Accuracy of parental perception of nighttime breathing in children with Down syndrome after adenotonsillectomy.

STUDY OBJECTIVES: Clinical practice guidelines recommend screening all children with Down syndrome for obstructive sleep apnea with polysomnography by age 4 years. Because persistent obstructive sleep apnea (obstructive apnea-hypopnea index > 1 event/h) following adenotonsillectomy (T&A) is common in children with Down syndrome, it is important to know whether clinicians can rely on parental assessment postoperatively. The primary objective is to identify accuracy of parental perception of nighttime breathing following T&A compared with preoperative assessment. METHODS: Patients with Down syndrome who underwent T&A along with polysomnography prior to and after the surgical procedure were included. Parents completed a 3-question pre- and postsurgery survey regarding nighttime symptoms. The responses were categorized into 3 groups: infrequent (< 3 nights/wk), sometimes (> 3 nights/wk but < 6 nights/wk), or frequent (≥ 6 nights/wk) on at least 1 question. The primary end point was identifying the accuracy of parental perception of nighttime breathing in children with Down syndrome following T&A. RESULTS: A total of 256 children met inclusion criteria, of which 117 (46%) were included. A total of 71 (68%) children had an obstructive apnea-hypopnea index > 5 events/h preoperatively compared with 55 (47%) postoperatively. There was no association between parents' perception of symptoms and obstructive sleep apnea categorization postoperatively (P > .05) or of parents' perception of symptoms improving and obstructive sleep apnea categorization improving postoperatively (P > .05). CONCLUSIONS: Despite previous experience, parents are unable to predict nighttime breathing patterns following a T&A. We recommend obtaining a polysomnogram rather than relying on parental assessment to determine whether a T&A has been successful. CITATION: Chabuz CA, Lackey TG, Pickett KL, Friedman NR. Accuracy of parental perception of nighttime breathing in children with Down syndrome after adenotonsillectomy. J Clin Sleep Med. 2024;20(2):237-243.

The impact of the COVID-19 pandemic on pediatric firearm injuries in Colorado.

BACKGROUND: In 2019 firearm injuries surpassed automobile-related injuries as the leading cause of pediatric death in Colorado. In the spring of 2020, the COVID-19 pandemic led to community-level social, economic, and health impacts as well as changes to injury epidemiology. Thus, we sought to determine the impact of the COVID-19 pandemic on pediatric firearm injuries in Colorado. METHODS: We conducted a retrospective review of pediatric firearm injured patients (≤ 18-years-old) evaluated at three trauma centers in Colorado from 2018-2021. Patients were stratified into two groups based on the time of their firearm injury: pre- COVID injuries and post- COVID injuries. Group differences were examined using t-tests for continuous variables and Chi Squared or Fisher's exact tests for categorical variables. RESULTS: Overall, 343 firearm injuries occurred during the study period. There was a significant increase in firearm injuries as a proportion of overall pediatric ED trauma evaluations following the onset of the COVID-19 pandemic (pre COVID: 5.18/100 trauma evaluations; post COVID: 8.61/100 trauma evaluations, p<0.0001). Assaults were the most common injury intent seen both pre and post COVID (70.3% vs. 56.7%, respectively); however, unintentional injuries increased significantly from 10.3% to 22.5% (p = 0.004) following the onset of the pandemic. Additionally, the COVID-19 pandemic was associated with a 177% increase in unintentional injuries in adolescents. CONCLUSION: Pediatric firearm injuries, particularly unintentional injuries, increased significantly in Colorado following the onset of the COVID-19 pandemic. The substantial increase in unintentional injuries among adolescents highlights the necessity of multi-disciplinary approaches to limit or regulate their access to firearms. LEVEL OF EVIDENCE: Level III. STUDY TYPE: Retrospective.

Outcomes in Pediatric Trauma Patients Who Receive Blood Transfusion.

INTRODUCTION: Increased blood volumes, due to massive transfusion (MT), are known to be associated with both infectious and noninfectious adverse outcomes. The aim of this study was to assess the association between MT and outcomes in pediatric trauma patients, and, secondarily, determine if these outcomes are differential by age once MT is reached. METHODS: Pediatric patients (ages 1-18 y old) in the ACS pediatric Trauma Quality Improvement Program (TQIP) database (2015-2018) who received blood were included. Patients were stratified by MT status, which was defined as blood product volume of 40 mL/kg within 24 h of admission (MT+) and compared to children who received blood products but did not meet the MT threshold (MT-). Defined MT + patients were matched 1:1 to MT-patients via propensity score matching of characteristics before comparisons. Adjusted logistic regression was performed on univariably significant outcomes of interest. RESULTS: There were 2318 patients in the analytic cohort. Patients who received MT had higher rates of deep venous thrombosis (DVT) (2.5% versus 1.0%, P < 0.001), acute kidney injury (AKI) (1.5% versus 0.0%, P = 0.022), CLABSI (4.0% versus 2.0% P = 0.008), and severe sepsis (2.3% versus. 1.1%, P = 0.02). On logistic regression MT was an independent risk factor for these outcomes. There was no differential effect of MT on these outcomes based on age. CONCLUSIONS: Outcomes associated with blood transfusion in pediatric trauma patients are low overall, but rates of DVT, AKI, CLABSI, and sepsis are higher in those who receive MT+ with no differences based on age.

National trends in pediatric firearm and automobile fatalities.

BACKGROUND: Successful public health policies and injury prevention efforts have reduced pediatric automobile fatalities across the United States. In 2019, firearm injuries exceeded motor vehicle crashes (MVC) as the leading cause of childhood death in Colorado. We sought to determine if similar trends exist nationally and if state gun laws impact firearm injury fatality rates. METHODS: Annual pediatric (≤19 years-old) fatality rates for firearm injuries and MVCs were obtained from the CDC WONDER database (1999-2020). State gun law scores were based on the 2014-2020 Gifford's Annual Gun Law Scorecard and strength was categorized by letter grades A-F. Poisson generalized linear mixed models were used to model fatality rates. Rates were estimated for multiple timepoints and compared between grade levels. RESULTS: In 1999, the national pediatric fatality rate for MVCs was 248% higher than firearm injuries (Incidence Rate Ratio (IRR) 95% Confidence Interval (CI): 2.25-2.73, p<0.0001). By 2020, the fatality rate for MVCs was 16% lower than that of firearm injuries (IRR 95% CI: 0.75- 0.93, p = 0.0014). For each increase in letter grade for gun law strength there was an 18% reduction in the firearm fatality rate (IRR 95%CI: 0.78-0.86, p<0.0001). States with the strongest gun laws (A) had a 55% lower firearm fatality rate compared to those with the weakest laws (F). CONCLUSION: Firearm injuries are the leading cause of death in pediatric patients across the United States. State gun law strength has a significant impact on pediatric firearm injury fatality rates. New public health policies, political action, media attention and safer guns are urgently needed to curb this national crisis. LEVEL OF EVIDENCE/STUDY TYPE: Level III, retrospective.

Residual OSA in Down syndrome: does body position matter?

STUDY OBJECTIVES: To examine children with Down syndrome with residual obstructive sleep apnea (OSA) to determine if they are more likely to have positional OSA. METHODS: A retrospective chart review of children with Down syndrome who underwent adenotonsillectomy at a single tertiary children's hospital was conducted. Children with Down syndrome who had a postoperative polysomnogram with obstructive apnea-hypopnea index (OAHI) > 1 event/h, following adenotonsillectomy with at least 60 minutes of total sleep time were included. Patients were categorized as mixed sleep (presence of ≥ 30 minutes of both nonsupine and supine sleep), nonsupine sleep, and supine sleep. Positional OSA was defined as an overall OAHI > 1 event/h and a supine OAHI to nonsupine OAHI ratio of ≥ 2. Group differences are tested via Kruskal-Wallis test for continuous variables and Fisher's exact tests for categorical. RESULTS: There were 165 children with Down syndrome who met inclusion criteria, of which 130 individuals had mixed sleep. Patients who predominately slept supine had a greater OAHI than mixed and nonsupine sleep (P = .002). Sixty (46%) of the mixed-sleep individuals had positional OSA, of which 29 (48%) had moderate/severe OSA. Sleeping off their backs converted 14 (48%) of these 29 children from moderate/severe OSA to mild OSA. CONCLUSIONS: Sleep physicians and otolaryngologists should be cognizant that the OAHI may be an underestimate if it does not include supine sleep. Positional therapy is a potential treatment option for children with residual OSA following adenotonsillectomy and warrants further investigation. CITATION: Lackey TG, Tholen K, Pickett K, Friedman N. Residual OSA in Down syndrome: does body position matter? J Clin Sleep Med. 2023;19(1):171-177.

Electrolyte derangements in critically ill children receiving balanced versus unbalanced crystalloid fluid resuscitation.

BACKGROUND: Adult studies have demonstrated potential harm from resuscitation with 0.9% sodium chloride (0.9%NaCl), resulting in increased utilization of balanced crystalloids like lactated ringers (LR). The sodium and potassium content of LR has resulted in theoretical safety concerns, although limited data exists in pediatrics. We hypothesized that use of LR for resuscitation would not be associated with increased electrolyte derangements compared to 0.9%NaCl. METHODS: A prospective, observational cohort study of critically ill children who received ≥ 20 ml/kg of fluid resuscitation and were admitted to two pediatric intensive care units from November 2017 to February 2020. Fluid groups included patients who received > 75% of fluids from 0.9%NaCl, > 75% of fluids from LR, and a mixed group. The primary outcome was incidence of electrolyte derangements (sodium, chloride, potassium) and acidosis. RESULTS: Among 559 patients, 297 (53%) received predominantly 0.9%NaCl, 74 (13%) received predominantly LR, and 188 (34%) received a mixture. Extreme hyperkalemia (potassium ≥ 6 mmol/L) was more common in 0.9%NaCl group (5.8%) compared to LR group (0%), p 0.05. Extreme acidosis (pH > 7.1) was more common in 0.9%NaCl group (11%) compared to LR group (1.6%), p 0.016. CONCLUSIONS: LR is associated with fewer electrolyte derangements compared to 0.9%NaCl. Prospective interventional trials are needed to validate these findings.

Social media communities for patients and families affected by congenital pediatric surgical conditions.

BACKGROUND: Social media has become a means to allow individuals affected by rare diseases to connect with others. This study aimed to characterize the use of three popular social media platforms by individuals affected by common pediatric surgery index conditions. METHODS: A systematic search of Instagram, Facebook, and Twitter was performed using standardized terms for the following pediatric surgical conditions: anorectal malformation, biliary atresia, bronchopulmonary sequestration, congenital diaphragmatic hernia (CDH), congenital pulmonary airway malformation, duodenal atresia, esophageal atresia/tracheoesophageal fistula (EA/TEF), gastroschisis, Hirschsprung disease (HD), and omphalocele. Accounts active within the last year were analyzed and assessed. RESULTS: A total of 666 accounts were identified. Instagram was the most common platform, but accounts dedicated to support and story sharing were most common on Facebook. Biliary atresia and CDH had the largest communities identified. Support groups were most common among those dedicated to EA/TEF (43.3%, p < 0.001). Most accounts were created by parents, but accounts dedicated to HD had the greatest proportion founded by patients (22.4%, p = 0.04). CONCLUSIONS: Social media use is common among patients and families affected by congenital surgical conditions. Certain diseases have larger communities and support networks. Knowledge about their existence may allow surgeons to direct patients towards supportive communities and resources. LEVEL OF EVIDENCE: IV.

Thrombelastography and transfusion patterns in severely injured pediatric trauma patients with blunt solid organ injuries.

BACKGROUND: Thrombelastography (TEG) has emerged as a useful tool to diagnose coagulopathy and guide blood product usage during trauma resuscitations. This study sought to evaluate the correlation between TEG-directed blood product administration in severely injured pediatric trauma patients with blunt solid organ injuries (BSOIs). METHODS: Patients (≤18 years) with severe BSOIs who presented as highest-level trauma activations at two pediatric trauma centers were included. Thrombelastography results were evaluated to determine indications for blood product administration and rates of TEG-directed resuscitation. Tetrachoric correlations and regression modeling were used to correlate TEG-directed resuscitation with clinical outcomes. RESULTS: Of 64 patients who met the inclusion criteria, 32.8% (21) had elevated R times and 23.4% (15) had shortened α angles. Maximum amplitude was shortened in 29.7% (19), and percent clot lysis 30 minutes after maximum amplitude that is >3% was seen in 17.0% (9). Thrombelastography-directed resuscitation of fresh frozen plasma was followed 54.7% of the time compared with 67.2% and 81.2% for platelets and cryoprecipitate, respectively. Thrombelastography-directed resuscitation with platelets (odds ratio, 0.56; 95% confidence interval, 0.33-0.93; p = 0.03) and/or cryoprecipitate (odds ratio, 0.09; 95% confidence interval, 0.01-0.42, p = 0.003) were associated with decreased hospital length of stay and mortality, respectively. CONCLUSION: Severely injured pediatric trauma patients with BSOIs were often coagulopathic upon presentation to the emergency department. Thrombelastography-directed resuscitation with platelets and/or cryoprecipitate was followed for the majority of patients and was associated with improved outcomes. LEVEL OF EVIDENCE: Therapeutic/Care Management, level III.

Tonsillectomy Outcomes for Children With Severe Obesity.

OBJECTIVES/HYPOTHESIS: To determine the success of an adenotonsillectomy (T&A) in treating children with severe obesity utilizing a more accurate obesity scale. STUDY DESIGN: Retrospective cohort. METHODS: A retrospective cohort of children with obesity between 5 and 10 years of age who underwent a T&A at Children's Hospital of Colorado (CHCO) was used. This study also utilized publicly available data from the Childhood Adenotonsillectomy Trial (CHAT) study. The cohort was divided into three obesity classes using age- and sex-specific body mass index (BMI) expressed as a percentage of the 95th percentile (%BMIp95) and compared for operative success differences. RESULTS: There were 132 patients included in our primary analysis, with obesity distribution as follows: Class 1 to 53 patients (40%), Class 2 to 45 patients (34%), and Class 3 to 34 patients (26%). Overall, 52 patients (35.9%) experienced a cure (obstructive apnea/hypopnea index [OAHI] <1), with 27 (52%) patients in Class 1 obesity, 18 (35%) in Class 2, and 7 (13%) in Class 3. Class 3 had a significantly lower obstructive sleep apnea cure rate compared with Class 1 patients (P = .013), but after adjusting for covariates, this difference was no longer present (P > .05). There was no significant difference in the preoperative to postoperative percent change in mean oxygen saturation (P = .82 CHCO, P = .43 CHAT), oxygen nadir (P = .20 CHCO, P = .49 CHAT), or OAHI (P = .12 CHCO, P = .26 CHAT) between the obesity classes. CONCLUSION: After adjusting for covariates, children with Class 3 obesity are as likely to be cured with a T&A as those with Class 1 obesity. A T&A should be considered a first line treatment for all children with obesity. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:461-469, 2022.

Massive transfusion in pediatric trauma-does more blood predict mortality?

BACKGROUND: Treatment of severe hemorrhage focuses on the control of bleeding and intravascular volume expansion through massive transfusion (MT). This study aimed to determine if transfusion volumes in pediatric trauma patients who receive MT is associated with increased risk of death, and to establish if there is a threshold above which further resuscitation with blood products is futile. METHODS: Pediatric patients (2-18 years old) in the 2014-2017 Trauma Quality Improvement Program (TQIP) database with complete age and blood transfusion data who met the MT definition of 40 mL/kg/24 h were included in analysis. Data elements were cleaned to eliminate discrepancies in reporting blood volumes and erroneous values were excluded. Early mortality was defined as death within 24 h. Late mortality was defined as death more than 24 h after hospital admission. Area under the curve (AUC) was calculated from receiver operating characteristic (ROC) curve analyses to determine upper volume thresholds to predict early versus late mortality. RESULTS: There were 633 patients who met the MT definition of 40 mL/kg/24 h. The overall mortality rate was 21.6%. Volume of blood had poor predicting early and late mortality with an AUC of 0.50 [95% CI (0.42, 0.59)] and 0.50 [95% CI (0.43,0.57)], respectively. Regardless of mechanism, no transfusion volume was associated with a predictably high rate of mortality. CONCLUSIONS: There is no upper transfusion volume threshold to predict mortality in pediatric trauma patients who are massively transfused, regardless of mechanism. Severely injured children can tolerate massive amounts of blood products and still survive. LEVEL OF EVIDENCE: III.

Does the mechanism matter? Comparing thrombelastography between blunt and penetrating pediatric trauma patients.

BACKGROUND/PURPOSE: The utility of thrombelastography (TEG) in pediatric trauma remains unknown, and differences in coagulopathy between blunt and penetrating mechanisms are not established. We aimed to compare TEG patterns in pediatric trauma patients with blunt solid organ injuries (BSOI) and penetrating injuries to determine the role of mechanism in coagulopathy. METHODS: Highest-level pediatric trauma activations with BSOI or penetrating injuries and admission TEG at two pediatric trauma centers were included. TEG abnormalities were defined by each institution's normative values and compared separately by injury mechanism and evidence of shock (elevated SIPA) using Kruskal-Wallis or Fisher's exact tests. RESULTS: Of 118 patients included, 64 had BSOI and 54 had penetrating injuries. There were no significant differences in TEG abnormalities between the BSOI and penetrating injury groups. Patients with shock were more likely to have decreased alpha-angles (30.9% vs. 8.0%, p = 0.01) and decreased maximum amplitude (MA) (44.1% vs. 8.0%, p < 0.001) compared to those without shock, regardless of mechanism of injury. CONCLUSIONS: TEG abnormalities were not significantly different between the BSOI and penetrating groups, but there were significant differences in alpha-angle and MA in those with shock, independent of mechanism. Hemodynamic status, rather than mechanism of injury, may be more predictive of coagulopathy in pediatric trauma patients. LEVEL OF EVIDENCE/STUDY TYPE: Level III, retrospective.

To give or not to give? Blood for pediatric trauma patients prior to pediatric trauma center arrival.

PURPOSE: This study evaluates the indications, safety and clinical outcomes associated with the administration of blood products prior to arrival at a pediatric trauma center (prePTC). METHODS: Children (≤ 18 years) who were highest level activations at an ACS level 1 pediatric trauma center (PTC) from 2009-2019 were divided into groups:(1) patients with transport times < 4 h who received blood prePTC(preBlood) versus (2) age matched controls with transport times < 4 h who only received crystalloid prePTC (preCrystalloid). RESULTS: Of 1269 trauma activations, 38 met preBlood and 38 met preCrystalloid inclusion criteria. A similar volume of prePTC crystalloid infusion was observed between cohorts (p = 0.311). PreBlood patients evidenced greater hemodynamic instability as demonstrated by higher prePTC pediatric age-adjusted shock index (SIPA) scores. PreBlood patients showed improvement in lactate (p = 0.038) and hemoglobin (p = 0.041) levels upon PTC arrival. PreBlood patients received less crystalloid within 12 h of PTC admission (p = 0.017). No significant differences were found in blood transfusion volumes within six (p = 0.293) and twenty-four (p = 0.575) hours of admission, nor in mortality between cohorts (p = 0.091). CONCLUSIONS: The administration of blood to pediatric trauma patients prior to arrival at a PTC is safe, transiently improves markers of shock, and was not associated with worse outcomes.

The use of social media among the pediatric colorectal community.

PURPOSE: Pediatric colorectal conditions require complex medical care and can require lifelong support. Caregivers often seek medical information on the internet. The aim of this study was to characterize the use of three social media platforms for information sharing about pediatric colorectal conditions. METHODS: A systematic study of Instagram, Facebook, and Twitter was performed using standardized search terms. Accounts with activity within the last year were included. Quantitative data were collected. Accounts were qualitatively assessed and assigned a functional category. Group differences were tested via Kruskal-Wallis test and Fisher's exact tests for continuous and categorical variables, respectively. RESULTS: A total of 96 Instagram accounts, 57 Twitter accounts, 49 Facebook pages, and 45 Facebook groups were identified. Accounts originated from 24 countries and the greatest number of accounts was created in 2013. The most common source of information on Instagram was from personal accounts (74.0%), on Facebook was from support groups (45.7%), and on Twitter was from health care providers (35.1%), (p < 0.001). The most common functional categories on Instagram were personal story (69.8%), on Twitter were scientific information/medical research (57.9%), and on Facebook were supportive/story sharing (47.8%), (p < 0.001). CONCLUSIONS: Social media serves as a source for medical information and allows for supportive communities for pediatric colorectal patients and their families to exist.

Child physical abuse and COVID-19: Trends from nine pediatric trauma centers.

BACKGROUND: Economic, social, and psychologic stressors are associated with an increased risk for abusive injuries in children. Prolonged physical proximity between adults and children under conditions of severe external stress, such as witnessed during the COVID-19 pandemic with "shelter-in-place orders", may be associated with additional increased risk for child physical abuse. We hypothesized that child physical abuse rates and associated severity of injury would increase during the early months of the pandemic as compared to the prior benchmark period. METHODS: We conducted a nine-center retrospective review of suspected child physical abuse admissions across the Western Pediatric Surgery Research Consortium. Cases were identified for the period of April 1-June 30, 2020 (COVID-19) and compared to the identical period in 2019. We collected patient demographics, injury characteristics, and outcome data. RESULTS: There were no significant differences in child physical abuse cases between the time periods in the consortium as a whole or at individual hospitals. There were no differences between the study periods with regard to patient characteristics, injury types or severity, resource utilization, disposition, or mortality. CONCLUSIONS: Apparent rates of new injuries related to child physical abuse did not increase early in the COVID-19 pandemic. While this may suggest that pediatric physical abuse was not impacted by pandemic restrictions and stresses, it is possible that under-reporting, under-detection, or delays in presentation of abusive injuries increased during the pandemic. Long-term follow-up of subsequent rates and severity of child abuse is needed to assess for unrecognized injuries that may have occurred.

Pediatric cardiac and great vessel injuries: Recent experience at two pediatric trauma centers.

BACKGROUND: Cardiac injuries are rare in pediatric trauma patients and data regarding this type of injury is limited. There is even less data on traumatic great vessel injuries. This study sought to examine and summarize our recent experience at two pediatric trauma centers, which serve a major metropolitan area and large geographic region. METHODS: This is a retrospective review of pediatric (<18 years) patients who sustained cardiac or great vessel injuries and were managed at a Level 1 or Level 2 pediatric trauma center between January 1, 2010 and June 30, 2020. Demographic and clinical characteristics were compared using two-sample t-tests, Wilcoxon Rank-Sum tests, Fisher's exact tests and chi-squared tests for continuous, non-normally distributed continuous, and categorical variables, respectively. RESULTS: A total of 53 patients sustained cardiac and/or great vessel injuries. Of these, 37 (70%) sustained cardiac, 9 (17%) sustained great vessel, and 7 (13%) sustained both types of injuries. The median age was 14.9 years and 74% (n = 39) were male. The median injury severity score (ISS) was 36.0 and the injury mechanism was blunt in 31 (58%) patients. The most common cardiac and great vessel injury locations were left ventricle (n = 9) and thoracic aorta (n = 11), respectively. The overall mortality rate was 53% (n = 28). Mortality was highest among those who sustained great vessel injuries (89%). CONCLUSIONS: There is substantial heterogeneity in cardiac and great vessel injuries. Regardless, they are highly morbid and lethal, despite aggressive surgical and catheter-based interventions.

Lung inflammation and simulated airway resistance in infants with cystic fibrosis.

Cystic fibrosis (CF) is characterized by small airway disease; but central airways may also be affected. We hypothesized that airway resistance estimated from computational fluid dynamic (CFD) methodology in infants with CF was higher than controls and that early airway inflammation in infants with CF is associated with airway resistance. Central airway models with a median of 51 bronchial outlets per model (interquartile range 46,56) were created from chest computed tomography scans of 18 infants with CF and 7 controls. Steady state airflow into the trachea was simulated to estimate central airway resistance in each model. Airway resistance was increased in the full airway models of infants with CF versus controls and in models trimmed to 33 bronchi. Airway resistance was associated with markers of inflammation in bronchoalveolar lavage fluid obtained approximately 8 months earlier but not with markers obtained at the same time. In conclusion, airway resistance estimated by CFD modeling is increased in infants with CF compared to controls and may be related to early airway inflammation.

Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.

Rationale: Neuroendocrine cell hyperplasia of infancy (NEHI) is an important form of children's interstitial and diffuse lung disease for which the diagnostic strategy has evolved. The prevalence of comorbidities in NEHI that may influence treatment has not been previously assessed.Objectives: To evaluate a previously unpublished NEHI clinical score for assistance in diagnosis of NEHI and to assess comorbidities in NEHI.Methods: We performed a retrospective chart review of 199 deidentified patients with NEHI from 11 centers. Data were collected in a centralized Research Electronic Data Capture registry and we performed descriptive statistics.Results: The majority of patients with NEHI were male (66%). The sensitivity of the NEHI Clinical Score was 87% (95% confidence interval [CI], 0.82-0.91) for all patients from included centers and 93% (95% CI, 0.86-0.97) for those with complete scores (e.g., no missing data). Findings were similar when we limited the population to the 75 patients diagnosed by lung biopsy (87%; 95% CI, 0.77-0.93). Of those patients evaluated for comorbidities, 51% had gastroesophageal reflux, 35% had aspiration or were at risk for aspiration, and 17% had evidence of immune system abnormalities.Conclusions: The NEHI Clinical Score is a sensitive tool for clinically evaluating NEHI; however, its specificity has not yet been addressed. Clinicians should consider evaluating patients with NEHI for comorbidities, including gastroesophageal reflux, aspiration, and immune system abnormalities, because these can contribute to the child's clinical picture and may influence clinical course and treatment.