Comparison of combined heart‒liver vs heart-only transplantation in pediatric and young adult Fontan recipients.

BACKGROUND: Indications for a heart‒liver transplantation (HLT) for Fontan recipients are not well defined. We compared listing characteristics, post-operative complications, and post-transplant outcomes of Fontan recipients who underwent HLT with those of patients who underwent heart-only transplantation (HT). We hypothesized that patients who underwent HLT have increased post-operative complications but superior survival outcomes compared with patients who underwent HT. METHODS: We performed a retrospective review of Fontan recipients who underwent HLT or HT at a single institution. Characteristics at the time of listing, including the extent of liver disease determined by laboratory, imaging, and biopsy data, were compared. Post-operative complications were assessed, and the Kaplan‒Meier survival method was used to compare post-transplant survival. Univariate regression analyses were performed to identify the risk factors for increased mortality and morbidity among patients who underwent HT. RESULTS: A total of 47 patients (9 for HLT, 38 for HT) were included. Patients who underwent HLT were older, were more likely to be on dual inotrope therapy, and had evidence of worse liver disease. Whereas ischemic time was longer for the group who underwent HLT, post-operative complications were similar. Over a median post-transplant follow-up of 17 (interquartile range: 5-52) months, overall mortality for the cohort was 17%; only 1 patient who underwent HLT died (11%) vs 7 patients who underwent HT (18%) (p = 0.64). Among patients who underwent HT, cirrhosis on pre-transplant imaging was associated with worse outcomes. CONCLUSIONS: Despite greater inotrope need and more severe liver disease at the time of listing, Fontan recipients undergoing HLT have post-transplant outcomes comparable with those of patients undergoing HT. HLT may offer a survival benefit for Fontan recipients with liver disease.

Racial and Socioeconomic Disparities in Hospitalization of Pediatrics with Liver Disease from 2005 to 2015.

BACKGROUND: Adult liver-related hospitalizations have recently increased in the USA, but data are limited for the pediatric population. AIMS: Utilizing the Office of Statewide Health Planning and Development hospital claims database (covering > 98% of all California hospitalizations), we aimed to characterize the demographic, clinical, and socioeconomic factors of liver disease-associated admissions among children between 2005 and 2015. METHODS: We used ICD-9 codes to identify admissions associated with liver disease in patients up to 21 years of age. Patient characteristics were described as percentages and evaluated using the χ2 test. We used linear regression to examine changes over time. RESULTS: We analyzed 37,372 eligible admissions. Overall, close to one-third (28%) and one-half (48.0%) of admissions occurred in the age group 0-5 years and 16-21 years, respectively, with the remaining 23.1% occurring in the age group between 5 and 15 years. Over half (54.9%) were in males. By race, blacks made up half of the admission (49.7%), while by ethnicity, Hispanic also accounted for half of the admission (49.7%). Medicaid and Medicare payors were also disproportionately represented (54.6%). The most common liver disease was Alagille syndrome (29.2%) in 2005. Between 2005 and 2015, both the number of pediatric liver-associated admissions and the proportion of pediatric liver admissions over total admissions increased from 3130 to 3429 and 1.2% to 1.6%, respectively (both p = 0.001). By 2015, while Alagille syndrome admissions decreased to 26.4% (p = 0.004), NAFLD admission increased to 19.7% (p < 0.001). CONCLUSION: Major disparities exist in inpatient liver disease burden for blacks and Hispanics with liver disease, while NAFLD emerged as a rapidly rising liver disease in pediatrics.

Procalcitonin as a Predictive Marker for Bacteremia in Children With a Central Line and Fever.

OBJECTIVES: Unnecessary use of antibiotics is an increasing problem. In this study, we sought to determine the diagnostic accuracy of procalcitonin in predicting bacteremia in children with a central line and fever, and we sought to determine optimal cutoff values to maximize sensitivity and specificity. This is the largest study to date in which procalcitonin is examined as a predictive marker of bacteremia in pediatric patients with a central line and fever. METHODS: We conducted a retrospective cohort study of children aged 0 to 23 years with a central line and fever of 38°C who had procalcitonin and blood cultures drawn before initiation of antibiotics and had no other identified bacterial infection. Patients were also prospectively monitored via a custom-built electronic medical record dashboard for eligibility. RESULTS: There were 523 patients and >2500 procalcitonin values reviewed for eligibility. Of these, 169 (47%) patients and 335 blood cultures with procalcitonin were included. There were 94 (28%) positive bacterial blood cultures and 241 (72%) negative bacterial blood cultures. In bacteremic cultures, the mean procalcitonin level was 9.96 ± 15.96 ng/mL, and the median procalcitonin level was 4.85 ng/mL (interquartile range 18.5). In nonbacteremic cultures, the mean procalcitonin level was 1.23 ± 10.37 ng/mL, and the median procalcitonin level was 0.3 ng/mL (interquartile range 0.7). A receiver operating characteristic analysis indicated a procalcitonin level of ≥0.6 ng/mL as the best cutoff point that produced a sensitivity of 85.6% and a specificity of 65.7% (area under the curve 0.85). CONCLUSIONS: Procalcitonin is a sensitive biomarker in predicting bacteremia in children with a central line and fever.

Depression and Health Care Use in Patients With Inflammatory Bowel Disease.

BACKGROUND: Depression frequently co-occurs in patients with inflammatory bowel disease [IBD] and is a driver in health care costs and use. AIM: This study examined the associations between depression and total health care costs, emergency department [ED] visits, computed tomography [CT] during ED/inpatient visits, and IBD-related surgery among IBD patients. METHODS: Our sample included 331772 IBD patients from a national administrative claims database [Truven Health MarketScan® Database]. Gamma and Poisson regression analyses assessed differences related to depression, controlling for key variables. RESULTS: Approximately 16% of the IBD cohort was classified as having depression. Depression was associated with a $17,706 (95% confidence interval [CI] [$16,892, 18,521]) increase in mean annual IBD-related health care costs and an increased incidence of ED visits (adjusted incidence rate ratio [aIRR] of 1.5; 95% CI [1.5, 1.6]). Among patients who had one or more ED/inpatient visits, depression was associated with an increased probability of receiving repeated CT [one to four scans, adjusted odds ratio [aOR] of 1.6; 95% CI [1.5, 1.7]; five or more scans, aOR of 4.6; 95% CI [2.9, 7.3]) and increased odds of undergoing an IBD-related surgery (aOR of 1.2; 95% CI [1.1, 1.2]). Secondary analysis with a paediatric subsample revealed that approximately 12% of this cohort was classified as having depression, and depression was associated with increased costs and incidence rates of ED visits and CT, but not of IBD-related surgery. CONCLUSIONS: Quantifiable differences in health care costs and patterns of use exist among patients with IBD and depression. Integration of mental health services within IBD care may improve overall health outcomes and costs of care.

Starting Young: Trends in Opioid Therapy Among US Adolescents and Young Adults With Inflammatory Bowel Disease in the Truven MarketScan Database Between 2007 and 2015.

Background: Opioids are commonly prescribed for relief in inflammatory bowel disease (IBD). Emerging evidence suggests that adolescents and young adults are a vulnerable population at particular risk of becoming chronic opioid users and experiencing adverse effects. Objectives: This study evaluates trends in the prevalence and persistence of chronic opioid therapy in adolescents and young adults with IBD in the United States. Method: A longitudinal retrospective cohort analysis was conducted with the Truven MarketScan Database from 2007 to 2015. Study subjects were 15-29 years old with ≥2 IBD diagnoses (Crohn's: 555/K50; ulcerative colitis: 556/K51). Opioid therapy was identified with prescription claims within the Truven therapeutic class 60: opioid agonists. Persistence of opioid use was evaluated by survival analysis for patients who remained in the database for at least 3 years following index chronic opioid therapy use. Results: In a cohort containing 93,668 patients, 18.2% received chronic opioid therapy. The annual prevalence of chronic opioid therapy increased from 9.3% in 2007 to 10.8% in 2015 (P < 0.01), peaking at 12.2% in 2011. Opioid prescriptions per patient per year were stable (approximately 5). Post hoc Poisson regression analyses demonstrated that the number of opioid pills dispensed per year increased with age and was higher among males. Among the 2503 patients receiving chronic opioid therapy and followed longitudinally, 30.5% were maintained on chronic opioid therapy for 2 years, and 5.3% for all 4 years. Conclusion: Sustained chronic opioid use in adolescents and young adults with IBD is increasingly common, underscoring the need for screening and intervention for this vulnerable population.

Nationwide Trends in Acute and Chronic Pancreatitis Among Privately Insured Children and Non-Elderly Adults in the United States, 2007-2014.

BACKGROUND & AIMS: Epidemiologic analyses of acute pancreatitis (AP) and chronic pancreatitis (CP) provide insight into causes and strategies for prevention and affect allocation of resources to its study and treatment. We sought to determine current and accurate incidences of AP and CP, along with the prevalence of CP, in children and adults in the United States. METHODS: We collected data from the Truven MarketScan Research Databases of commercial inpatient and outpatient insurance claims in the United States from 2007 through 2014 (patients 0-64 years old). We calculated the incidences of AP and CP and prevalence of CP based on International Classification of Diseases, 9th Revision diagnosis codes. Children were defined as 18 years or younger and adults as 19 to 64 years old. RESULTS: The incidence of pediatric AP was stable from 2007 through 2014, remaining at 12.3/100,000 persons in 2014. Meanwhile, the incidence for adult AP decreased from 123.7/100,000 persons in 2007 to 111.2/100,000 persons in 2014. The incidence of CP decreased over time in children (2.2/100,000 persons in 2007 to 1.9/100,000 persons in 2014) and adults (31.7/100,000 persons in 2007 to 24.7/100,000 persons in 2014). The prevalences of pediatric and adult CP were 5.8/100,000 persons and 91.9/100,000 persons, respectively, in 2014. Incidences of AP and CP increased with age. We found little change in incidence during the first decade of life but linear increases starting in the second decade. CONCLUSIONS: We performed a comprehensive epidemiologic analysis of privately insured, non-elderly adults and children with AP and CP in the United States. Changes in gallstone formation, smoking, and alcohol consumption, along with advances in pancreatitis management, may be responsible for the stabilization and even decrease in the incidences of AP and CP.

Near-Fatal Gastrointestinal Hemorrhage in a Child with Medulloblastoma on High Dose Dexamethasone.

A four-year-old female was admitted to a university-based children's hospital with a newly-diagnosed posterior fossa tumor. She was started on famotidine and high-dose dexamethasone and underwent gross total resection of a medulloblastoma. She was continued on dexamethasone and famotidine. She exhibited postoperative posterior fossa syndrome and was started on enteral feeds via the nasoduodenal tube. She had small gastrointestinal bleeds on postoperative days eight, 11, and 18, and was found to have a well-circumscribed posterior duodenal ulcer. On postoperative day 19, she suffered a massive life-threatening gastrointestinal bleed requiring aggressive resuscitation with blood products. She required an emergent laparotomy due to ongoing blood loss and she was found to have posterior duodenal wall erosion into her gastroduodenal artery. She recovered and subsequently began delayed chemotherapy. This case demonstrates a rare and life-threatening complication of high-dose dexamethasone therapy in the setting of posterior fossa pathology despite stress ulcer prophylaxis. We present a historical perspective with the review of the association between duodenal and intracranial pathology and the usage of high-dose dexamethasone in such cases.

Implementation of Depression Screening and Global Health Assessment in Pediatric Subspecialty Clinics.

PURPOSE: Adolescents with chronic illness face greater risk of psychosocial difficulties, complicating disease management. Despite increased calls to screen for patient-reported outcomes, clinical implementation has lagged. Using quality improvement methods, this study aimed to investigate the feasibility of standardized screening for depression and assessment of global health and to determine recommended behavioral health follow-up, across three pediatric subspecialty clinics. METHODS: A total of 109 patients aged 12-22 years (median = 16.6) who were attending outpatient visits for treatment of diabetes (80% type 1), inflammatory bowel disease, or cystic fibrosis completed the 9-item Patient Health Questionnaire (PHQ-9) depression and Patient-Reported Outcomes Measurement Information System (PROMIS) Pediatric Global Health measures on electronic tablets. Patients screening positive on the PHQ-9 received same-day behavioral health assessment and regular phone check-ins to facilitate necessary follow-up care. RESULTS: Overall, 89% of 122 identified patients completed screening during a 6-month window. Patients completed measures in a timely manner (within 3 minutes) without disruption to clinic flow, and they rated the process as easy, comfortable, and valuable. Depression scores varied across disease type. Patients rated lower global health relative to a previously assessed validation cohort. Depression and global health related significantly to certain medical outcomes. Fifteen percent of patients screened positive on the PHQ-9, of whom 50% confirmed attending behavioral health appointments within 6 months of screening. CONCLUSIONS: A standardized depression and global health assessment protocol implemented across pediatric subspecialties was feasible and effective. Universal behavioral health screening for adolescents and young adults living with chronic disease is necessary to meet programmatic needs in pediatric subspecialty clinics.

Diabetes Device Use in Adults With Type 1 Diabetes: Barriers to Uptake and Potential Intervention Targets.

OBJECTIVE: Diabetes devices (insulin pumps, continuous glucose monitors [CGMs]) are associated with benefits for glycemic control, yet uptake of these devices continues to be low. Some barriers to device uptake may be modifiable through psychosocial intervention, but little is known about which barriers and which patients to target. RESEARCH DESIGN AND METHODS: We surveyed 1,503 adult T1D Exchange participants (mean age 35.3 [SD 14.8] years, mean diagnosis duration 20.4 [SD 12.5] years) to investigate barriers to device uptake, understand profiles of device users versus nonusers, and explore differences by age and sex. Scales used were the Diabetes Distress Scale, Technology Use Attitudes (General and Diabetes-Specific), and Barriers to Device Use and Reasons for Discontinuing Devices. RESULTS: Most commonly endorsed modifiable barriers were related to the hassle of wearing devices (47%) and disliking devices on one's body (35%). CGM users (37%) were older than nonusers (mean 38.3 vs. 33.5 years), had diabetes for longer (22.9 vs. 18.8 years), had more positive technology attitudes (22.6-26.0 vs. 21.4-24.8), and reported fewer barriers to using diabetes technology than nonusers (3.3 vs. 4.3). The youngest age-group (18-25 years) had the lowest CGM (26% vs. 40-48%) and insulin pump (64% vs. 69-77%) uptake, highest diabetes distress (2.2 vs. 1.8-2.1), and highest HbA1c levels (8.3% [67 mmol/mol] vs. 7.2-7.4% [55-57 mmol/mol]). CONCLUSIONS: Efforts to increase device use need to target physical barriers to wearing devices. Because young adults had the lowest device uptake rates, highest distress, and highest HbA1c compared with older age-groups, they should be the focus of future interventions to increase device use.

Spanish and English Language Symposia to Enhance Activation in Pediatric Inflammatory Bowel Disease.

OBJECTIVES: Patient activation is an important consideration for improved health outcomes in the management of chronic diseases. Limited English proficiency among patients and primary care providers has been shown to be a predictor for worse health across disease states. We aimed to determine the baseline patient activation measure (PAM) among Spanish-speaking (SP) and English-speaking (ES) pediatric patients with IBD and parents, and to describe the feasibility and efficacy of a novel peer-group education symposium designed to enhance patient activation as measured with the PAM. METHODS: Two separate half-day educational symposia in either Spanish or English were presented and moderated by 2 native SP physicians. Content for each of the presentations were highly standardized and interactive, designed to address each of the activation domains (self-management, collaboration with a health care provider, maintenance of function and prevention of disease exacerbation, and appropriate access to high-quality care). Descriptive statistics were used to describe changes between pre- and postsymposium PAM trends. RESULTS: Eleven primarily SP and 21 ES families participated in their respective symposium. Paired pre- and post-PAM scores were available from 24 pediatric patients with IBD (8 SP, 16 ES) and 41 parents (15 SP, 26 ES). The mean age for SP and ES patients was 11.6 and 12.0 years, and female sex in 80% and 62%, respectively. Paired pre- and post-PAM scores for all participants (n = 65) were analyzed. PAM scores uniformly increased in all 4 groups after the symposia (SP patients 59.1-70.3, P = 0.05; SP parents 69.8-75.2, P = 0.2; ES patients 59.9-64.0, P = 0.08; ES parents 61.9-69.1, P = 0.002), although only the ES-parents group had sufficient sample size (n = 26) to achieve statistical significance. The overall cohort had an aggregate increase from pre-PAM of 62.9 (SD 14.5) to post-PAM of 69.4 (SD 13.9) (<0.001). CONCLUSIONS: We describe a novel peer-group educational symposium presented in Spanish and English languages to increase patient and parent activation in pediatric patients with IBD and their caregiving parents. The use of PAM to assess levels of activation appears to be feasible and effective in these groups.

Better health, less spending: Redesigning the transition from pediatric to adult healthcare for youth with chronic illness.

Adolescents and young adults (AYA) with serious chronic illnesses face costly and dangerous gaps in care as they transition from pediatric to adult health systems. New, financially sustainable approaches to transition are needed to close these gaps. We designed a new transition model for adolescents and young adults with a variety of serious chronic conditions. Our explicit goal was to build a model that would improve the value of care for youth 15-25 years of age undergoing this transition. The design process incorporated a review, analysis, and synthesis of relevant clinical and health services research; stakeholder interviews; and observations of high-performing healthcare systems. We identified three major categories of solutions for a safer and lower cost transition to adult care: (1) building and supporting self-management during the critical transition; (2) engaging receiving care; and (3) providing checklist-driven guide services during the transition. We propose that implementation of a program with these interventions would have a positive impact on all three domains of the triple aim - improving health, improving the experience of care, and reducing per capita healthcare cost. The transition model provides a general framework as well as suggestions for specific interventions. Pilot tests to assess the model's ease of implementation, clinical effects, and financial impact are currently underway.

Transitions in Pediatric Gastroenterology: Results of a National Provider Survey.

OBJECTIVES: Transition and transfer to adult-oriented health care is an important yet challenging task for adolescents and young adults with chronic medical conditions. Transition practices vary widely, but a paucity of data makes determination of best practices difficult. We described North American pediatric gastroenterologists' preferences and present transition practice patterns and explored whether experience affected providers' perspectives. METHODS: An online survey was distributed via e-mail to members of the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition. Participation was voluntary and answers were anonymous. Quantitative and qualitative analysis was performed. RESULTS: Almost three quarters of the 175 respondents describe providing transition or self-care management education, but only 23% use structured readiness assessments. Most respondents (88%) report having age cutoffs above which they no longer accept new referrals, with the most common age being 18 years (57%). One third report the ability to provide age-appropriate care to patients older than 21 years. Only 6% indicate that their practice or institution should provide care for individuals older than 25 years. Many (63%) indicate that their practice or institution has a policy regarding age of transfer, but most (79%) are flexible. Provider preferences for triggers to transfer to adult care diverge widely between age, milestones, and comorbidities. Overall, parent (81%) and patient (74%) attachment to pediatric health care providers are cited as the most common barriers to transition. CONCLUSIONS: Preferences and practices surrounding transition preparation and transfer to adult care vary widely, reflecting continued uncertainty regarding optimal transition strategies.

Legislative advocacy: evaluation of a grand rounds intervention for pediatricians.

OBJECTIVE: To evaluate the impact of a Grand Rounds Action Alert (GRAA) intervention on the behaviors, knowledge, and attitudes of pediatric grand rounds (GR) attendees; and to assess its acceptability. METHODS: A cross-sectional, quasi-experimental study was performed at a freestanding children's hospital. GRAA on child health legislative topics were presented in the first 2 minutes of the pediatric GR session as well as posted outside. Each session included an action item, such as writing/signing letters to elected officials or informational sheets with legislator contact information. Main outcome measures included self-reported behavior, advocacy knowledge, attitudes, and acceptability. RESULTS: One year after GRAA implementation, GR attendees with high exposure to the intervention were more likely to have written/signed a letter to a legislator compared to those with low/no exposure (60% vs 35%, P = .016). Those with high exposure were also more knowledgeable regarding financing of health care for low-income children (20% vs 5%, P = .027). Attitudes toward advocacy at baseline were positive: respondents agreed it is important to remain informed about (98%) and advocate for (94%) legislation favorable to children's health. Implementing this program was challenging, but the intervention was accepted favorably: 93% of respondents agreed that GRAA should continue. CONCLUSIONS: GRAA facilitated participation in legislative advocacy behaviors while improving self-perceived knowledge of legislative issues relating to children's health. They were well received in a large tertiary children's hospital.

Geographical rural status and health outcomes in pediatric liver transplantation: an analysis of 6 years of national United Network of Organ Sharing Data.

OBJECTIVE: To determine whether children in rural areas have worse health than children in urban areas after liver transplantation (LT). STUDY DESIGN: We used urban influence codes published by the US Department of Agriculture to categorize 3307 pediatric patients undergoing LT in the United Network of Organ Sharing database between 2004 and 2009 as urban or rural. Allograft rejection, patient death, and graft failure were used as primary outcome measures of post-LT health. Pediatric end-stage liver disease/model of end-stage liver disease scores >20 was used to measure worse pre-LT health. RESULTS: In a multivariate analysis, we found greater rates of allograft rejection within 6 months of LT (OR 1.27; 95% CI 1.05-1.53) and a lower occurrence of posttransplantation lymphoproliferative disorder (OR 0.64; 95% CI 0.41-0.99) in patients in rural areas. The difference in allograft rejection was eliminated at 1 year of LT (OR 1.18; 95% CI 0.98-1.42). Rural location did not impact other outcome measures. CONCLUSION: We conclude that rural location makes a negative impact on patient health within the first 6 months of LT by increasing the risk for allograft rejection, although patients in rural areas may have lower rates of developing posttransplantation lymphoproliferative disorder. Long-term adverse health effects were not seen.

Effects of rural status on health outcomes in pediatric liver transplantation: a single center analysis of 388 patients.

Rural status of patients may impact health before and after pediatric LT. We used UI codes published by the USDA to stratify patients as urban or rural depending county residence. A total of 388 patients who had LT and who met criteria were included. Rejection, PTLD, and survival were used as primary outcome measures of post-LT health. UNOS Status 1 and PELD/MELD scores >20 were used as secondary outcome measures of poorer pre-LT health. Logistic regression models were run to determine associations. We did not find any statistically significant differences in pre- or post-LT outcomes with respect to rurality. Among rural patients, there was a general trend for decreased incidence of rejection (25.0% vs. 33.4%; OR 0.64, 95% CI 0.29-1.44), increased risk of PTLD (5.6% vs. 3.4%; OR 1.86, 95% CI 0.36-3.31), and decreased survival (OR 0.85, 95% CI 0.34-2.13) after LT. Rural patients also tended to be sicker at the time of LT than patients from urban areas, with increased proportion of Status 1 (OR 1.17, 95% CI 0.51-2.70) and PELD/MELD scores >20 (OR 1.20, 95% CI 0.59-2.45). From a single center experience, we conclude that rurality did not significantly affect health outcomes after LT, although a larger study may validate the general trends that rural patients may have decreased rejection, increased PTLD, and mortality, and be in poorer health at the time of LT.