Familial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome.

BACKGROUND: Immune thrombocytopenia (ITP) and Evans syndrome (ES) are manifestations of immune dysregulation. Genetic variants in immune-related genes have been identified in patients with ITP and especially ES. We aimed to explore familial autoimmunity in patients with ITP and ES to understand possible contributions to chronicity. PROCEDURE: We assessed family history in two ways: via patient report for ITP and ES and by population-based analysis using the Utah Population Database (UPDB) for ITP. A total of 266 patients with ITP and 21 patients with ES were identified via chart review, and 252 of the 266 patients with ITP were also identified in the UPDB. RESULTS: Chart review showed familial autoimmunity in 29/182 (15.9%) and 25/84 (29.8%) of patients with newly diagnosed+persistent (nd+p) ITP and chronic ITP (cITP), respectively, (p = .009). The UPDB analysis revealed that autoimmunity in relatives of patients with nd+pITP was higher than in relatives of controls (odds ratio [OR]: 1.69 [1.19-2.41], p = .004), but was not significantly increased in relatives of patients with cITP (OR 1.10 [0.63-1.92], p = .734). Incomplete family history in medical records likely contributed to the observed discrepancy. CONCLUSIONS: The findings suggest that familial autoimmunity may have a stronger association with the development of ITP rather than its duration. Twelve (57.1%) patients with ES reported autoimmunity in their relatives. UPDB analysis was omitted due to the small number of patients with ES. The use of population databases offers a unique opportunity to assess familial health and may provide clues about contributors to immune dysregulation features within families.

Early iron supplementation and iron sufficiency at one month of age in NICU patients at-risk for iron deficiency.

In order to reduce iron deficiency in neonates at-risk for iron deficiency, we implemented a guideline to increase the consistency of early iron supplementation in infants of diabetic mothers, small for gestational age neonates and very low birthweight premature neonates. Three years following implementation we performed a retrospective analysis in order to assess adherence to the guideline and to compare timing of early iron supplementation and reticulocyte-hemoglobin (RET-He) values at one month of life in at-risk infants. Adherence with early iron supplementation guidelines was 73.4% (399/543) with 51% (275/543) having RET-He values obtained at one month. Despite good adherence, 16% (44/275) had RET-He <25 pg (5th percentile for gestational age). No infants receiving red blood cell transfusion (0/20) had RET-He <25 pg vs. 26.1% (40/153) of those treated with darbepoetin (p < 0.001). There was no evidence of increased feeding intolerance (episodes of emesis/day) with early iron supplementation.

Influence of Geographic Access on Surgical Center Readmissions After Index Congenital Heart Surgery.

OBJECTIVE: To assess the impact of geographic access to surgical center on readmission risk and burden in children after congenital heart surgery. STUDY DESIGN: Children <6 years old at discharge after congenital heart surgery (Risk Adjustment for Congenital Heart Surgery-1 score 2-6) were identified using Pediatric Health Information System data (46 hospitals, 2004-2015). Residential distance from the surgery center, calculated using ZIP code centroids, was categorized as <15, 15-29, 30-59, 60-119, and ≥120 miles. Rurality was defined using rural-urban commuting area codes. Geographic risk factors for unplanned readmissions to the surgical center and associated burden (total hospital length of stay [LOS], costs, and complications) were analyzed using multivariable regression. RESULTS: Among 59 696 eligible children, 19 355 (32%) had ≥1 unplanned readmission. The median LOS was 9 days (IQR 22) across the entire cohort. In those readmitted, median total costs were $31 559 (IQR $90 176). Distance from the center was inversely related but rurality was positively related to readmission risk. Among those readmitted, increased distance was associated with longer LOS, more complications, and greater costs. Compared with urban patients, highly rural patients were more likely to have an unplanned readmission but had fewer average readmission days. CONCLUSIONS: Geographic measures of access differentially affect readmission to the surgery center. Increased distance from the center was associated with fewer unplanned readmissions but more complications. Among those readmitted, the most isolated patients had the greatest readmission costs. Understanding the contribution of geographic access will aid in developing strategies to improve care delivery to this population.

Neonatal Reference Intervals for the Complete Blood Count Parameters MicroR and HYPO-He: Sensitivity Beyond the Red Cell Indices for Identifying Microcytic and Hypochromic Disorders.

OBJECTIVE: To create neonatal reference intervals for the MicroR and HYPO-He complete blood count (CBC) parameters and to test whether these parameters are sensitive early markers of disease at early stages of microcytic/hypochromic disorders while the CBC indices are still normal. STUDY DESIGN: We retrospectively collected the CBC parameters MicroR and HYPO-He, along with the standard CBC parameters, from infants aged 0-90 days at Intermountain Healthcare hospitals using Sysmex hematology analyzers. We created reference intervals for these parameters by excluding values from neonates with proven microcytic disorders (ie, iron deficiency or alpha thalassemia) from the dataset. RESULT: From >11 000 CBCs analyzed, we created reference intervals for MicroR and HYPO-He in neonates aged 0-90 days. The upper intervals are considerably higher in neonates than in adults, validating increased anisocytosis and polychromasia among neonates. Overall, 52% of neonates with iron deficiency (defined by reticulocyte hemoglobin equivalent <25 pg) had a MicroR >90% upper interval (relative risk, 4.14; 95% CI, 3.80-4.53; P < .001), and 68% had an HYPO-He >90% upper interval (relative risk, 6.64; 95% CI, 6.03-7.32; P < .001). These 2 new parameters were more sensitive than the red blood cell (RBC) indices (P < .001) in identifying 24 neonates with iron deficiency at birth. CONCLUSIONS: We created neonatal reference intervals for MicroR and HYPO-He. Although Sysmex currently designates these as research use only in the US, they can be measured as part of a neonate's CBC with no additional phlebotomy volume or run time and can identify microcytic and hypochromic disorders even when the RBC indices are normal.

Changes in Provider Prescribing Behavior for Infants with Single Ventricle Physiology After Evidence-Based Publications.

BACKGROUND: The impact of published evidence on clinical practice has been understudied in pediatric cardiology. OBJECTIVE: We sought to assess changes in prescribing behavior for angiotensin-converting enzyme inhibitor (ACEI) and digoxin at discharge after initial palliation of infants with single ventricle (SV) physiology following the publication of two large studies: The Pediatric Heart Network Infant Single Ventricle (PHN-ISV) trial showing no benefit with routine ACEI use and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) analysis showing an association between digoxin and survival. METHODS: ICD-9-10 codes identified SV infants from the Pediatric Health Information System (1/2004 to 1/2018) and charge codes identified medications at discharge. Generalized estimating equations implementing segmented logistic regressions modeled medication use, before and after (with a 3-month washout period) the relevant publication (ACEI 7/1/2010; digoxin 4/1/2016). A subgroup analysis was performed for hypoplastic left heart syndrome (HLHS). RESULTS: ACEI use (37 centers, n = 4700) at discharge did not change over time during the pre-publication period. After publication of the PHN-ISV trial, ACEI use decreased (OR: 0.61, CI 0.44-0.84, p = 0.003). Digoxin use (43 centers, n = 4778) decreased by 1% monthly before publication. After the NPC-QIC publication, digoxin use increased (OR: 2.07, CI 1.05-4.08, p = 0.04) with an ongoing increase of 9% per month. Results were similar for the HLHS subgroup. CONCLUSIONS: Prescribing behavior changed congruently after the publication of evidence-based studies, with decreased ACEI use and increased digoxin use at discharge following initial palliation of SV infants. Our findings suggest scientific findings were rapidly implemented into clinical practice.

Trends in Imaging Findings, Interventions, and Outcomes Among Children With Isolated Head Trauma.

OBJECTIVE: The aim was to analyze the impact of decreased head computed tomography (CT) imaging on detection of abnormalities and outcomes for children with isolated head trauma. METHODS: The study involves a multicenter retrospective cohort of patients younger than 19 years presenting for isolated head trauma to emergency departments in the Pediatric Health Information System database from 2003 to 2015. Patients directly admitted or transferred to another facility and those with a discharge diagnosis code for child maltreatment were excluded. Outcomes were ascertained from administrative and billing data. Trends were tested using mixed effects logistic regression, accounting for clustering within hospitals and adjusted for age, sex, insurance type, race, presence of a complex chronic condition, and hospital-level case mix index. RESULTS: Between 2003 and 2015, 306,041 children presented for isolated head trauma. The proportion of children receiving head CT imaging was increasing until 2008, peaking at just under 40%, before declining to 25% by 2015. During the recent period of decreased head CT imaging, the detection of skull fractures (odds ratio [OR]/year, 0.96; 95% confidence interval [CI], 0.95-0.97) and intracranial bleeds (OR/year, 0.96; 95% CI, 0.94-0.97), hospitalization (OR/year, 0.96; 95% CI, 0.95-0.96), neurosurgery (OR/year, 0.91; 95% CI, 0.87-0.95), and revisit (OR/year, 0.98; 95% CI, 0.96-1.00) also decreased, without significant changes in mortality (OR/year, 0.93; 95% CI, 0.84-1.04) or persistent neurologic impairment (OR/year, 1.03; 95% CI, 0.92-1.15). CONCLUSIONS: The recent decline in CT scanning in children with isolated head trauma was associated with a reduction in detection of intracranial abnormalities, and a concomitant decrease in interventions, without measurable patient harm.

Geographic and Specialty Access Disparities in US Pediatric Leukodystrophy Diagnosis.

OBJECTIVE: To examine disparities in the diagnosis of leukodystrophies including geographic factors and access to specialty centers. STUDY DESIGN: Retrospective cohort study of pediatric patients admitted to Pediatric Health Information System hospitals. Patients with leukodystrophy were identified with International Classification of Diseases, Tenth Revision, Clinical Modification diagnostic codes for any of 4 leukodystrophies (X-linked adrenoleukodystrophy, Hurler disease, Krabbe disease, and metachromatic leukodystrophy). We used 3-level hierarchical generalized logistic modeling to predict diagnosis of a leukodystrophy based on distance traveled for hospital, neighborhood composition, urban/rural context, and access to specialty center. RESULTS: We identified 501 patients with leukodystrophy. Patients seen at a leukodystrophy center of excellence hospital were 1.73 times more likely to be diagnosed than patients at non-center of excellence hospitals. Patients who traveled farther were more likely to be diagnosed than those who traveled shorter. Patients living in a Health Professionals Shortage Area zip code were 0.86 times less likely to be diagnosed than those living in a non-Health Professionals Shortage Area zip code. CONCLUSIONS: Geographic factors affect the diagnosis of leukodystrophies in pediatric patients, particularly in regard to access to a center with expertise in leukodystrophies. Our findings suggest a need for improving access to pediatric specialists and possibly deploying specialists or diagnostic testing more broadly.

Current Epidemiology of Vocal Cord Dysfunction After Congenital Heart Surgery in Young Infants.

OBJECTIVES: Surgery of the aortic arch poses risk of recurrent laryngeal nerve injury due to the anatomic proximity and can manifest as vocal cord dysfunction after surgery. We assessed risk factors for vocal cord dysfunction and calculated surgical procedure associated rates in young infants after congenital heart surgery. DESIGN: Cross section analysis. SETTING: Forty-four children's hospitals reporting administrative data to Pediatric Health Information System. PARTICIPANTS: Cardiac surgical patients less than or equal to 90 days old and discharged between January 2004 and June 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Overall, 2,319 of 46,567 subjects (5%) had vocal cord dysfunction, increasing from 4% to 7% over the study period. Of those with vocal cord dysfunction, 75% had unilateral partial paralysis. Vocal cord dysfunction was significantly more common in newborn infants (74%), those with aortic arch procedures (77%) and with greater surgical complexity. Rates of vocal cord dysfunction ranged from 0.7% to 22.4% across surgical procedure groups. Vocal cord dysfunction was significantly associated with greater use of: prolonged mechanical ventilation (53% vs 40%), diaphragmatic plication (3% vs 1%), feeding tube use (32% vs 8%), surgical airways (4% vs 2%), and prolonged length of stay (44 vs 21 d). Vocal cord dysfunction testing increased significantly over the study (6-14 %), and vocal cord dysfunction diagnosis increased almost two-fold (odds ratio, 1.9; 95% CI, 1.7-2.1) comparing the last to first study quarters with the increase in vocal cord dysfunction diagnosis occurring predominately in surgeries to the aortic arch supported by cardiopulmonary bypass. However, aortic procedures without cardiopulmonary bypass and nonaortic arch procedures were common surgeries accounting for 27% and 23% of vocal cord dysfunction cases despite low overall vocal cord dysfunction rates (3.7% and 2.6%). CONCLUSIONS: Vocal cord dysfunction complicated all cardiac surgical procedures among infants including those without aortic arch involvement. Increased efforts to determine appropriate indications for prevention, screening and treatment of vocal cord dysfunction among young infants after congenital heart surgery are needed.

Mortality and Resource Use Following Cardiac Interventions in Children with Trisomy 13 and Trisomy 18 and Congenital Heart Disease.

We sought to evaluate the mortality, risk factors for mortality, and resource utilization following cardiac interventions in trisomy 13 (T13) and 18 (T18) children. All T13 and T18 children who underwent a cardiac intervention from January 1999 to March 2015 were identified from the Pediatric Health Information System database. Data collected included demographics, type of congenital heart disease (CHD), cardiac interventions, comorbidities, length of stay (LOS), hospital charges, and deaths (within 30 days). Logistic regression analysis was used to determine factors associated with mortality. There were 49 (47% females) T13 and 140 (67% females) T18 subjects. The two cohorts were similar in distribution for race, geographic region, insurance type, and median household income. The most common CHD in both groups was a shunt lesion followed by conotruncal defects. Compared to T18, the T13 cohort had higher mortality (29% vs. 12%), tracheostomies (12% vs. 4%), gastrostomies (18% vs. 6%), and overall resource use (P < 0.05 for all). White race (OR 0.23, 95% CI 0.06-0.81) in T13 and older age (in weeks) at surgery in T18 (OR 0.75, 95% CI 0.64-0.86) were associated with lower mortality. A select group of T13 and T18 CHD patients can undergo successful cardiac interventions, albeit with a higher mortality and resource use. T13 patients have higher mortality and resource use compared to T18. In T13 and T18 patients, interventions for CHD may be an acceptable and ethical option following a careful individualized selection and counseling by a team of experts.

Resource Use and Morbidities in Pediatric Cardiac Surgery Patients with Genetic Conditions.

OBJECTIVE: To evaluate and describe resource use and perioperative morbidities among those patients with genetic conditions undergoing cardiac surgery. STUDY DESIGN: Using the Pediatric Health Information System database, we identified patients ≤18 years old with cardiac surgery classified by Risk Adjustment for Congenital Heart Surgery (RACHS) during 2003-2014. A total of 95 253 patients met study criteria and included no genetic conditions (84.6%), trisomy 21 (9.9%), trisomy 13 or 18 (0.2%), 22q11 deletion (0.8%), Turner syndrome (0.4%), and "other" genetic conditions (4.2%). We compared perioperative complications and procedures in each genetic condition with patients without genetic conditions using regression analysis. RESULTS: All groups with genetic conditions, excluding trisomy 21 RACHS 3-5, experienced increased length of stay and cost among survivors. Complications varied by genetic condition, with patients with trisomy 21 having increased odds of pulmonary hypertension and nosocomial infections. Patients with 22q11 only had increased odds of infection. Patients with Turner syndrome had increased odds of acute renal failure (OR 2.35). Patients with trisomy 13 or 18 had increased odds of pulmonary hypertension (OR 3.13), acute renal failure (OR 2.93), cardiac arrest (OR 2.84), and nosocomial infections (OR 3.53), and those with "other" genetic conditions had increased odds of all complications. CONCLUSIONS: Children with congenital heart disease and genetic conditions, except trisomy 21 RACHS 3-5, had increased costs and length of stay. Perioperative morbidities were more common and differed across genetic condition subgroups. Patient-specific risk factors are important for risk stratification, benchmarking, and counseling with families.

Variation in Adolescent Idiopathic Scoliosis Surgery: Implications for Improving Healthcare Value.

OBJECTIVES: To investigate the variation in care and cost of spinal fusion for adolescent idiopathic scoliosis (AIS), and to identify opportunities for improving healthcare value. STUDY DESIGN: Retrospective cohort study from the Pediatric Health Information Systems database, including children 11-18 years of age with AIS who underwent spinal fusion surgery between 2004 and 2015. Multivariable regression was used to evaluate the relationships between hospital cost, patient outcomes, and resource use. RESULTS: There were 16 992 cases of AIS surgery identified. There was marked variation across hospitals in rates of intensive care unit admission (0.5%-99.2%), blood transfusions (0%-100%), surgical complications (1.8%-32.3%), and total hospital costs ($31 278-$90 379). Hospital cost was 32% higher at hospitals that most frequently admitted patients to the intensive care unit (P = .009), and 8% higher for each additional 25 operative cases per hospital (P = .003). Hospital duration of stay was shorter for patients admitted to hospitals with highest intensive care unit admission rates and higher surgical volumes. There was no association between cost and duration of stay, 30-day readmission, or surgical complications. The largest contribution to hospital charges was supplies (55%). Review of a single hospital's detailed cost accounting system also found supplies to be the greatest single contributor to cost, the majority of which were for spinal implants, accounting for 39% of total hospital costs. CONCLUSIONS: The greatest contribution to AIS surgery cost was supplies, the majority of which is likely attributed to spinal implant costs. Opportunities for improving healthcare value should focus on controlling costs of spinal instrumentation, and improving quality of care with standardized treatment protocols.

Development and Validation of a Score to Predict Mortality in Children Undergoing Extracorporeal Membrane Oxygenation for Respiratory Failure: Pediatric Pulmonary Rescue With Extracorporeal Membrane Oxygenation Prediction Score.

OBJECTIVE: Our objective was to develop and validate a prognostic score for predicting mortality at the time of extracorporeal membrane oxygenation initiation for children with respiratory failure. Preextracorporeal membrane oxygenation mortality prediction is important for determining center-specific risk-adjusted outcomes and counseling families. DESIGN: Multivariable logistic regression of a large international cohort of pediatric extracorporeal membrane oxygenation patients. SETTING: Multi-institutional data. PATIENTS: Prognostic score development: A total of 4,352 children more than 7 days to less than 18 years old, with an initial extracorporeal membrane oxygenation run for respiratory failure reported to the Extracorporeal Life Support Organization's data registry during 2001-2013 were used for derivation (70%) and validation (30%). Bidirectional stepwise logistic regression was used to identify factors associated with mortality. Retained variables were assigned a score based on the odds of mortality with higher scores indicating greater mortality. External validation was accomplished using 2,007 patients from the Pediatric Health Information System dataset. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The Pediatric Pulmonary Rescue with Extracorporeal Membrane Oxygenation Prediction score included mode of extracorporeal membrane oxygenation; preextracorporeal membrane oxygenation mechanical ventilation more than 14 days; preextracorporeal membrane oxygenation severity of hypoxia; primary pulmonary diagnostic categories including, asthma, aspiration, respiratory syncytial virus, sepsis-induced respiratory failure, pertussis, and "other"; and preextracorporeal membrane oxygenation comorbid conditions of cardiac arrest, cancer, renal and liver dysfunction. The area under the receiver operating characteristic curve for internal and external validation datasets were 0.69 (95% CI, 0.67-0.71) and 0.66 (95% CI, 0.63-0.69). CONCLUSIONS: Pediatric Pulmonary Rescue with Extracorporeal Membrane Oxygenation Prediction is a validated tool for predicting in-hospital mortality among children with respiratory failure receiving extracorporeal membrane oxygenation support.

Metrics to Assess Extracorporeal Membrane Oxygenation Utilization in Pediatric Cardiac Surgery Programs.

OBJECTIVES: Only a small fraction of pediatric cardiac surgical patients are supported with extracorporeal membrane oxygenation following cardiac surgery, but extracorporeal membrane oxygenation use is more common among those undergoing higher complexity surgery. We evaluated extracorporeal membrane oxygenation metrics indexed to annual cardiac surgical volume to better understand extracorporeal membrane oxygenation use among U.S. cardiac surgical programs. DESIGN: Retrospective analysis SETTING:: Forty-three U.S. Children's Hospitals in the Pediatric Health Information System that performed cardiac surgery and used extracorporeal membrane oxygenation. PATIENTS: All patients (< 19 yr) undergoing cardiac surgery during January 2003 to July 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Both extracorporeal membrane oxygenation use and surgical mortality were risk adjusted using Risk Adjustment for Congenital Heart Surgery 1. Extracorporeal membrane oxygenation metrics indexed to annual cardiac surgery cases were calculated for each hospital and the metric values divided into quintiles for comparison across hospitals. Among 131,786 cardiac surgical patients, 3,782 (2.9%) received extracorporeal membrane oxygenation. Median case mix adjusted rate of extracorporeal membrane oxygenation use was 2.8% (interquartile range, 1.6-3.4%). Median pediatric cardiac case mix adjusted surgical mortality was 3.5%. Extracorporeal membrane oxygenation-associated surgical mortality was 1.3% (interquartile range, 0.7-1.6%); without extracorporeal membrane oxygenation, median case mix adjusted surgical mortality would increase from 3.5% to 5.0%. Among patients who died, 36.7% (median) were supported with extracorporeal membrane oxygenation. The median reduction in case mix adjusted surgical mortality from extracorporeal membrane oxygenation surgical survival was 30.1%. The median extracorporeal membrane oxygenation free surgical survival was 95% (interquartile range, 94-96%). Centers with less than 150 annual surgical cases had significantly lower median extracorporeal membrane oxygenation use (0.78%) than centers with greater than 275 cases (≥ 2.8% extracorporeal membrane oxygenation use). Extracorporeal membrane oxygenation use and mortality varied within quintiles and across quintiles of center annual surgical case volume. CONCLUSIONS: Risk adjusted extracorporeal membrane oxygenation metrics indexed to annual surgical volume provide potential for benchmarking as well as a greater understanding of extracorporeal membrane oxygenation utilization, efficacy, and impact on cardiac surgery mortality.

Use of Extracorporeal Membrane Oxygenation and Mortality in Pediatric Cardiac Surgery Patients With Genetic Conditions: A Multicenter Analysis.

OBJECTIVE: Congenital heart disease is commonly a manifestation of genetic conditions. Surgery and/or extracorporeal membrane oxygenation were withheld in the past from some patients with genetic conditions. We hypothesized that surgical care of children with genetic conditions has increased over the last decade, but their cardiac extracorporeal membrane oxygenation use remains lower and mortality greater. DESIGN: Retrospective cohort study. SETTING: Patients admitted to the Pediatric Health Information System database 18 years old or younger with cardiac surgery during 2003-2014. Genetic conditions identified by International Classification of Diseases, 9th Edition codes were grouped as follows: trisomy 21, trisomy 13 or 18, 22q11 deletion, and all "other" genetic conditions and compared with patients without genetic condition. PATIENTS: A total of 95,253 patients met study criteria, no genetic conditions (85%), trisomy 21 (10%), trisomy 13 or 18 (0.2%), 22q11 deletion (1%), and others (5%). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Annual surgical cases did not vary over time. Compared to patients without genetic conditions, trisomy 21 patients, extracorporeal membrane oxygenation use was just over half (odds ratio, 0.54), but mortality with and without extracorporeal membrane oxygenation were similar. In trisomy 13 or 18 patients, extracorporeal membrane oxygenation use was similar to those without genetic condition, but all five treated with extracorporeal membrane oxygenation died. 22q11 patients compared with those without genetic condition had similar extracorporeal membrane oxygenation use, but greater odds of extracorporeal membrane oxygenation mortality (odds ratio, 3.44). Other genetic conditions had significantly greater extracorporeal membrane oxygenation use (odds ratio, 1.22), mortality with extracorporeal membrane oxygenation (odds ratio, 1.42), and even greater mortality odds without (odds ratio, 2.62). CONCLUSIONS: The proportion of children undergoing cardiac surgery who have genetic conditions did not increase during the study. Excluding trisomy 13 or 18, all groups of genetic conditions received and benefited from extracorporeal membrane oxygenation, although extracorporeal membrane oxygenation mortality was greater for those with 22q11 deletion and other genetic conditions.

A multi-institutional study of factors affecting resource utilisation following the Fontan operation.

The few studies evaluating data on resource utilisation following the Fontan operation specifically are outdated. We sought to evaluate resource utilisation and factors associated with increased resource use after the Fontan operation in a contemporary, large, multi-institutional cohort. This retrospective cohort study of children who had the Fontan between January, 2004 and June, 2013 used the Pediatric Health Information Systems Database. Generalised linear regression analyses evaluated factors associated with resource use. Of 2187 Fontan patients included in the study, 62% were males. The median age at Fontan was 3.2 years (inter-quartile range (IQR): 2.6-3.8). The median length of stay following the Fontan was 9 days (IQR: 7-14). The median costs and charges in 2012 dollars for the Fontan operation were $93,900 (IQR: $67,800-$136,100) and $156,000 (IQR: $112,080-$225,607), respectively. Postoperative Fontan mortality (30 days) was 1% (n=21). Factors associated with increased resource utilisation included baseline and demographic factors such as region, race, and renal anomaly, factors at the bidirectional Glenn such as seizures, valvuloplasty, and surgical volume, number of admissions between the bidirectional Glenn and the Fontan, and factors at the Fontan such as surgical volume and age at Fontan. The most strongly associated factors for both increased Fontan length of stay and increased Fontan charges were number of bidirectional Glenn to Fontan admissions (p<0.001) and Fontan surgical volume per year (p<0.001). As patient characteristics and healthcare-related delivery variables accounted for most of the factors predicting increased resource utilisation, changes should target healthcare delivery factors to reduce costs in this resource-intensive population.

Extracorporeal Membrane Oxygenation for Pediatric Respiratory Failure: Risk Factors Associated With Center Volume and Mortality.

OBJECTIVES: Recent analyses show higher mortality at low-volume centers providing extracorporeal membrane oxygenation. We sought to identify factors associated with center volume and mortality to explain survival differences and identify areas for improvement. DESIGN: Retrospective cohort study. SETTING: Patients admitted to children's hospitals in the Pediatric Health Information System database and supported with extracorporeal membrane oxygenation for respiratory failure from 2003 to 2014. PATIENTS: A total of 5,303 patients aged 0-18 years old met inclusion criteria: 3,349 neonates and 1,954 children. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Low center volume was defined as less than 20, medium 20-49, and large greater than or equal to 50 cases per year. Center volume was also assessed as a continuous integer. Among neonates, clinical factors including intraventricular hemorrhage (relative risk, 1.4; 95% CI, 1.24-1.56) and acute renal failure (relative risk, 1.38; 95% CI, 1.20-1.60) were more common at low-volume compared to larger centers and were associated with in-hospital death. After adjustment for differences in demographic factors and primary pulmonary conditions, mild prematurity, acute renal failure, intraventricular hemorrhage, and receipt of dialysis remained independently associated with mortality, as did center volume measured as a continuous number. Among children, the risk of acute renal failure was almost 20% greater (relative risk, 1.18; 95% CI, 1.02-1.38) in small compared to large centers, but dialysis and bronchoscopy were used significantly less but were associated with mortality. After adjustment for differences in demographic factors and primary pulmonary conditions, acute renal failure, acute liver necrosis, acute pancreatitis, and receipt of bronchoscopy remained independently associated with mortality. Center volume measurement was not associated with mortality given these factors. CONCLUSIONS: Among neonates, investigation for intraventricular hemorrhage prior to extracorporeal membrane oxygenation and preservation of renal function are important factors for improvement. Earlier initiation of extracorporeal membrane oxygenation and careful attention to preservation of organ function are important to improve survival for children.

Cervical spine imaging in hospitalized children with traumatic brain injury.

OBJECTIVES: The purposes of this study, in children with traumatic brain injury (TBI), to describe cervical spine imaging practice, to assess for recent changes in imaging practice, and to determine whether cervical spine computed tomography (CT) is being used in children at low risk for cervical spine injury. METHODS: The setting was children's hospitals participating in the Pediatric Health Information System database, from January 2001 to June 2011. Participants were children (younger than 18 y) with TBI who were evaluated in the emergency department, admitted to the hospital, and received a head CT scan on the day of admission. The primary outcome measures were cervical spine imaging studies. This study was exempted from institutional review board assessment. RESULTS: A total of 30,112 children met study criteria. Overall, 52% (15,687/30,112) received cervical spine imaging. The use of cervical spine radiographs alone decreased between 2001 (47%) and 2011 (23%), with an annual decrease of 2.2% (95% confidence interval [CI], 1.1%-3.3%), and was largely replaced by an increased use of CT, with or without radiographs (8.6% in 2001 and 19.5% in 2011, with an annual increase of 0.9%; 95% CI, 0.1%-1.8%). A total of 2545 children received cervical spine CT despite being discharged alive from the hospital in less than 72 hours, and 1655 of those had a low-risk mechanism of injury. CONCLUSIONS: The adoption of CT clearance of the cervical spine in adults seems to have influenced the care of children with TBI, despite concerns about radiation exposure.

Pediatric and neonatal extracorporeal membrane oxygenation: does center volume impact mortality?*.

OBJECTIVE: Extracorporeal membrane oxygenation, an accepted rescue therapy for refractory cardiopulmonary failure, requires a complex multidisciplinary approach and advanced technology. Little is known about the relationship between a center's case volume and patient mortality. The purpose of this study was to analyze the relationship between hospital extracorporeal membrane oxygenation annual volume and in-hospital mortality and assess if a minimum hospital volume could be recommended. DESIGN: Retrospective cohort study. SETTING: A retrospective cohort admitted to children's hospitals in the Pediatric Health Information System database from 2004 to 2011 supported with extracorporeal membrane oxygenation was identified. Indications were assigned based on patient age (neonatal vs pediatric), diagnosis, and procedure codes. Average hospital annual volume was defined as 0-19, 20-49, or greater than or equal to 50 cases per year. Maximum likelihood estimates were used to assess minimum annual case volume. PATIENTS: A total of 7,322 pediatric patients aged 0-18 were supported with extracorporeal membrane oxygenation and had an indication assigned. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Average hospital extracorporeal membrane oxygenation volume ranged from 1 to 58 cases per year. Overall mortality was 43% but differed significantly by indication. After adjustment for case-mix, complexity of cardiac surgery, and year of treatment, patients treated at medium-volume centers (odds ratio, 0.86; 95% CI, 0.75-0.98) and high-volume centers (odds ratio, 0.75; 95% CI, 0.63-0.89) had significantly lower odds of death compared with those treated at low-volume centers. The minimum annual case load most significantly associated with lower mortality was 22 (95% CI, 22-28). CONCLUSIONS: Pediatric centers with low extracorporeal membrane oxygenation average annual case volume had significantly higher mortality and a minimum volume of 22 cases per year was associated with improved mortality. We suggest that this threshold should be evaluated by additional study.

Resource utilization and outcomes of infective endocarditis in children.

OBJECTIVE: To evaluate resource use and outcomes of infective endocarditis in children with and without preexisting heart disease via a national cohort. STUDY DESIGN: Children <19 years of age hospitalized from 2004 to 2010 with infective endocarditis at 37 centers in the Pediatric Health Information Systems database were included. We excluded children primarily hospitalized for chronic medical conditions. We used regression analysis to evaluate factors associated with poor outcomes (defined as mortality, mechanical cardiac support, or stroke). RESULTS: There were 1033 cases of infective endocarditis, of which 663 had heart disease and 370 did not. Compared with the group without heart disease, infective endocarditis in the cohort with heart disease occurred at younger age, was more commonly attributable to streptococcus, was more likely to require cardiac surgery for infective endocarditis, and was associated with a lower risk of stroke. Mortality was 6.7% (n = 45) and 3.5% (n = 13) in groups with and without heart disease, respectively. Factors associated with poor outcome in the cohort with heart disease included greater risk of mortality score (OR 7.9), mechanical ventilation (OR 3.1), use of antiarrhythmics (OR 2.7), and use of vasoactive medications (OR 3.8). In the cohort without heart disease, factors associated with poor outcome included renal failure (OR 19.3), greater risk of mortality score (OR 4.2), use of antiarrhythmics (OR 3.8), and mechanical ventilation (OR 2.2). Median charge of hospitalization was $131,893 in the group without heart disease and $140,655 in the group with heart disease. CONCLUSION: Infective endocarditis remains a significant cause of morbidity, mortality, and resource use particularly in children with heart disease.

Pediatric Digit Replantation: A Nationwide Analysis of Failure Rate, Complications, and Potential Factors Affecting Failure.

Purpose: Function and cosmesis may be improved by replantation following digital amputation in pediatric patients. However, accurate failure and complication rate estimates may be limited as most pertinent studies reflect single center/surgeon experience and therefore are limited by small sample sizes. The primary aim of this study was to assess the rate of failure (amputation) following pediatric digital replantation. Secondary aims include evaluating the rate of complications and associated resource utilization (intensive care unit stays, readmission rate, and hospital length of stay). Methods: Digital replantation patients were identified from 47 pediatric hospitals using the 2004 to 2020 Pediatric Health Information System nationwide database. Using applicable International Classification of Disease 9/10 and Current Procedural Terminology codes, we identified complications after replantation, including revision amputation, infection, surgical complications, medical complications, admission to intensive care unit (ICU), and length of stay. Results: Of the 348 patients who underwent replantation the mean age was 8.3 ± 5.1 years, and 27% were female. Mean hospital length of stay was 5.8 ± 4.7 (range, 1-28) days. Of the 53% of patients who required ICU admission, the mean ICU length of stay was 2.4 ± 3.3 days. Failure/amputation after replantation occurred in 71 (20.4%) patients, at a mean of 9.7 ± 27.2 days postoperatively. Surgical complications occurred in 58 (17%) patients, 30-day hospital readmissions occurred in 5.7% of patients, and 90-day readmissions occurred in 6.3% patients. Conclusion: The estimated rate of failure following pediatric digit replantation was 20%. Our data on failure and complication rates and associated resource utilization may be useful in counseling pediatric replantation patients and their families and provide an update on prior literature. Level of Evidence: IV, Prognosis.