in the medical literature to guide care. This paper reviews the case reports and case series of crossbow injuries and looks for trends regarding morbidity and mortality based on the type of arrow, anatomic location of injury, and intent of injury. METHODS: Multiple databases were searched for cases of crossbow injuries and data were abstracted into a spreadsheet. Statistics were done in SPSS. RESULTS: 358 manuscripts were returned in the search. After deduplication and removal of nonclinical articles, 101 manuscripts remained. Seventy-one articles describing 90 incidents met the inclusion criteria. The mean age was 36.5 years. There were 10 female and 79 male victims. Fatality was 36% for injuries by field tip arrows and 71% for broadhead arrows, p = .024. Assaults were fatal in 84% of cases, suicides in 29%, and accidental injuries in 17%, p < .001. Mortality was similar for wounds to the head and neck (41%), chest (42%), abdomen (33%), extremities (50%), and multiple regions, p = .618. CONCLUSIONS: Crossbows are potentially lethal weapons sold with fewer restrictions than firearms. Injuries caused by broadhead arrows are more likely to be fatal than injuries from field tip arrows. The anatomic location of injury does not correlate with fatality. More than half of crossbow injuries are due to attempted suicide, with a high case-fatality rate.

The Effect of a Merit Point Incentive System on the Willingness to Donate Organs.

PURPOSE: Although over 90% of the population of the United States supports organ donation, only 60% of the population is registered as donors. Currently, there is a need for a nonmonetary incentive that will improve willingness to donate. We assessed the young adult population's perspective on their willingness to donate organs when merit points are granted to their family members to prioritize their potential transplant if needed. METHODS: We administered a Qualtrics survey from March 2022 to September 2022 to the undergraduate students volunteering to participate at Saint Louis University, which comprised 10 questions that addressed the attitudes of participants regarding the effects of various factors, including the type of donation and the presence of merit points (vouchers granted to self or a family member to facilitate a potential transplant if needed), on participant's willingness to donate an organ while alive or after death. The responses were analyzed by using SAS software (SAS Institute). RESULTS: A total of 572 participants completed the survey. Overall, only 6.5% of surveyed students were unwilling to donate after death. The willingness to donate while alive to a family member was significantly higher than donating to a stranger (95.8% vs 71.2%, P < .0001). When merit points were added, the unwillingness to donate significantly decreased from 6.5% to 3.8%. However, this change was observed only when the merit points were given to a family member and not to self. When merit points were granted, unwillingness to provide a living donation to a stranger decreased from 28.8% to 16.4% (P < .0001). CONCLUSIONS: Merit points to first-degree family members improve students' expressed willingness to donate organs after death; however, self-merit points did not decrease the rate of "unwillingness to donate after death." When living donation is assessed, offering merit points appears to decrease the "unwillingness to donate to strangers." The adoption of a merit point system in the United States may increase the rates of organ donation.

Plant identification applications do not reliably identify toxic and edible plants in the American Midwest.

INTRODUCTION: Exposure to potentially toxic plants is a global problem, resulting in thousands of calls to poison centers and emergency department visits annually and occasional deaths. Persons with limited botanical knowledge may be tempted to rely on smartphone applications to determine if plants are safe to forage. This study evaluated the reliability of several popular smartphone applications to identify foraged foods and distinguish them from potentially toxic plants in the Midwestern United States. METHODS: Sixteen plant species were selected based on local availability, attractiveness as foraged food, and potential for misidentification. Of the 16 species, five are edible, three are potentially toxic if improperly harvested or prepared, and eight are considered to be toxic. Plant specimens were identified by graduate-level botanists and photographed during multiple stages of their growth cycles. LeafSnap, PictureThis, Pl@ntNet and PlantSnap were used to identify the plants. RESULTS: Overall accuracy of the applications in identifying plant genus was 76% (95% confidence interval: 73-79, range 96% for PictureThis to 53% for PlantSnap). Accuracy for identification of plant species was 58% (95% confidence interval 55-62%, range 94% for PictureThis to 34% for PlantSnap). Five of eleven potentially toxic species were identified as an edible species by at least one application. CONCLUSION: Accuracy of the smartphone applications varies, with PictureThis outperforming other apps. At this time, apps cannot be used to safely identify edible plants. Foragers must have adequate botanical knowledge to ensure safe harvesting of wild plants.

Arrhythmia in chronic hemodialysis as a function of predialysis electrolytes and interdialytic interval.

INTRODUCTION: People with end-stage renal disease on hemodialysis are at increased risk for death due to arrhythmia associated with the prolonged interdialytic interval that typically spans the weekend, with bradycardia being the arrhythmia most closely associated with sudden death. In this prospective observational study we assessed whether predialysis fluid and electrolytes values including hyperkalemia are risk factors for the arrhythmias associated with the prolonged interdialytic interval. METHODS: Sixty patients on hemodialysis with a history of hyperkalemia underwent cardiac monitoring for 1 week. Arrhythmia frequency, average QTc interval, and average root mean square of successive differences (rMSSD) per 4-h period were reported. Predialysis electrolytes and electrocardiograms were collected prior to pre- and post-weekend dialysis sessions. Clinical variables were assessed for correlation with arrhythmias. FINDINGS: Predialysis hyperkalemia occurred in 29 subjects and was more common at the post-weekend dialysis session. Bradycardia occurred in 11 subjects and increased before and during the post-weekend dialysis session, but was not correlated with any electrolyte or clinical parameter. Ventricular ectopy occurred in 50 subjects with diurnal variation unrelated to dialysis. Pre-dialysis prolonged QTc was common and not affected by interdialytic interval. Average QTc increased and rMSSD decreased during dialysis sessions and were not correlated with clinical parameters. DISCUSSION: The results confirm that arrhythmias are prevalent in dialysis subjects with bradycardia particularly associated with the longer interdialytic interval; EKG markers of arrhythmia risk are increased during dialysis independent of interdialytic interval. Larger sample size and/or longer recording may be necessary to identify the clinical parameters responsible.

Absence of Injury Is Not Absence of Pain: Prevalence of Preseason Musculoskeletal Pain and Associated Factors in Collegiate Soccer and Basketball Student Athletes.

Unlike musculoskeletal (MSK) injuries, MSK pain is rarely studied in athletes. In this study, we examined the prevalence of preseason MSK pain in apparently healthy collegiate soccer and basketball players and its relationship with previous injuries (1-year history), among other factors. Ninety-seven eligible student athletes (mean age: 20.1 (SD: 1.6) years; 43% male; 53% soccer players) completed a baseline questionnaire comprising questions related to demographics, medical and 1-year injury history and any current MSK pain and the corresponding body location. The overall prevalence of preseason MSK pain was 26% (95% CI: 17-36%) and it did not differ by sex or sport. The back (6.2%) and knee (5.2%) regions were reported to be the most frequently affected body parts for preseason MSK pain. Athletes with a previous injury and with perception of incomplete healing had 3.5-fold higher odds (OR: 3.50; 95% CI: 1.28-9.36) of baseline MSK pain compared with those without a previous injury. One in four collegiate soccer and basketball players had preseason MSK pain. Collegiate sports medicine professionals should consider conducting routine preseason evaluations of MSK pain in their athletes and initiate appropriate interventions for the prevention of MSK pain and its potential consequences among athletes.

An electronic health record alert increases human immunodeficiency virus screening and case identification in a high-risk emergency department population.

Increased screening for HIV is required to reduce mortality and transmission. Patients with risk factors for HIV may lack access to routine care and emergency departments are an important site for screening and linkage to care. We implemented an electronic health record algorithm to identify patients meeting criteria for HIV screening. Compared to unstructured clinical judgement, the EHR alert increased the number of patients screened and case identification.

Impact of Early Fortification in Very Low Birth Weight Infants on the Incidence of Malnutrition During a Trophamine Shortage.

OBJECTIVE: The devastation of pharmaceutical production facilities from Hurricane Maria caused a national shortage of parenteral amino acids in October 2017. Our institution decreased trophamine in very low birth weight (VLBW) infants and initiated human milk fortification at a lower feeding volume to increase enteral protein intake more quickly. The objective of this study was to assess how protein management during the shortage period affected the incidence of malnutrition. METHODS: This was a retrospective cohort study of infants admitted to 2 neonatal intensive care units from June 1, 2017 to May 31, 2018. Infants between 23 and 32 weeks' gestation were included in this study. The primary outcome was the incidence of malnutrition at 14 days, defined as a z score decline of ≥0.8 SDs, in the pre-shortage period compared with the shortage period. Clinical data regarding adverse effects associated with early fortification and pharmacy costs were recorded. RESULTS: There were 68 infants prior to and 65 during the shortage who met inclusion criteria. There was no difference in malnutrition between the pre-shortage and shortage groups; however, a significant increase in malnutrition was observed in infants who did not receive early fortification during the shortage. No difference in time to full enteral feeds or necrotizing enterocolitis was observed with early fortification. CONCLUSIONS: Early fortification in VLBW infants receiving less trophamine during the shortage was not associated with an increase in malnutrition. Restricting trophamine in neonates during the shortage allowed for distribution to other critically ill patients.

Presumed adrenal insufficiency in neonates treated with corticosteroids for the prevention of bronchopulmonary dysplasia.

OBJECTIVE: To determine if extremely preterm (EPT) neonates receiving dexamethasone for the prevention of BPD have a higher incidence of presumed adrenal insufficiency (PAI). STUDY DESIGN: Retrospective cohort study of neonates <28 weeks gestation examining PAI after dexamethasone use and PAI after intratracheal budesonide with surfactant administration. RESULT: Of 332 neonates, 38% received dexamethasone. The incidence of PAI was higher in neonates who had received dexamethasone (20.8% vs 2.9%, p < 0.001). However, for intubated babies receiving surfactant, dexamethasone was not independently associated with increased PAI after adjusting for gestational age, birthweight, and race (aOR 2.92, 95% CI: 0.79-10.85). Dexamethasone was independently associated with increased PAI in infants previously receiving budesonide/surfactant treatment (aOR 5.38, 95% CI: 1.38-20.90). CONCLUSION: The use of dexamethasone alone was not associated with increased PAI, when adjusted for prematurity-related factors. The combination of budesonide with dexamethasone was significantly associated with increased PAI.

Factors affecting STEMI performance in six hospitals within one healthcare system.

BACKGROUND: How quickly percutaneous coronary intervention is performed in patients with ST-elevation myocardial infarction (STEMI) is a quality measure, reported as door-to-balloon (D2B) time. OBJECTIVES: To explore factors affecting STEMI performance in six hospitals in one healthcare system. METHODS: This was a retrospective chart review of clinical features and D2B times. Predictors for D2B times were identified using multivariate linear regression. RESULTS: The median D2B time for all six hospitals was 63 minutes and all hospitals surpassed the minimal recommended percentage of patients achieving D2B time ≤90 minutes (87.8%vs75%,p<0.001). Patient confounders adversely affect D2B times (+21.5 minutes, p<0.001). Field ECG/activation with emergency department (ED) transport (-22.0 minutes) or direct cardiac catheterization laboratory (CCL) transport (-27.3 minutes) was superior to ED ECG/activation (p<0.001). CONCLUSION: Field ECG/STEMI activation significantly shortened D2B time. To improve D2B time, hospital and Emergency Medical Service collaboration should be advocated to increase field activation and direct patient transportation to CCL.

Dysbiosis and Intestinal Barrier Dysfunction in Pediatric Congenital Heart Disease Is Exacerbated Following Cardiopulmonary Bypass.

There are no data evaluating the microbiome in congenital heart disease following cardiopulmonary bypass. The authors evaluated patients with congenital heart disease undergoing cardiopulmonary bypass and noncardiac patients undergoing surgery without bypass. Patients with congenital heart disease had differences in baseline microbiome compared with control subjects, and this was exacerbated following surgery with bypass. Markers of barrier dysfunction were similar for both groups at baseline, and surgery with bypass induced significant intestinal barrier dysfunction compared with control subjects. This study offers novel evidence of alterations of the microbiome in congenital heart disease and exacerbation along with intestinal barrier dysfunction following cardiopulmonary bypass.

Analysis of outcomes of single-unit cord blood transplantation with umbilical cord blood units processed with two different red blood cell sedimentation reagents.

BACKGROUND: Various processing methodologies are routinely used to reduce volume and red blood cell content of umbilical cord blood (UCB) units collected for hematopoietic stem cell transplantation. There is limited information regarding effects of UCB processing techniques on clinical outcomes. STUDY DESIGN AND METHODS: Retrospective data analysis compared laboratory and clinical outcomes following single-unit UCB transplantation performed between 1999 and 2015. All UCB units were from St. Louis Cord Blood Bank and all were manually processed with either Hetastarch processed cord blood units (HCB) (n = 661) or PrepaCyte processed cord blood units (PCB) (n = 84). Additional sensitivity analysis focused on units transplanted from 2010 to 2015 and included 105 HCB and 84 PCB. RESULTS: There were no significant differences in patient characteristics between the two groups. Pre-freeze total nucleated and CD34+ cell counts, cell doses/kg of recipient weight, and total colony-forming units (CFUs) were higher in PCB compared with HCB. Post-thaw, the PCB group had a significantly better total nucleated cell recovery, while there were no significant differences in cell viability, CFU recovery, or CD34+ cell recovery. Primary analysis demonstrated faster neutrophil and platelet engraftment for PCB but no differences in overall survival (OS), whereas sensitivity analysis found no effect of processing method on engraftment, but better OS in the HCB group compared with PCB group. CONCLUSION: The UCB processing method had no significant impact on engraftment. However, we cannot completely exclude the effect of processing method on OS. Additional studies may be warranted to investigate the potential impact of the PCB processing method on clinical outcomes.

Elevated neutrophil-lymphocyte ratios in extremely preterm neonates with histologic chorioamnionitis.

OBJECTIVE: Histologic chorioamnionitis (HCA) is a placental inflammation linked to preterm birth and adverse neonatal outcome. The neutrophil-lymphocyte ratio (NLR) can identify various inflammatory disorders, however its utility in HCA is not clear. Our goal was to examine NLR values and HCA diagnoses in at-risk pregnancies and neonates. STUDY DESIGN: We retrospectively analyzed the EHR of mothers and preterm (<33 wk GA) neonates with or without HCA (identified by placental histology). The NLR was calculated from complete blood counts in laboring women and in their neonates (0-24 h of life). RESULT: In 712 consecutive gestations, 50.8% had HCA (26.5% fetal HCA). The neonatal NLR (0-12 h, 13-24 h) predicted fetal HCA better than chance alone (p = 0.01 and 0.002, respectively). CONCLUSION: Early NLR elevation in preterm neonates is consistent with a diagnosis of fetal HCA. The NLR may identify preterm neonates at risk for HCA-related complications.

Multilevel Associations Between Patient- and Hospital-Level Factors and In-Hospital Mortality Among Hospitalized Patients With Head and Neck Cancer.

Importance: Risk factors for in-hospital mortality of patients with head and neck cancer (HNC) are multilevel. Studies have examined the effect of patient-level characteristics on in-hospital mortality; however, there is a paucity of data on multilevel correlates of in-hospital mortality. Objective: To examine the multilevel associations of patient- and hospital-level factors with in-hospital mortality and develop a nomogram to predict the risk of in-hospital mortality among patients diagnosed with HNC. Design, Setting, and Participants: This cross-sectional study used the 2008-2013 National Inpatient Sample database. Hospitalized patients 18 years and older diagnosed (both primary and secondary diagnosis) as having HNC using the International Classification of Diseases, Ninth Revision, Clinical Modification codes were included. Analysis began December 2018. Main Outcomes and Measures: The primary outcome of interest was in-hospital mortality. A weighted multivariable hierarchical logistic regression model estimated patient- and hospital-level factors associated with in-hospital mortality. Moreover, a multivariable logistic regression analysis was used to build an in-hospital mortality prediction model, presented as a nomogram. Results: A total of 85 440 patients (mean [SD] age, 62.2 [13.5] years; 61 281 men [71.1%]) were identified, and 4.2% (n = 3610) died in the hospital. Patient-level risk factors associated with higher odds of in-hospital mortality included age (adjusted odds ratio [aOR], 1.03 per 1-year increase; 95% CI, 1.02-1.03), male sex (aOR, 1.23; 95% CI, 1.12-1.35), higher number of comorbidities (aOR, 1.14; 95% CI, 1.11-1.17), having a metastatic cancer (aOR, 1.49; 95% CI, 1.36- 1.64), having a nonelective admission (aOR, 3.26; 95% CI, 2.83-3.75), and being admitted to the hospital on a weekend (aOR, 1.30; 95% CI, 1.16-1.45). Of the hospital-level factors, admission to a nonteaching hospital (aOR, 1.48; 95% CI, 1.24-1.77) was associated with higher odds of in-hospital mortality. The nomogram showed fair in-hospital mortality discrimination (area under the curve of 72%). Conclusions and Relevance: This cross-sectional study found that both patient- and hospital-level factors were associated with in-hospital mortality, and the nomogram estimated with fair accuracy the probability of in-hospital death among patients with HNC. These multilevel factors are critical indicators of survivorship and should thus be considered when planning programs or interventions aimed to improve survival among this unique population.

Avoiding NICU Transfers for Newborns With Neonatal Opioid Withdrawal Syndrome (NOWS): A Quality Improvement Initiative to Manage NOWS on the Mother-baby Unit.

OBJECTIVES: To reduce transfers to the neonatal intensive care unit (NICU) for neonates with opioid withdrawal while also reducing length of stay and pharmacologic intervention, and maintaining standards of safety. PATIENTS AND METHODS: This was a single-center quality-improvement (QI) initiative in a free-standing maternity hospital comparing outcomes for neonatal opioid withdrawal syndrome (NOWS) before and after a series of QI bundles in infants >36 weeks' gestation age (GA). We compared outcomes to our preintervention period (January, 2013 to December, 2013; n = 42) with outcomes postintervention cycle 1 (October, 2016 to September, 2017; n = 126), and postintervention cycle 2 (November, 2017 to October, 2018; n = 160). Cycle 1 included organizing a multidisciplinary task force who focused on emphasis on nonpharmacologic and dyad-centered care, and also standardized pharmacologic management. Cycle 2 reflects the transition to a functional assessment tool and as-needed morphine administration on the postpartum floor. RESULTS: Transfer to the NICU for management of NOWS dropped from 71.4% before the quality improvement project down to 5.6% (P < 0.001), with the remainder managed on the mother-baby unit. Length of stay decreased from 17.8 days to 7.2 days, and opioid replacement dropped from 60% down to 16% (P < 0.001 for both). There were no adverse events from morphine administration for any of the infants in this series. CONCLUSIONS: Our study demonstrates how care can be safely provided to most infants with neonatal opioid withdrawal on a postpartum unit without needing transfer to another unit or a higher level of care facility.