Monitoring Practices After Pediatric Liver Transplantation.

OBJECTIVES: This study sought to understand the current monitoring practices after pediatric liver transplantation (LT), specifically regarding follow-up clinic visits, outpatient laboratory testing, protocol biopsies, and diagnostic imaging, and to identify potential center and provider characteristics associated with such practices. METHODS: A cross-sectional survey of pediatric LT providers at centers participating in the Society of Pediatric Liver Transplantation (SPLIT) registry was conducted from February 2020 to April 2021. RESULTS: The overall response rate was 79% (38/48 SPLIT centers), with the majority representing large volume centers (>10 LTs per year). Frequency of clinic visits and laboratory monitoring varied by center, but all centers decreased frequency after the first post-transplant year. The most common practice included an annual clinic visit and laboratory sampling every 2-3 months. Surveillance liver biopsy is seldom done during the first post-transplant year, while being routinely performed by 50% of centers after this time period. Centers forgoing surveillance biopsies assert that the results would likely not change management. Only 39% of centers have a hepatologist perform the liver biopsy while the remaining centers consult interventional radiology. Most diagnostic imaging is obtained only as needed. Routine abdominal ultrasounds were obtained by only 50% of responding centers after the first year post-transplant. CONCLUSIONS: SPLIT centers vary widely in the routine management of LTs after the first year post-transplant. While common themes emerge, future studies will be needed to connect protocols to outcomes to determine best practice.

The effect of neuropsychiatric medication on pediatric nonalcoholic fatty liver disease.

Obese and overweight children are at risk of developing nonalcoholic fatty liver disease (NAFLD), which can lead to steatohepatitis, cirrhosis, and liver transplantation. Neuropsychiatric conditions affect an increasing proportion of children and often require neuropsychiatric medications (NPMs) that are associated with weight gain and/or drug-induced liver injury. We sought to evaluate the role that the extended use of NPMs play in pediatric NAFLD. Medical chart review was conducted for 260 patients with NAFLD (NPM = 77, non-NPM = 183) seen in the Liver Care Center at Children's Mercy Hospital between 2000 and 2016. Outcome measures included body mass index (BMI) percentile, BMI z-score, aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin, and gamma glutamyltransferase, and were collected at diagnosis, 6-18 month follow-up, and 18-36 months. Controlling for race and metformin, there was a significant increase over time in BMI z-score (p < 0.01) and total bilirubin (p = 0.03), with only initial decreases in ALT (p < 0.01) and AST (p < 0.01). Except for higher total bilirubin in the non-NPM group, no main effect of group or interaction effect was found. Similar patterns remained when subjects were analyzed by NPM drug class. Further study is needed to confirm these findings and to evaluate the effects of NPM dose and duration of exposure, by drug class, on pediatric NAFLD outcomes.

Adherence to laboratory testing in pediatric liver transplant recipients.

BACKGROUND: The objectives of this retrospective cohort study are to describe rates of adherence to laboratory testing 6 months to 3 years post-liver transplantation and to examine demographic and clinical factors related to lab non-adherence and the association with medication adherence and clinical outcomes. METHODS: Medical chart review was conducted for 54 youth (mean age = 5.0 years) transplanted between 2003 and 2014. Lab adherence (≥80%) was measured as the proportion of completed labs out of the number expected. Immunosuppressant drug-level variability was used as a proxy for medication adherence. Clinical outcomes included LAR, viral infection, hospitalization, and non-routine clinic visit ≥12 months after transplant. RESULTS: Lab adherence decreased substantially over time. Single-parent household (aOR 5.86; 95% CI: 1.38-24.93) and no history of early rejection (aOR 3.96; 95% CI: 1.04-15.24) were independently associated with non-adherence. Lab non-adherence was significantly associated with medication non-adherence (P < .05), LAR (P = .02), and non-routine clinic visits (P = .03). CONCLUSIONS: Systematic monitoring of lab adherence may help in identifying pediatric LT recipients at increased risk for excessive healthcare use and adverse outcomes possibly due to poor disease management.

Conservative Management of Pneumatosis Intestinalis and Portal Venous Gas After Pediatric Liver Transplantation.

BACKGROUND: Pneumatosis intestinalis (PI) is a rare pathologic finding in pediatric liver transplant (PLT) recipients. The presentation and course of PI can range from asymptomatic and clinically benign to life threatening, with no consensus regarding management of PI in children. We aim to review the clinical presentation and radiologic features of PLT recipients with PI and to report the results of conservative management. METHODS: A retrospective medical chart review was conducted on PLT recipients between November 1995 and May 2016. Parameters evaluated at PI diagnosis included pneumatosis location, presence of free air or portal venous gas (PVG), symptoms, laboratory findings, and medication regimen. RESULTS: PI developed in 10 of 130 PLT patients (7.7%) between 8 days and 7 years (median: 113 days) posttransplant. Five of the patients were male, and the median age was 2 years (range, 1-17 years). PI was located in 1 to 2 abdominal quadrants in 6 patients, and 3 patients had PVG. At diagnosis, all patients were on steroids and immunosuppressant medication and 6 patients had a concurrent infection. Laboratory findings were unremarkable. Symptoms were present in 7 patients. Nine patients were managed conservatively, and 1 patient received observation only. All patients had resolution of PI at a median of 7 days (range, 2-14 days). CONCLUSIONS: PI can occur at any time after PLT and appears to be associated with steroid use and infectious agents. If PI/PVG is identified and the patient is clinically stable, initiation of a standard management algorithm may help treat these patients conservatively, thus avoiding surgical intervention.

Child Attitude Toward Illness Scale (CATIS): A systematic review of the literature.

OBJECTIVE: The objective of this study was to systematically review the literature utilizing the Child Attitude Toward Illness Scale (CATIS) as a measure of illness attitudes within pediatric chronic illness, including epilepsy, and provide recommendations for its use. This review includes an examination of the psychometric properties of the CATIS and the relationship between the CATIS and psychological, academic, behavioral, and illness variables. METHOD: Electronic searches were conducted using Medline and PsychINFO to identify twenty-two relevant publications. RESULTS: The CATIS was identified as a reliable and valid self-report assessment tool across chronic illnesses, including pediatric epilepsy. Although originally developed for children ages 8-12, the CATIS has demonstrated reliability and validity in youth ages 8-22. The CATIS scores were reliably associated with cognitive appraisal variables and internalizing symptoms. Initial support exists for the relation between illness attitudes and externalizing behavior, academic functioning, and psychosocial care needs. Mixed findings were reported with regard to the relation between illness attitudes and demographic and disease variables, as well as both social and family functioning. CONCLUSION: The CATIS is a psychometrically sound self-report instrument for measuring illness attitudes and demonstrates clinical utility for examining adjustment outcomes across chronic illnesses, particularly pediatric epilepsy.

Quality of Life Changes and Health Care Charges Among Youth With Epilepsy.

OBJECTIVE: To examine differences in health care charges following a pediatric epilepsy diagnosis based on changes in health-related quality of life (HRQOL). METHODS: Billing records were obtained for 171 youth [M (SD) age = 8.9 (4.1) years] newly diagnosed with epilepsy. Differences in health care charges among HRQOL groups (stable low, declining, improving, or stable high as determined by PedsQL(™) scores at diagnosis and 12 months after diagnosis) were examined. RESULTS: Patients with persistently low or declining HRQOL incurred higher total health care charges in the year following diagnosis (g = .49, g = .81) than patients with stable high HRQOL after controlling for epilepsy etiology, seizure occurrence, and insurance type. These relationships remained consistent after excluding health care charges for behavioral medicine or neuropsychology services (g = .49, g = .80). CONCLUSIONS: Monitoring HRQOL over time may identify youth with epilepsy at particular risk for higher health care charges.

Health care charges for youth with newly diagnosed epilepsy.

OBJECTIVES: To estimate first-year health care charges for youth with newly diagnosed epilepsy seen within an interdisciplinary pediatric epilepsy team and examine demographic, clinical, and psychosocial predictors of annual charges. METHODS: Retrospective chart review was conducted to extract medical, hospital, and physician billing data from the year following an epilepsy diagnosis for 258 patients (aged 2-18 years) seen in a New Onset Seizure Clinic between July 2011 and December 2012. Descriptive statistics were used to estimate per-patient total first-year charges and health care utilization patterns (e.g., hospitalizations, emergency department visits, outpatient visits). Univariate analyses examined differences in health care charges between demographic, clinical, and psychosocial factors. Predictors of health care charges were examined using hierarchical multiple regression analysis. RESULTS: The estimated per-patient total first-year health care charge was $20,084 (95% confidence interval [CI] $16,491-$23,677). Charges were higher for patients who reported having seizures since diagnosis ($25,509; 95% CI $20,162-$30,856) and were associated with more antiepileptic drug side effects (r = 0.18; 95% CI 0.03-0.32). Controlling for demographic and clinical factors, poorer baseline health-related quality of life was associated with higher per-patient health care charges (B = -445.40; 95% CI -865 to -25). CONCLUSIONS: The economic impact of pediatric epilepsy in the year following diagnosis is substantial. Cost reduction efforts would be optimized by improving seizure control and targeting health-related quality of life, an outcome amenable to behavioral intervention.

Topical Review: Theoretical Frameworks in Pediatric Adherence-Promotion Interventions: Research Findings and Methodological Implications.

OBJECTIVE: To summarize the guiding theoretical frameworks included in pediatric adherence-promotion interventions and characterize targeted domains using the theoretical domains framework (TDF), a standardized system developed by adult behavior change researchers. METHODS: A systematic review of PubMed, PsycINFO, and CINAHL databases identified 47 articles describing pediatric adherence-promotion interventions. Data extraction was completed independently by two authors. Targeted intervention domains were classified using the TDF. RESULTS: The majority of interventions did not cite a guiding theoretical framework or cited multiple theories with overlapping domains. The TDF was a reliable categorization system and suggested that pediatric adherence-promotion interventions most commonly target knowledge, skills, and social influences. CONCLUSIONS: Pediatric adherence-promotion interventions draw from a variety of theories and lack a consistent language for describing targeted domains. The adapted TDF proposed here is one method of reducing variability in intervention development and reporting and may facilitate efforts to identify the processes that improve adherence.

Examination of parent-child adjustment in juvenile rheumatic diseases using depression-specific indices of parent and youth functioning.

Studies demonstrate a link between parental distress, youth illness appraisals, and depression symptoms in youth with juvenile rheumatic diseases. However, the exclusive use of broadband (i.e. general) measures of parental distress in these studies has resulted in conceptual and clinical imprecision regarding the parent-child adjustment process. Our aim was to reanalyze previously published data (i.e. Wagner et al., 2003) using a depression-specific scale derived from the general adult distress measure in the original study. Parents completed the Brief Symptom Inventory (BSI), youth completed the Child Depression Inventory (CDI), and the Illness Intrusiveness Scale (IIS-C). Thirteen Diagnostic and Statistical Manual of Mental Disorders (Fourth Edition) depression-specific items from the BSI comprised the parent measure of Depressive Symptoms Scale (DS). Consistent with Wagner et al. (2003), adult DS scores were associated with youth CDI scores. However, youth illness appraisals had unique effects on the parent-child depression relation. Elevated youth perceptions of illness intrusiveness amplified the negative effect of parent depressive symptoms on youth depressive symptoms; decreased illness intrusiveness buffered the negative effect of parent depression. The empirical and clinical implications of assessing parent and youth adjustment in a domain-specific manner are discussed.

An examination of the Allocation of Treatment Responsibility scale in adolescents with epilepsy.

The purpose of the current study was to examine the psychometric properties of the adapted Allocation of Treatment Responsibility (ATR) scale and the distribution of tasks related to oral medication and clinic and laboratory visits in a sample of adolescents with epilepsy. Adolescents with epilepsy (N = 50; ages 13-17 years) and their caregivers completed the adapted ATR and a measure of medication management. Internal consistency for the adapted ATR was strong (total and subscale range: 0.75-0.97). Validity was partially supported by significant correlations between adolescent age and ATR oral medication responsibility for both respondent measures. Allocation of Treatment Responsibility total scores were not associated with adherence to medications and clinic appointments. Initial findings are promising and have important implications for assessing the distribution of treatment responsibility among adolescents with epilepsy and their families.

Treatment adherence in patients with headache: a systematic review.

OBJECTIVE: To review and critically evaluate the extant research literature pertaining to adherence in youth and adults with headache and to provide recommendations for future research. BACKGROUND: This article provides the first systematic review of pediatric headache adherence and updates a previous review of treatment adherence in adults with headache. DESIGN: Systematic review of empirical literature. METHODS: A literature search with no date restriction was conducted using PubMed and PsycINFO electronic databases and bibliographies of relevant articles. RESULTS: Adherence rates in adults with headache range considerably from 25% to 94% across treatment, assessment method, and definition of adherence utilized. Methods to assess adherence included retrospective prescription claims data, paper or electronic diaries, follow-up appointment attendance, written and verbal self-report of general adherence, verbal self-report of adherence over a specific amount of time via in person interview or telephone, validated adherence measures, adherence questionnaires without validation, and counselor ratings of homework. Each methodology and assessment tool demonstrated strengths and weaknesses. No studies have systematically examined medication adherence in children with headache, and the few available studies examining adherence to behavioral treatment have documented adherence rates ranging from 52% to 86%. CONCLUSIONS: Adherence research in adults with headache is growing, but studies demonstrate a number of methodological shortcomings. Adherence research in children with headache, and adherence intervention research in both adults and children, is scant. Future research should use objective measures of adherence, consider over-the-counter medications and medication overuse, examine demographic, psychological, and behavioral correlates of adherence, assess adherence to botulinum toxin type A, and examine the efficacy of adherence interventions in individuals with headache.

The clinical utility of health-related quality of life screening in a pediatric inflammatory bowel disease clinic.

BACKGROUND: Adjusting to symptom flares, treatment regimens, and side effects places youth with inflammatory bowel disease (IBD) at increased risk for emotional and behavioral problems and adverse disease outcomes. Implementation of psychosocial screening into clinical practice remains a challenge. This study examines the clinical utility of health-related quality of life (HRQOL) screening in predicting disease outcome and healthcare utilization. METHODS: One hundred twelve youth of 7 to 18 years diagnosed with IBD and their parents. Youth completed standardized measures of HRQOL and depression. Parents completed a proxy report of HRQOL. Pediatric gastroenterologists provided the Physician Global Assessment. Families were recruited from a pediatric gastroenterology clinic. Retrospective chart reviews examined disease outcome and healthcare utilization for 12 months after baseline measurement. RESULTS: Linear regressions, controlling for demographic and disease parameters, revealed that baseline measurement of youth and parent proxy-reported HRQOL predicted the number of IBD-related hospital admissions, gastroenterology clinic visits, emergency department visits, psychology clinic visits, telephone contacts, and pain management referrals over the next 12 months. Disease outcome was not significant. CONCLUSIONS: Lower HRQOL was predictive of increased healthcare utilization among youth with IBD. Regular HRQOL screening may be the impetus to providing better case management and allocating resources based on ongoing care needs and costs. Proactive interventions focused on patients with poor HRQOL may be an efficient approach to saving on healthcare costs and resource utilization.

Caregiver demand and parent distress in juvenile rheumatic disease: the mediating effect of parent attitude toward illness.

Parents of youth with juvenile rheumatic diseases (JRD) often take on illness management responsibilities that can become burdensome, potentially resulting in poor parent adjustment outcomes. However, not all caregivers will experience increased distress as a result of variability in stress appraisals. The current study examined the role of parent illness attitudes in the relation between perceived caregiver demand and parental distress. Youth (N = 70) ages 7-18 years diagnosed with a JRD and their parents were recruited from a pediatric rheumatology clinic. Parents completed measures of caregiver demand, parental distress, and illness attitudes. Hierarchical regression revealed a relationship between caregiver demand and parental distress. A significant relationship was also found between caregiver demand and parent illness attitudes, as well as parent illness attitudes and parental distress. Thus, parent illness attitudes mediated the relationship between caregiver demand and parental distress. Techniques aimed at altering negative illness attitudes may help parents cope with their caregiving responsibilities.

Utility of the Illness Intrusiveness Scale in parents of children diagnosed with juvenile rheumatic diseases.

OBJECTIVE: To examine the factor structure and convergent validity of the Illness Intrusiveness Scale--Parent Version in mother and fathers of children and adolescents ages 7 to 18 (M = 13.56 years, SD = 2.67) diagnosed with a juvenile rheumatic disease. DESIGN: Parents of 122 children and adolescents (82 girls, 40 boys) completed the Illness Intrusiveness Scale-Parent Version, and both parents and children and adolescents completed measures of functional disability, general distress, and illness uncertainty. An exploratory factor analysis was conducted on the Illness Intrusiveness Scale--Parent Version to identify the factor structure. The factors were then compared with parent- and child-report measures of functional disability, general distress, and uncertainty. Finally, analyses were conducted to determine whether the magnitude of the correlations was significantly different between factors for parents and children and adolescents. RESULTS: The Illness Intrusiveness Scale--Parent Version was found to have a two-factor structure. The Relationships/Personal Development factor contained items related to self-fulfillment and interactions with others, and the Instrumental factor contained items related to health and work. These factors were found to have good internal consistency and were significantly correlated with measures of parent-reported functional disability and parent- and youth-reported distress and uncertainty. The magnitude of these correlations was also found to differ depending on informant and outcome measure. CONCLUSION: The Illness Intrusiveness Scale--Parent Version appears to be a valid measure for use in parents of children with juvenile rheumatic disease.

Exploration of age and sex differences in depressive symptoms and illness attitudes for youth with epilepsy and juvenile rheumatic disease.

The aim was to explore the relationship of youth age and sex to depressive symptoms and illness attitudes in youth with epilepsy and juvenile rheumatic disease. Youth with epilepsy or juvenile rheumatic disease between the ages of 7 and 19 years completed measures of depressive symptoms and illness attitudes. A significant 3-way interaction, Disease Group × Sex × Age, was found when predicting depressive symptoms. Post hoc analyses revealed a simple 2-way Disease Group × Age interaction for males and females. Younger males and adolescent females with epilepsy reported greater depressive symptoms than younger males and adolescent females with a juvenile rheumatic disease. Youth with epilepsy reported more negative illness attitudes than youth diagnosed with a juvenile rheumatic disease, but no other main effects or interactions were found. Results suggest early screening of depressive symptoms, with particular attention to younger males and adolescent females with epilepsy.

The relationship of maternal and child illness uncertainty to child depressive symptomotology: a mediational model.

OBJECTIVE: To examine the relationship of parent and child ratings of illness uncertainty to depressive symptomotology in children with a chronic illness using a mediational model framework. METHOD: Mother-child dyads (N = 103 pairs) each completed measures of perceived illness uncertainty, while youth also completed a measure of depressive symptomotology. RESULTS: Maternal uncertainty was directly related to child depressive symptoms; however, this relationship was mediated by child uncertainty. CONCLUSION: It would appear that a key mechanism by which parent-related uncertainty influences child depressive symptoms is through child uncertainty, underscoring the importance of examining cognitive appraisal variables and means of transmission in parent-child interactions.

The association of illness uncertainty to parent and youth adjustment in juvenile rheumatic diseases: effect of youth age.

OBJECTIVE: : This study sought to examine the differential effect of youth age on the association between parental uncertainty and both parent distress and youth depressive symptoms within the context of juvenile rheumatic disease. METHOD: : The sample comprised 51 youth aged 9 to 17 years diagnosed with a juvenile rheumatic disease and their parents. Youth completed a measure of depressive symptoms (i.e., Children's Depression Inventory); parents completed measures of illness uncertainty (i.e., Parental Perceptions of Uncertainty Scale) and parental distress (i.e., Brief Symptom Inventory). RESULTS: : Parental uncertainty demonstrated a significant main effect on both parent distress and youth depressive symptoms. However, these main effects were qualified by significant parental uncertainty × youth age interactions. Parental uncertainty was significantly related to parent distress for both younger and older youth but was only significantly related to youth depressive symptoms in the sample of older youth. CONCLUSION: : Parental uncertainty due to illness seems to be more predictive of both parent and youth distress with increasing youth age. These findings indicate that the impact of parental illness uncertainty on adjustment outcomes is pronounced as children transition into developmental periods of increasing autonomy and independence.

The Relationship of Allergy Severity to Depressive and Anxious Symptomatology: The Role of Attitude toward Illness.

The current study examined the relationship between self-reported allergy severity, depressive and anxious symptoms, and attitude toward illness in adolescents and young adults (AYAs) with allergies. Participants were 214 undergraduate students between the ages of 17-25 years with self-reported allergies. Participants completed the Center for Epidemiological Studies Depression Scale (CES-D), the Zung Self-Rating Anxiety Scale (SAS), and the Child Attitude Toward Illness Scale (CATIS) as measures of depressive symptoms, anxious symptoms, and attitude toward illness, respectively. Using the bootstrapping method, results revealed that attitude toward illness mediated the relationship between self-reported disease severity and depressive and anxious symptoms. Results of the current study suggest that attitude toward illness is one pathway by which subjective disease severity impacts psychological functioning in AYAs with allergies.

Parental overprotection, perceived child vulnerability, and parenting stress: a cross-illness comparison.

The current study sought to investigate differences in parenting capacity variables across four disease groups. Parents (N = 425), the majority of whom were mothers, of children with either cancer, asthma, Type 1 diabetes, or cystic fibrosis, completed measures of parental overprotection, perceived child vulnerability, and parenting stress. After controlling for significant demographic variables, parents of children with cystic fibrosis and asthma reported higher perceived child vulnerability than parents of children with either diabetes or cancer, while parents of children with asthma and diabetes reported higher parenting stress than parents of children with cancer or cystic fibrosis. No differences between disease groups were found for parental overprotection. The current study provides support for an illness-specific approach to understanding parenting capacity variables in the context of childhood chronic illnesses.