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Pediatric heart transplantation (HT) is resource intensive. Event-driven pediatric databases do not capture data on resource use. The objective of this study was to evaluate resource utilization and identify associated factors during initial hospitalization for pediatric HT. This multicenter retrospective cohort study utilized the Pediatric Health Information Systems database (43 children's hospitals in the United States) of children ≤19 years of age who underwent transplant between January 2007 and July 2013. Demographic variables including site, payer, distance and time to center, clinical pre- and post-transplant variables, mortality, cost, and charge were the data collected. Total length of stay (LOS) and charge for the initial hospitalization were used as surrogates for resource use. Charges were inflation adjusted to 2013 dollars. Of 1,629 subjects, 54% were male, and the median age at HT was 5 years (IQR [interquartile range] 0 to 13). The median total and intensive care unit LOS were 51 (IQR 23 to 98) and 23 (IQR 9 to 58) days, respectively. Total charge and cost for hospitalization were $852,713 ($464,900 to $1,609,300) and $383,600 ($214,900 to $681,000) respectively. Younger age, lower volume center, southern region, and co-morbidities before transplant were associated with higher resource use. In later years, charges increased despite shorter LOS. In conclusion, this large multicenter study provides novel insight into factors associated with resource use in pediatric patients having HT. Peritransplant morbidities are associated with increased cost and LOS. Reducing costs in line with LOS will improve health-care value. Regional and center volume differences need further investigation for optimizing value-based care and efficient use of scarce resources.

Objective. Home O2 has been shown to reduce hospitalizations for bronchiolitis but data on outpatient management of home O2 are lacking. We aim to describe outpatient management and challenges to home O2 for bronchiolitis. Methods. We surveyed Colorado and Utah (where home O2 use is prevalent) chapter members of the American Academy of Pediatrics regarding bronchiolitis home O2 management. Results. A total of 1030 providers were surveyed. The response rate was 21% (n = 214). Ninety percent of practicing primary care providers reported experience with home O2. Of those, 46% see patients on postdischarge day 1. Most providers see patients 1 to 3 times before stopping O2. Eighty percent continue O2 for 3 to 7 days. Weaning procedures vary and 56% practice more than 1 method. Most (41%) do not use continuous pulse oximetry. Challenges include parental noncompliance (51%) and difficulty knowing when to stop the O2 (57%). Conclusions. Management of home O2 in patients with bronchiolitis is a common in UT and CO. Weaning practices vary. Further research is needed.

Background: In 2000, a 7-valent pneumococcal conjugate vaccine (PCV7) was licensed for use among US children. Many sites have since reported changes in invasive pneumococcal disease (IPD). We recognized an opportunity to describe the changes in epidemiology, clinical syndromes, and serotype distribution during a 14-year period including 4 years before vaccine introduction and spanning the entire PCV7 era. Methods: Cases were defined as children 18 years of age who were cared for at Primary Children’s Medical Center for culture-confirmed IPD. We defined the prevaccine period as the time frame spanning from 1997 to 2000 and the postvaccine period from 2001 to 2010. Demographics, clinical data, and outcomes were collected through electronic query and chart review. Streptococcus pneumoniae serotyping was performed using the capsular swelling method. Results: The median age of children with IPD increased from 19 months during the prevaccine period to 27 months during postvaccine period (P 0.02), with a larger proportion of IPD among children older than 5 years. The proportion of IPD associated with pneumonia increased substantially from 29% to 50% (P 0.001). This increase was primarily attributable to an increase in complicated pneumonia (17% to 33%, P 0.001). Nonvaccine serotypes 7F, 19A, 22F, and 3 emerged as the dominant serotypes in the postvaccine period. In children with IPD who were younger than 5 years, for whom vaccine is recommended, 67% of the cases were caused by serotypes in 13-valent PCV during 2005 to 2010. Conclusions: After PCV7 was introduced, significant changes in IPD were noted. One-third of IPD occurred in children older than 5 years, who were outside the age-group for which PCV is recommended. Continued surveillance is warranted to identify further evolution of the epidemiology, clinical syndromes, and serotype distribution of S. pneumoniae after 13-valent PCV licensure.

Disorders of the Ras/mitogen-activated protein kinase (MAPK) pathway have an overlapping skeletal phenotype (e.g. scoliosis, osteopenia). The Ras proteins regulate cell proliferation and differentiation and neurofibromatosis type 1 (NF1) individuals have osteoclast hyperactivity and increased bone resorption as measured by urine pyridinium crosslinks [pyridinoline (Pyd) and deoxypyridinoline (Dpd)]. Pyd and Dpd are hydroxylysine-derived crosslinks of collagen found in bone and cartilage and excreted in the urine. Dpd is most abundant in bone. The aim of this study was to evaluate if other syndromes of the Ras/MAPK pathway have increased bone resorption, which may impact the skeletal phenotype. Participants were individuals with Noonan syndrome (n = 14), Costello syndrome (n = 21), and cardiofaciocutaneous (CFC) syndrome (n = 14). Pyridinium crosslinks from two consecutive first morning urines were extracted after acid hydrolysis and analyzed by high performance liquid chromatography. Three separate analyses of covariance were performed to compare Pyd, Dpd, and Dpd/Pyd ratio of each group to controls after controlling for age. Data were compared to 99 healthy controls. The Dpd and the Dpd/Pyd ratio were elevated (p < 0.0001) in all three conditions compared to controls suggesting that collagen degradation was predominantly from bone. The data suggest that the Ras/MAPK signal transduction pathway is important in bone homeostasis.

TOC Summary: The infection disproportionately affected patients >65 years of age., Since the introduction of the Haemophilus influenzae type b vaccine, the incidence of invasive H. influenzae type b disease among children has fallen dramatically, but the effect on invasive H. influenzae disease among adults may be more complex. In this population-based study we examined the epidemiology and outcomes of invasive disease caused by typeable and nontypeable H. influenzae among Utah adults during 1998–2008. The overall incidence increased over the study period from 0.14/100,000 person-years in 1998 to 1.61/100,000 person-years in 2008. The average incidence in persons >65 years old was 2.74/100,000 person-years, accounting for 51% of cases and 67% of deaths. The incidence was highest for nontypeable H. influenzae (0.23/100,000 person-years), followed by H. influenzae type f (0.14/100,000 person-years). The case-fatality rate was 22%. The incidence of invasive H. influenzae in Utah adults appears to be increasing. Invasive H. influenzae infection disproportionately affected the elderly and was associated with a high mortality rate.

Identify parental beliefs and barriers related to influenza immunization of school-aged children and acceptance of school-based influenza immunization.We conducted a cross-sectional survey of parents of elementary school-aged children in November 2008. Outcomes were receipt of influenza vaccine, acceptance of school-based immunization, and barriers to immunization.Response rate was 65% (259/397). Parents reported that 26% of children had received the vaccine and 24% intended receipt. A total of 50% did not plan to immunize. Factors associated with receipt were belief that immunization is a social norm (adjusted odds ratios [AOR], 10.8; 95% CI, 2.8-41.8), belief in benefit (AOR, 7.8; CI, 1.8-33.8), discussion with a doctor (AOR, 7.0; CI, 2.9-16.8), and belief that vaccine is safe (AOR, 4.0; CI, 1.0-15.8). A total of 75% of parents would immunize their children at school if the vaccine were free, including 59% (76/129) who did not plan to immunize. Factors associated with acceptance of school-based immunization were belief in benefit (AOR, 6.1; 95% CI, 2.7-14.0), endorsement of medical setting barriers (AOR, 3.7; 95% CI, 1.3-10.3), and beliefs that immunization is a social norm (AOR, 3.3; 95% CI, 1.4-7.6) and that the child is susceptible to influenza (AOR, 2.6; 95% CI, 1.2-5.7). Medical setting barriers were competing time demands, inconvenience, and cost; school barriers were parents' desire to be with children and competence of person delivering the vaccine.School-based immunization programs can increase immunization coverage by targeting parents for whom time demands and inconvenience are barriers, demonstrating that immunization is a social norm, and addressing concerns about influenza vaccine benefit and safety.

Background: Pediatric heart transplantation (HT) is resource intensive. Event driven pediatric HT databases do not capture data on resource use. The objective of this study was to evaluate resource utilization and identify associated factors during initial hospitalization for pediatric HT in a large multi-institutional cohort. Methods: This is a multicenter retrospective cohort study using the PHIS database (43 US children’s hospitals) of children ≤ 19 years of age who underwent HT between 1/07 and 7/13. Data collected: Demographic variables including site, payer, distance and time to center, clinical pre and post-transplant variables, mortality, cost and charge. Total length of stay (LOS) and charge for initial hospitalization were used as surrogates for resource use. Charges were inflation adjusted to 2013 $. Gamma regression analysis was performed to evaluate factors associated with resource use. Results: Of 1629 subjects, 54% were male, and the median age at HT was 5 years (IQR 0-13). The median total and ICU LOS were 51 (IQR: 23-98) and 23 (IQR: 9-58) days respectively, and mortality occurred in 82 (5%). Total charge and cost for hospitalization were $852,713 ($464,900-$1,609,300) and $383,600 ($214,900-$681,000) respectively. Factors associated with resource use on multivariate analysis are shown in Table 1. Younger age, lower center volume, southern region, and comorbidities prior to HT were associated with higher resource use. In later years, costs increased despite shorter LOS. Conclusions: This large multicenter study provides novel insight into factors associated with resource use in pediatric HT that cannot be assessed in alternative event driven transplant databases. Peri-transplant morbidities are associated with increased cost and LOS. Reducing costs in line with LOS will improve health care value. Regional and center volume differences need further investigation for optimizing value-based care and efficient use of scarce resources.

The objective of the present study was to characterize the outcomes and resource utilization of all infants born with hypoplastic left heart syndrome (HLHS) in the Intermountain West. This was a retrospective cohort study of all infants born with HLHS in the Intermountain West from January 1995 and January 2010. The cohort was divided into 3 eras: era 1, 1995 to 1999; era 2, 2000 to 2004; and era 3, 2005 to 2010. Cox proportional hazards regression analysis was performed to assess mortality. The lifetime hospitalization days and charges were also determined. Of the 245 infants identified, 65% were male infants and 172 (70%) underwent Stage 1 palliation. The transplant-free survival rate for the entire cohort was 33% at 14 years. The 1-year transplant-free survival rate for the surgical cohort was 60% in era 3. The infants whose initial presentation included shock, restrictive or intact atrial septum, chromosomal defects, or multiorgan dysfunction had an increased risk of death. A recent era of birth, greater birthweight, and older gestational age were associated with improved survival. The factors associated with mortality after stage 1 included surgical procedure type (Blalock-Taussig vs Sano shunt, hazard ratio 2.1), requirement for postoperative extracorporeal membrane oxygenation (hazard ratio 4.2), postoperative renal dysfunction (hazard ratio 3.0), anomalous pulmonary venous return (hazard ratio 2.9), and moderate or greater tricuspid valve regurgitation at any point (hazard ratio 2.0). For patients who had undergone stage 1, 2, or 3 palliation, the median cumulative lifetime hospitalization was 32, 48, and 65 days, and the median cumulative lifetime charges for hospitalization were $201,812, $253,183, and $296,213, respectively. In conclusion, although hospital-based studies of HLHS have shown significantly improved survival after surgical palliation, population-based studies have shown that HLHS continues to have a high mortality and high resource utilization.

From 1996 to 2009, we analyzed changes in pneumococcal disease (PD) in Utah children aged