Katie Neighbors

Cross-Sectional Analysis of Health-Related Quality of Life in Pediatric Liver Transplant Recipients

OBJECTIVE To investigate the distribution of health-related quality of life in pediatric liver transplant recipients compared with a normative population. STUDY DESIGN This cross-sectional, multicenter study was conducted at select centers. Patients between 2 and 18 years of age, surviving liver transplantation by at least 12 months, were eligible. Parent/guardian fluency in English or Spanish was required. Children > or =8 years and parents of all children completed the age-appropriate versions of the PedsQL 4.0 (Mapi Research Institute, Lyon, France). Scores were compared with a sample of healthy children (n = 3911) matched by age group, sex, and race/ethnicity and with a sample of pediatric patients with cancer receiving chemotherapy and/or radiation. RESULTS Participants included 65% (873/1339) of eligible patients. Mean age was 8.17 +/- 4.43 years, and 55% were female. The total and subscale scores of PedsQL 4.0 were lower than in healthy children (P < .001), with effect sizes for self-report ranging from -0.25 for Emotional Functioning to -0.68 for School Functioning. Patients and their parents reported better physical functioning than patients with cancer but similar social and school functioning. Correlations between parent and self-reports were in the moderate agreement range. CONCLUSIONS Pediatric liver transplant recipients and their parents report lower health-related quality of life than control subjects with some domains equal to children receiving cancer therapy.

Functional outcomes of pediatric liver transplantation.

The functional status and health-related quality of life (HRQOL) of children who survive liver transplantation (LT) have not been well documented. The purpose of this study was to determine the functional status and HRQOL in this population using a validated measure for children, the Child Health Questionnaire-Parent Form 50 (CHQ-PF50). Methods The CHQ-PF50 instrument was completed by the parents of 55 children who agreed to participate in a mailing survey. Subscale scores for the sample were compared with those of a published normal population (n = 391). Results Study sample characteristics were: 87% Caucasian, 54.5% female, mean age at survey was 9.6 years (range, 5–17 years). Responding caregivers were 95% biologic parents and 93% female. Compared with the normal population, LT recipients had lower subscale scores for general health perceptions (P < 0.0005), emotional impact on parents (<0.0005) and disruption of family activities (0.0005). The mean physical summary score of the LT recipients was lower than that of the normal population 48.1 ± 12.1 (P = 0.005), but the mean psychosocial summary score was similar 48.8 ± 11.9 (P = 0.156). Within the LT population, the original diagnosis (biliary atresia vs. other), type of LT (living donor vs. cadaveric), age at LT, z score for height, and hospital days did not significantly influence any of the subscale scores. Conclusions Children who have survived LT have functional outcomes in the physical domain that are lower than those of normal children. Self-esteem and mental health in this group appeared normal. The parents in this sample experienced more emotional stress and disruption of family activities than did parents in a normal population.

Health-Related Quality of Life and Family Function Following Pediatric Liver Transplantation

This multicenter study compared health‐related quality of life (HRQOL) and family function of pediatric liver transplant recipients to those of healthy children to determine if this population differed from a healthy population and to distinguish which pretransplant and posttransplant factors impact HRQOL and family function. HRQOL data from 102 patients achieving 2‐year survival were collected with the Infant Toddler Quality of Life Instrument or the Child Health Questionnaire Parent Form 50 parent surveys. Family functioning was assessed with the Family Assessment Device (FAD) completed by each participants family members. Demographic and clinical information were retrieved from the Studies of Pediatric Liver Transplant database. Recipients 5 years of age and older scored lower than a normative sample in physical health (P < 0.001), general health (P < 0.001), parental emotional impact (P < 0.001), and disruption of family activities (P < 0.001). Younger children, 2 to 5 years of age, scored lower than controls in global health (P = 0.004) and general health perceptions (P < 0.001) but did not differ in subscales measuring physical and psychosocial outcomes. Univariate analysis among the subscales identified demographic but not clinical variables as significant predictors of HRQOL. Mean scores of FAD scales were below published thresholds indicating healthy family functioning. As reported in previous studies, parents of older recipients reported higher levels of stress, although their level of family function appears normal. Significant associations were also observed between FAD scores and demographic variables, suggesting that further investigation of the impact of race, parental marital status, and socio‐economic status on the patient rehabilitation process is needed. Liver Transpl 14:460–468, 2008.

Adolescent Health‐Related Quality of Life Following Liver and Kidney Transplantation

Health perceptions of adolescent transplant patients should be considered in providing appropriate healthcare. Objectives: (i) quantify health‐related quality of life (HRQOL) in adolescent liver and kidney transplant patients, (ii) compare caregiver ratings of their childrens HRQOL to adolescent self‐reports and (iii) examine the relationship between HRQOL and disease‐specific disability (DSD). Adolescent liver (n = 51) and kidney (n = 26) transplant recipients and caregivers were surveyed using the CHQ‐CF87 and CHQ‐PF50. DSD scores were calculated for each patient. The response rate was >70%. Adolescents psychological and physical health was similar to a healthy population, but general health poorer (p = 0.0006). Caretakers reported lower physical functioning and general health (p = 0.0001) but similar psychological health to a normative population. All caregivers expressed negative emotional impact of their childs health on themselves and family activities (p = 0.0001). Positive correlations were found between liver transplant recipients and caregivers: perceptions of behavior (ICC = 0.55, p < 0.001), mental health (ICC = 0.56, p < 0.001), self‐esteem (ICC = 0.68, p ≤0.001). Positive correlations were found for kidney transplant patients and caregivers: physical function (ICC = 0.85, p < 0.001), bodily pain (ICC = 0.70, p < 0.001), behavior (ICC = 0.67, p < 0.01). Kidney transplant recipients showed negative correlations between physical functioning (R =−0.76, p = 0.0003) and general health (R =−0.60, p = 0.008) with overall DSD. Physical and psychological functioning of adolescent liver and kidney transplant patients is high. Caregivers may serve as adequate proxies of psychological but not physical health.

Cognitive and Academic Outcomes after Pediatric Liver Transplantation: Functional Outcomes Group (FOG) Results

This multicenter study examined prevalence of cognitive and academic delays in children following liver transplant (LT). One hundred and forty‐four patients ages 5–7 and 2 years post‐LT were recruited through the SPLIT consortium and administered the Wechsler Preschool and Primary Scale of Intelligence, 3rd Edition (WPPSI‐III), the Bracken Basic Concept Scale, Revised (BBCS‐R), and the Wide Range Achievement Test, 4th edition (WRAT‐4). Parents and teachers completed the Behavior Rating Inventory of Executive Function (BRIEF). Participants performed significantly below test norms on intelligence quotient (IQ) and achievement measures (Mean WPPSI‐III Full Scale IQ = 94.7 ± 13.5; WRAT‐4 Reading = 92.7 ± 17.2; WRAT‐4 Math = 93.1 ± 15.4; p < 0001). Twenty‐six percent of patients (14% expected) had ‘mild to moderate’ IQ delays (Full Scale IQ = 71–85) and 4% (2% expected) had ‘serious’ delays (Full Scale IQ ≤ 70; p < 0.0001). Reading and/or math scores were weaker than IQ in 25%, suggesting learning disability, compared to 7% expected by CDC statistics (p < 0.0001). Executive deficits were noted on the BRIEF, especially by teacher report (Global Executive Composite = 58; p < 0.001). Results suggest a higher prevalence of cognitive and academic delays and learning problems in pediatric LT recipients compared to the normal population.

Health-related quality of life in pediatric liver transplant recipients compared with other chronic disease groups

Limbers CA, Neighbors K, Martz K, Bucuvalas JC, Webb T, Varni JW, Alonso EM, on behalf of the Studies of Pediatric Liver Transplantation (SPLIT) Functional Outcomes Group (FOG). Health‐related quality of life in pediatric liver transplant recipients compared with other chronic disease groups.
Pediatr Transplantation 2011: 15: 245–253.

Outcomes and risk factors for failure of radiologic treatment of biliary strictures in pediatric liver transplantation recipients

Posttransplantation biliary strictures occur in 5–34% of the pediatric liver transplant patients and are conventionally managed by interventional radiological techniques. The aim of this manuscript is to assess the outcomes of patients with biliary strictures treated by percutaneous dilatation at our institution. Included in the study were 35 children with posttransplant biliary strictures that were treated with percutaneous dilatation and stenting. Initial dilation and biliary stent placement was accomplished in all patients without complications requiring surgical intervention. Recurrent strictures developed in 23 (66%) of 35 patients. The recurrence rate was 45% for anastomotic strictures, 90% for intrahepatic strictures, and 100% for those with both an anastomotic and intrahepatic component. Seven patients required revision of the choledochojejunostomy, 5 of them with a successful outcome and 2 requiring retransplant. Five patients were treated with retransplantation without surgical revision. Patients with an intrahepatic or a “combined” stricture were less likely to have a successful outcome after radiologic treatment. In conclusion, the radiological treatment of biliary strictures with balloon dilation and stenting can be performed successfully with minimal complications avoiding the need for surgical correction in many cases. Liver Transpl 12:821–826, 2006.

Health Status in Young Adults Two Decades After Pediatric Liver Transplantation

We conducted a cross‐sectional study of patients who underwent pediatric liver transplant (LT) between 1988 and 1992 to evaluate long‐term health status. Survivors completed socio‐demographic, medical and Health‐Related Quality of Life (HRQOL) surveys by mail including the SF‐36v2, PedsQL™4.0 Generic Core Scale, PedsQL™ Cognitive Functioning Scale and PedsQL™3.0 Transplant Module. SF‐36 scores were converted to SF6D‐based utilities and risk factors for lower outcomes were assessed.

Histologic characteristics of late cellular rejection, significance of centrilobular injury, and long-term outcome in pediatric liver transplant recipients.

Cellular rejection is a common event in orthotopic liver transplantation, leading to significant morbidity and mortality. Late acute cellular rejection, which occurs at least 3 months post‐transplant, affects 8–32% of pediatric liver transplant recipients. The histopathology and clinical outcome of patients affected by late cellular rejection are incompletely understood. The aims of this study were 1) to characterize late cellular rejection in the pediatric liver transplant population and describe the histopathology of late cellular rejection, with specific attention to centrilobular injury, including necrosis, inflammation, and endothelialitis and 2) to characterize the long‐term outcome of pediatric liver transplant recipients with centrilobular injury, including necrosis, inflammation, and endothelialitis. All liver biopsies performed from August 1997 to August 2002 on pediatric patients at least 6 months post‐transplant were reviewed, scored for rejection by Banff criteria and examined for centrilobular inflammatory changes. Histology was then correlated with clinical outcomes. Fifteen percent of biopsies had late cellular rejection by Banff criteria. Centrilobular inflammation, necrosis and central vein endothelialitis were common in these biopsies. In patients with similar centrilobular changes, but not meeting Banff criteria for rejection, 29% were treated for rejection and responded well clinically. The long‐term outcome of patients with isolated centrilobular injury is similar to that of patients with centrilobular changes associated with portal based rejection. In conclusion, the presence of centrilobular inflammation, necrosis, or central vein endothelialitis should prompt consideration of late cellular rejection. Liver Transpl 12:58–64, 2006.

Regional Variation and Use of Exception Letters for Cadaveric Liver Allocation in Children with Chronic Liver Disease

The Pediatric End‐Stage Liver Disease (PELD) score was designed to reduce subjectivity in liver allocation and to advantage patients with a higher probability of waiting list mortality. The aims of this study were to determine the impact of PELD implementation for children with chronic liver disease and to assess whether PELD met its goal of standardization of liver allocation for children. This study used data reported to the United Network for Organ Sharing (UNOS) registry for children with chronic liver disease receiving primary cadaveric liver transplant between January 2000 and December 2001 (pre‐PELD) and March 2002 and July 2003 (PELD). PELD reduced the percentage of children transplanted while in an intensive care unit and as status 1. A calculated PELD score was used for allocation in only 52% of recipients. Thirty percent were status 1 at transplant and PELD scores granted by exception were used for allocation in 18% of patients. There was regional variation in PELD score at allocation and use of exception scores with a significant relationship between PELD score and percentage of exception cases. Regional variation suggests that PELD has not resulted in standardization of listing practices in pediatric liver transplantation.