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Objective:A subset of necrotizing enterocolitis (NEC) cases is fulminant, characterized by rapid progression to death with massive bowel necrosis found at laparotomy or autopsy. We sought to identify and report all such cases in a multihospital healthcare system during the past 9 years and to characterize this entity using case-control methodologies.Study Design:This was a multicentered, cross-sectional, historic cohort study conducted using Intermountain Healthcare hospital patient data. All neonates who died of NEC within 48 h of onset, during 2001 to 2009, were compared with two matched control groups: (1) demographically matched controls who developed non-fulminant NEC, (2) demographically matched controls that did not develop NEC.Result:During this period, 2 71 327 live births occurred in the Intermountain Healthcare hospitals. Of these, 318 had a diagnosis of NEC (Bell stage II). Also during this period, 205 other neonates were transferred into an Intermountain hospital for treatment of NEC. Of these 523 NEC cases, 35 (6.7%) had a fulminant course. Compared with the non-fulminant cases, the fulminant group were born at lower weight (1088±545 vs 1652±817 g, P=0.000) and earlier gestational age (27.5±3.3 vs 31.1±4.4 weeks, P=0.000), and were more likely to have: (1) radiographic evidence of portal venous air (P=0.000), (2) hematocrit <22% (P=0.000), (3) increase in feeding volume >20 ml/kg/day (P=0.003), (4) immature to total (I/T) neutrophil ratio >0.5 (P=0.005), (5) blood lymphocyte count <4000/μl (P=0.018), (6) an increase in concentration of human milk fortifier within 48 h before developing NEC (P=0.020).Conclusion:Portal venous air, anemia, rapid feeding escalation, a high I/T neutrophil ratio, a low lymphocyte count and recent increases in fortifier may all be associated with fulminant NEC. [ABSTRACT FROM AUTHOR]

Objective: The purpose of our study was to understand the healthcare burden and incidence of Krabbe disease (Krabbe)., Methods: Retrospective analysis of Krabbe patients identified October 1, 2015 through December 31, 2020, ages birth through age 3, evaluated in two national databases. We estimated point prevalence and incidence from year 2016 data., Results: We identified 98 unique Krabbe patients with 736 visits including 260 were inpatient admissions. Total healthcare charges were $51.5 million dollars. We determined a point prevalence of 34 68 Krabbe patients in 2016 ages 0 3 years. This estimates a birth incidence of ~1 in 310,000 live births. Significance: Krabbe disease patients had over $51 million in health care charges and hundreds of hospitalizations. Estimated prevalence and birth incidence is similar to rates observed from newborn screening. Our findings show the tremendous health impacts of Krabbe disease, and provide guidance for efforts in screening and treatment planning.

Objective: The aim of the study was to investigate the association between hospitals' nursing excellence accreditation and patient safety performance-measured by the Hospital-Acquired Conditions Reduction Program (HACRP)., Methods: We linked data from the American Nursing Credentialing Center Magnet Recognition Program, Centers for Medicare and Medicaid Services HACRP, and the American Hospital Association annual survey from 2014 to 2016. We constrained the analysis to hospitals participating in Centers for Medicare and Medicaid Services' HACRP and deployed propensity score matching models to calculate the coefficients for our HACRP patient safety measures. These measures consisted of (a) patient safety indicator 90, (b) hospital-associated infection measures, and (c) total HAC scores. In addition, we used propensity score matching to assess HACRP scores between hospitals achieving Magnet recognition in the past 2 versus longer and within the past 5 years versus longer., Results: Our primary findings indicate that Magnet hospitals have an increased likelihood of experiencing lower patient safety indicator 90 scores, higher catheter-associated urinary tract infection and surgical site infection scores, and no different total HAC scores. Finally, when examining the impact of Magnet tenure, our analysis revealed that there were no differences in Magnet tenure., Conclusions: Results indicate that the processes, procedures, and educational aspects associated with Magnet recognition seem to provide important improvements associated with care that is controlled by nursing practice. However, because these improvements do not differ when comparing total HAC scores nor Magnet hospitals with different tenure, there are likely opportunities for Magnet hospitals to continue process improvements focused on HACRP scores., Competing Interests: The authors disclose no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The use of immunoglobulins is gradually increasing. Intravenous immunoglobulins (IVIG) are used as replacement therapy for primary and secondary immune deficiencies, and as an anti-inflammatory and immunomodulatory medication for the treatment of neurologic, dermatologic, and rheumatologic diseases. The objective of this study was to analyze trends in the IVIG use in pediatric patients hospitalized to 47 US-based children's hospitals from 2007 to 2014. IVIG was used for the treatment of >2300 primary diagnoses in 53,648 unique patients. The number of IVIG admissions increased by 30.2% during the study period, while the mean rate of IVIG admissions/100,000 admissions increased only 5.8%. Most patients receiving IVIG were children and adolescents. IVIG was frequently used off-label or for the treatment of FDA-approved indications in children under two years of age and BMT patients <20 years of age. Primary immune deficiencies represented only 1.2% of all IVIG admissions. Pediatric patients with mucocutaneous lymph node syndrome (Kawasaki disease, KD) and idiopathic thrombocytopenic purpura (ITP) were two primary consumers of the IVIG. Another top-ranked indications were acute infectious polyneuritis (Guillain-Barré syndrome, GBS) and prophylaxis of infections in patients receiving antineoplastic chemotherapy. IVIG usage is a dynamic process guided by emerging evidence and FDA approval for new indications. IVIG was mostly prescribed for treatment of diseases with pathologic immune responses to foreign of self-antigens. These indications usually, require higher amounts of IVIG per admission. More studies are needed to understand whether IVIG treatments of off-label indications are effective and cost-efficient., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals., Design: All adults (≥18 years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups: ECMO-free survival, requiring ECMO, and DWE. Univariate analyses were performed, and multinomial logistic regression models were constructed examining ECMO and DWE as independent outcomes., Setting: Tertiary care children's hospitals., Results: A total of 4665 adult patients underwent ACHD surgery in 39 children's hospitals with 51 (1.1%) patients requiring ECMO and 64 (1.4%) patients experiencing DWE. Of the 51 ECMO patients, 34 (67%) died. Increasing patient age, surgical complexity, diagnosis of single ventricle heart disease, preoperative hospitalization, and the presence of noncardiac complex chronic conditions (CCC) were risk factors for both outcomes. Additionally, low and medium hospital ACHD surgical volume was associated with an increased risk of DWE in comparison with ECMO., Conclusions: There are overlapping but separate risk factors for ECMO support and DWE among adults undergoing congenital heart surgery in pediatric hospitals., (© 2019 Wiley Periodicals, Inc.)

Inherited leukodystrophies are a group of diseases affecting central nervous system myelin that lead to death or significant health problems. Although for most leukodystrophies there are no curative treatments, for a handful of diseases hematopoietic stem cell transplantation (HSCT; bone marrow transplant) can stop disease progression, and if initiated in a timely fashion, prevent many or all neurologic and other systems involvement. However, HSCT is a complex procedure with significant morbidity and mortality risks. The study goal was to determine whether HSCT was being more widely used outside of those leukodystrophies for which HSCT is typically employed. The authors conducted a 2-year retrospective review of HSCT performed across the United States in 51 children's hospitals that are part of the Pediatric Health Information System. The authors screened for 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) codes for leukodystrophies in which HSCT is "nonstandard," including sphingolipidoses, Fabry disease, Gaucher disease, and Niemann-Pick disease, and excluded patients who had ICD-10 codes for leukodystrophies that are HSCT candidates, specifically X-linked adrenoleukodystrophy, metachromatic leukodystrophy, Krabbe disease, and Hurler disease. The authors identified 91 patients (from a total cohort of 937) with one of the nonstandard leukodystrophies who had HSCT. HSCT was performed at 20 of the hospitals, with the majority performed at only 6 hospitals. Average costs ($786 846) per patient were more than 6 times higher than patients who did not have HSCT. The data show that an unexpectedly large number of leukodystrophy patients are receiving transplants for conditions in which HSCT is not typically used, and which are associated with high medical costs.

Importance: Inherited leukodystrophies are a group of neurological diseases affecting myelin that cause significant morbidities and death. Timely and correct diagnosis is important for initiating treatment, designing disease screening, and offering care and guidance to patients and families., Objective: To determine whether there are disparities in leukodystrophy diagnosis in different racial backgrounds., Design, Setting, and Participants: This case-control study involved a retrospective review of patients aged 18 years or younger who were diagnosed with 1 of 4 leukodystrophies (metachromatic leukodystrophy, X-linked adrenoleukodystrophy, Krabbe disease, and Hurler disease) in the US Children's Hospital Association's Pediatric Health Information System database from October 1, 2015, through September 30, 2017., Main Outcomes and Measures: Leukodystrophy diagnosis and racial background of the patients were analyzed. Adjusted prevalence estimates of leukodystrophies were obtained by controlling for sex, insurance type, urban or rural status, 2010 median household income for patient zip code, number of inpatient days, and age at first visit. Pathogenic leukodystrophy gene allele frequencies in different racial backgrounds for ABCD1, ARSA, GALC, and IDUA were determined using the gnomAD database., Results: Of the 557 patients identified with a leukodystrophy (221 [40%] female; 321 [58%] white non-Hispanic, 54 [10%] black non-Hispanic, and 51 [9%] white Hispanic; median [range] age, 7 [0-18] years), nonwhite race, including black non-Hispanic, black Hispanic, and white Hispanic, was associated with not having a leukodystrophy diagnosis. The adjusted prevalence for a leukodystrophy diagnosis in white non-Hispanic patients was 13.8 (95% CI, 10.6-17.9) per 100 000 patients, compared with 5.8 (95% CI, 3.8-8.9), 2.4 (95% CI, 1.1-5.2), and 7.4 (95% CI, 5.2-10.4) per 100 000 in black non-Hispanic, black Hispanic, and white Hispanic patients, respectively. This reduced rate of diagnosis was out of proportion to the frequency of the different races in the Pediatric Health Information System database. Similar or higher frequencies of missense or loss-of-function alleles were measured in populations of Latino and African descent for the pathogenic leukodystrophy gene alleles. For example, for ABCD1, allele frequencies in those of Latino or African descent were 2.1 × 10-5 and 2.2 × 10-5, as compared with 1.4 × 10-5 for those of European non-Finnish descent., Conclusions and Relevance: Patients of racial/ethnic minorities, including those from black, black Hispanic, and white Hispanic backgrounds, were significantly less likely to be diagnosed with a leukodystrophy. Leukodystrophy disease-associated allele frequencies were the same or higher in populations of Latino or African descent, arguing against a genetic founder effect being responsible for the lower diagnosis rates. This underdiagnosis has implications for newborn screening programs and treatment access and may reflect a more widespread problem in pediatric neurological and orphan diseases.

Objective: Compare trends in coronary artery (CA) abnormality diagnoses to trends in adverse cardiac outcomes among American children with Kawasaki disease (KD) to assess the fit of detection of CA abnormalities to an established model of overdiagnosis., Design: Multicenter retrospective cohort., Setting: 48 US children's hospitals in the Paediatric Health Information System database., Participants: Children <18 years receiving care for KD between 2000 and 2014., Main Outcome Measures: The main outcomes were rates of CA abnormality diagnoses and adverse cardiac outcomes, measured during a child's incident KD visit and longitudinally at all subsequent visits to the same hospital, through December 2016. CA abnormalities were considered severe if long-term anticoagulation other than aspirin was prescribed. Trends were tested using mixed effects logistic regression, adjusting for patient demographics., Results: Among 17 809 children treated for KD, a CA abnormality was diagnosed in 1435 children (8%), including 1117 considered non-severe and 318 severe. The rate of non-severe CA abnormality diagnoses increased from 45 per 1000 patients with KD in 2000 to 81 per 1000 patients with KD in 2014, representing an adjusted 2.3-fold increased odds (95% CI 1.8 to 3.0) of diagnosis. There was no significant change in diagnoses of severe CA abnormalities. Adverse cardiac outcomes were stable over the study period at 19 per 1000 patients with KD (P=0.24 for trend)., Conclusions: The rising rate of detection of non-severe CA abnormalities accompanied by an unchanging rate of adverse cardiac outcomes among American children with KD fits an overdiagnosis pattern., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objectives: Previous studies demonstrate racial and ethnic disparities among children undergoing congenital heart surgery. Extracorporeal membrane oxygenation (ECMO) is used to support critically ill children after congenital heart surgery and improve survival. Thus, racial or ethnic variations in postoperative ECMO use following congenital heart surgery may be associated with racial/ethnic disparities in hospital survival., Methods: All children in the Pediatric Health Information Systems dataset undergoing congenital heart surgery from 2004 to 2015 were examined. Multivariable, multinomial regression models examining hospital survival without ECMO use, survival after ECMO, death after ECMO, and death without ECMO support were constructed., Results: Of 130,860 congenital cardiac surgery patients, 95.4% survived to hospital discharge without requiring ECMO support, whereas 1.3% survived after ECMO support, 1.3% died after ECMO support, and 1.9% died without receiving ECMO support. After adjustment for other covariates, black patients (odds ratio, 1.22; 95% confidence interval [CI], 1.05-1.42) and patients of other race (odds ratio, 1.36; 95% CI, 1.17-1.58) were at increased odds of mortality compared with white patients. In multivariable multinomial models, black patients had increased risk of death without ECMO support (relative risk, 1.31; 95% CI, 1.11-1.56). Patients of other race (relative risk, 1.37; 95% CI, 1.10-1.69) and governmental insurance (relative risk, 1.24; 95% CI, 1.12-1.37) were also at increased risk of death without ECMO., Conclusions: Black children and children of other race are at increased odds of mortality after congenital heart surgery. These disparities can be traced to variations in ECMO utilization across racial/ethnic groups., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To describe asthma management, investigate practice variation, and describe asthma-associated charges and resource use during asthma management in the PICU., Methods: Children ages 2 to 18 years treated for status asthmaticus in the PICU from 2008 to 2011 are included in this study. This is a retrospective, single-center, cohort study. Data were collected by using the Intermountain Healthcare Enterprise Data Warehouse., Results: There were 262 patients included and grouped by maximal respiratory support intervention. Seventy percent of the patients did not receive escalation of respiratory support beyond nasal cannula or nonrebreather mask, and the majority of these patients received only first-tier recommended therapy. For all patients, medical imaging and laboratory charge fractions accounted for <3% and <5% of the total charges, respectively. Among nonintubated patients, the majority of these diagnostic test results were normal. Fifteen patients were intubated during our study period; 4 were intubated at our facility. Compared with outside hospital intubations, these 4 patients had longer time to intubation (>3 days versus <24 hours) and significantly longer median PICU length of stay (12.7 days versus 2.6 days)., Conclusions: In our study, the vast majority of patients with severe asthma were treated with minimal interventions alone (nasal cannula or nonrebreather mask and first-tier medications). Minimizing PICU length of stay is likely the most successful way to decrease expense during asthma care., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2018 by the American Academy of Pediatrics.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Inherited leukodystrophies are progressive, debilitating neurological disorders with few treatment options and high mortality rates. Our objective was to determine national variation in the costs for leukodystrophy patients and to evaluate differences in their care., Methods: We developed an algorithm to identify inherited leukodystrophy patients in deidentified data sets using a recursive tree model based on International Classification of Disease, 9th Edition, Clinical Modification, diagnosis and procedure charge codes. Validation of the algorithm was performed independently at two institutions, and with data from the Pediatric Health Information System (PHIS) of 43 US children's hospitals, for a 7-year period between 2004 and 2010., Results: A recursive algorithm was developed and validated, based on six International Classification of Disease, 9th Edition, Clinical Modification, codes and one procedure code that had a sensitivity up to 90% (range 61-90%) and a specificity up to 99% (range 53-99%) for identifying inherited leukodystrophy patients. Inherited leukodystrophy patients comprise 0.4% of admissions to children's hospitals and 0.7% of costs. During 7 years, these patients required $411 million of hospital care, or $131,000/patient. Hospital costs for leukodystrophy patients varied at different institutions, ranging from two to 15 times more than the average pediatric patient. There was a statistically significant correlation between higher volume and increased cost efficiency. Increased mortality rates had an inverse relationship with increased patient volume that was not statistically significant., Conclusions: We developed and validated a code-based algorithm for identifying leukodystrophy patients in deidentified national datasets. Leukodystrophy patients account for $59 million of costs yearly at children's hospitals. Our data highlight potential to reduce unwarranted variability and improve patient care., (Copyright © 2013 Elsevier Inc. All rights reserved.)