Kidney and liver transplantation in children with fibrocystic liver–kidney disease: Data from the US Scientific Registry of Transplant Recipients: 1990–2010

The natural history and survival of children with fibrocystic liver–kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US children with fibrocystic liver–kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver–kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re‐transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty‐nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.     

New insights into family functioning and quality of life after pediatric liver transplantation

Denny B, Beyerle K, Kienhuis M, Cora A, Gavidia‐Payne S, Hardikar W. New insights into family functioning and quality of life after pediatric liver transplantation. Abstract: Thorough research of the medical aspects of pediatric liver transplantation has given way to recent interest in the impact of the transplantation process on the QOL of recipients and their families. In this cross‐sectional study, we compared the family functioning and QOL of children (n = 30) aged between three and 16 yr (M = 10.10, s.d. = 3.62) who had received a liver transplant in the previous 1–12 yr (M = 5.31, s.d. = 3.44) with non‐transplant children (n = 33), as reported via parent proxy. Results showed that parents of pediatric liver transplant recipients made significantly more adjustments to family routines to accommodate their children, particularly in relation to childcare. Impaired family functioning was also found to be associated with decreased QOL. These preliminary findings of relative deficits in family functioning may inform psychosocial interventions to assist pediatric liver transplant patients and their families. Further investigation beyond a single‐center study incorporating subjective information from pediatric patients and their parents is recommended.     

Impact of liver transplantation on HRQOL in children less than 5 years old

Our primary goal was to assess health related quality of life (HRQOL) at transplantation and 1 yr after transplantation in pediatric liver transplant patients aged less than 5 years. We conducted a prospective longitudinal study of HRQOL in pediatric liver transplant recipients, aged less than 5 years to define the impact of liver transplantation on HRQOL and identify factors that predict HRQOL after transplantation. The infant toddler health status questionnaire (ITHQ) was completed at the time of listing for liver transplantation and at 6 and 12 months after liver transplantation. The primary outcome measures were the subscale scores that comprise ITHQ. The mean age (+/-s.e.m.) of the enrolled patients (n = 45) at transplantation was 1.4 (+/-1.2) yr. Thirty-eight (84%) of the enrolled patients completed the study. The highest mean baseline scores of 78.6 (+/-3.3) were for global mental health (GlobalMH). ITHQ subscale scores increased steadily after transplantation. The greatest increase was in the first 6 months after transplant. At 1 yr after transplantation, there were significant increases in all of the ITHQ subscale scores except for GlobalMH. ITHQ subscales were similar for patients who received LDLT compared with those who received cadaver donor liver transplantation (CDLT) at baseline and a year after transplant. Time elapsed as transplantation was a significant predictor of functional health in all of the models generated. Scores for general health (GH), global health (GGH), parental time-impact (PT) and parental time-emotion (PE) were higher for male children. Family cohesion (FC) improved with time elapsed since transplant and increased number of inpatient days. HRQOL improves after transplantation in all of our patients irrespective of the donor type. Functional health scores were higher in patients with normal serum bilirubin at 1 yr post-transplant. Assessment of HRQOL should be an integral part of care for liver transplant patients and their caregivers.     

Physical activity experiences in children post–liver transplant: Developing a foundation for rehabilitation interventions

Physical Activity (PA) plays an important role in the physical and psychosocial health of children and is beneficial in the treatment and prevention of comorbidities associated with transplantation. Despite this, PA participation in pediatric liver transplant recipients remains low compared to healthy peers. This qualitative‐focused mixed‐methods study explored the PA experiences and parental perception of these experiences, including perceived facilitators and barriers to PA in children post–liver transplant. Eighteen participants (9 children [median age 10.8 years] and 9 parents) took part in semi‐structured interviews and completed the PedsQL Multidimensional Fatigue Scale and PAQ. Most children reported they were physically active (PAQ median 3.08 [IQR] 2.60‐3.51), participating in PA for its enjoyment, regardless of their level of motor proficiency. Levels of fatigue (median 65.28 [IQR] 56.25‐90.97) were higher than healthy norms and impacted PA participation in some children. Children and parents perceived PA as central to post‐transplant recovery and valued its social and mental health benefits; however, parents struggled with ongoing uncertainty and perceived physical vulnerability of their child. This study indicates the need for continuing PA support and education and provides valuable information for family‐centered interventions to increase PA and improve health outcomes in children post‐transplant.     

Quality of life and metrics of achievement in long‐term adult survivors of pediatric heart transplant

Many children who undergo heart transplantation will survive into adulthood. We sought to examine the QOL and capacity for achievement in long‐term adult survivors of pediatric heart transplantation. Adults >18 yr of age who received transplants as children (≤18 yr old) and had survived for at least 10 yr post‐transplant completed two self‐report questionnaires: (i) Ferrans & Powers QLI, in which life satisfaction is reported as an overall score and in four subscale domains and is then indexed from 0 (very dissatisfied) to 1 (very satisfied); and (ii) a “Metrics of Life Achievement” questionnaire regarding income, education, relationships, housing status, and access to health care. A total of 20 subjects completed the survey. The overall mean QLI score was 0.77 ± 0.16. Subjects were most satisfied in the family domain (0.84 ± 0.21) and least satisfied in the psychological/spiritual domain (0.7 ± 0.28). Satisfaction in the domains of health/functioning and socioeconomic were intermediate at 0.78 and 0.76, respectively. Most respondents had graduated from high school, reported a median annual income >$50 000/yr, and lived independently. Adult survivors of pediatric heart transplant report a good QOL and demonstrate the ability to obtain an education, work, and live independently.     

Single‐center experience in pediatric renal transplantation using thymoglobulin induction and steroid minimization

Our center has offered thymoglobulin induction with steroid minimization to our pediatric renal transplant patients for the last 10 yr. Steroid minimization or avoidance has shown favorable results in survival, kidney function, and growth in previous studies of pediatric patients. We report our experience with this protocol over the past 10 yr with respect to patient/graft survival, acute rejection episodes, renal function, linear growth, bone density, cardiovascular risk factors, and opportunistic infections. A retrospective chart review was performed for pediatric renal transplant patients on the steroid‐minimized protocol between January 2002 and December 2011 on an intention to treat basis. Patient demographics, height, weight, serum creatinine, iGFR, biopsies, and survival data were collected. Height and weight z‐scores were calculated with EpiInfo 7, using the CDC 2000 growth charts. Survival was calculated using Kaplan–Meier analysis. eGFR was calculated using the original and modified Schwartz equations. Forty‐four pediatric patients were identified, aged 13 months to 19 yr. Five‐yr survival was 95.5% for males and 94.4% for females. Only five patients had biopsy‐proven ACR, two of which were at more than 12 months post‐transplantation. Height delta z‐scores from transplant to one, three, and five yr were 0.34, 0.38, and 0.79, respectively. Weight delta z‐scores from transplant to one, three, and five yr were 0.87, 0.79, and 0.84, respectively. Mean original Schwartz eGFR was 84.3 ± 15.8 mL/min/1.73 m², modified Schwartz eGFR was 59.3 ± 11.5 mL/min/1.73 m², and iGFR was 64.2 ± 8.5 mL/min/1.73 m² at three yr. Of 18 subjects who had a bone density exam, none had a z‐score less than ?2 on DEXA exam at one‐yr post‐transplantation. Fifty‐one percent of patients were on antihypertensives at the time of transplant compared with 43% at one‐yr post‐transplantation. Three yr post‐transplantation, the average LDL was <100 mg/dL, and average total cholesterol was <200 mg/dL. There were no clinical episodes of EBV or CMV infection. A steroid‐minimized protocol with thymoglobulin induction is safe and provides favorable improvement in linear growth, stable graft function, stable or improved cardiovascular risk factors, and normal bone density in pediatric renal transplant patients.     

A longitudinal retrospective analysis of left ventricular mass in a cohort of pediatric kidney transplant recipients

Childhood end‐stage kidney disease is associated with increased risk for early adulthood cardiovascular (CV) morbidity and mortality. Increased LVM is an early indicator of CV disease. Previous studies have suggested that LVM decreases after kidney transplantation; however, trends have been inconsistent. A single center retrospective longitudinal cohort analysis of LVM, documented annually, starting before kidney transplantation for up to 10 yr after transplantation was performed. BP documented by annual 24‐h ambulatory monitoring studies, and BMI values were also reviewed. Twenty‐seven children followed for a mean period of 5.3 yr were included. Depending on definition of LVH, its prevalence pretransplant and in the first years post‐transplant was up to 33% dropping to 0–25% thereafter. Individual longitudinal LVM z‐score trends were highly variable but generally trended toward the mean immediately after transplant and toward negative values in the following years. BP was stable during the follow‐up period while mean annual BMI increased in the first‐year post‐transplant but declined thereafter. In a cohort of pediatric renal transplant recipients, prevalence of LVH decreased after transplant; however, individual longitudinal LVM trends were highly variable among patients. Prospective studies are needed to correlate individual LVM trends with outcomes.     

Parental functioning improves the developmental quotient of pediatric liver transplant recipients

Psychomotor development in pediatric liver transplant (LT) recipients depends on several factors. Our aim was to evaluate the importance of parental involvement and family dynamics on psychomotor development by assessing (i) children and parents individually, (ii) the parent–child relationship, and (iii) the correlation between parental functioning and patient outcome, all before and after LT. Age‐appropriate scales were used before and after LT. Twenty‐one patients, 19 mothers, and 16 fathers were evaluated. Developmental quotient (DQ): No subjects scored in the “very good” range. The proportion of children with deficits increased from LT to two yr: 17.6% vs. 28.6%. Subjects 0–2 yr were more likely to have normal DQ at transplant (66.7% vs. 50% for older children). Abnormal DQ was more prevalent two yr post‐LT in children older at LT (p = 0.02). The mother–child relationship was normal in 59% of families pre‐LT and in 67% at two yr. The relationship was more favorable when the child received a transplant as an infant (p = 0.014 at 12 months post‐LT). Normal DQ was associated with higher maternal global functioning score pre‐LT (p = 0.03). Paternal performance scores were higher than maternal scores. Children transplanted after two yr of age suffer greater long‐term deficits than those transplanted as infants.     

Parent–Child Agreement of Child Health-Related Quality-of-Life in Maltreated Children

This article examines the differences between self-reports and parent-proxy reports of pediatric health-related quality of life among families receiving child welfare services for child physical abuse and neglect. This study assesses child well-being using a pediatric health-related quality of life measure (Pediatric Quality of Life Inventory; PedsQL 4.0) with parent-child dyads (N?=?129). Child and parent reports are compared for total and domain score on the PedsQL. Child-reported scores are lower than parent-proxy reports on total and all domain scores. For the total score, 57 % of child reports are below the clinical cutoff for poor well-being compared with 19 % of parent proxy reports. Analyses indicate poor agreement between parent and child reports, with this disagreement associated with high parent anger and parental self-report of poor mental health. Fully assessing child health and well-being requires multiple perspectives of child well-being. Gaining information from both the child and the parent provides different but equally useful information.     

Size‐reduced lung transplantation in children – an option worth to consider!

Benden C, Inci I, Weder W, Boehler A. Size‐reduced lung transplantation in children – an option worth to consider!
Pediatr Transplantation 2010: 14:529–533. © 2009 John Wiley & Sons A/S. Abstract: Lung transplantation is an accepted therapy for pediatric end‐stage lung disease. However, there is a shortage of suitable donor organs. Therefore, the use of downsized lung allografts seems a valuable option. We report our experience of downsized pediatric lung transplantation in comparison with standard full‐size pediatric lung transplantation over one decade. Pediatric recipients undergoing downsized or standard lung transplantation were included (January 1997–December 2006). We compared pretransplant clinical data and surgical and post‐operative complications and post‐transplant outcome. Ten pediatric lung transplants were performed (median patient age 15.6 yr [12.3–17.8]). Nine of 10 patients had CF. Five patients underwent standard full‐size lung transplantation; five had downsized lung transplants. “Downsized” recipients had significantly lower median height and weight Z‐scores. Donor/recipient length difference was significantly greater in the “Downsized” Group (p < 0.05). All patients had comparable post‐transplant functional outcome without additional surgical complications or morbidities in “downsized” recipients. Median post‐transplant survival was 65 months (5–77) in the “Standard” Group compared to 86 months (64–121) in the “Downsized” Group (p = 0.1). Our data suggest that downsized lung transplantation in pediatric recipients may have post‐transplant outcomes comparable to full‐size lung transplantation without significant complications.     

Effect of upper limb botulinum toxin-A therapy on health-related quality of life in children with hemiplegic cerebral palsy

Aim:   Currently, the use of upper limb botulinum toxin-A (UL BTX-A) is based on evidence of functional efficacy without supporting evidence of positive change in health-related quality of life (HRQOL). While function may improve, this cannot be directly correlated with an improvement in HRQOL. Most paediatric studies use caregiver/parent proxy reports. The inclusion of child self-reports is increasing as poor correlation with proxy reports is being demonstrated. This paper aims to study the effect of UL BTX-A therapy on HRQOL in children with hemiplegic cerebral palsy (CP).
Method:   Design: Pilot prospective randomised trial. Participants: 22 children with hemiplegic CP aged 7 years 0 month−13 years 11 months (12 treatment, 10 control). Treatment: One series BTX-A injections into UL. HRQOL assessed at baseline, and 1, 3 and 6 months post-injection by completion of Pediatric Quality of Life (PedsQL) 4.0 Generic Core Scales and PedsQL 3.0 CP Module. Outcome: 1. Change in PedsQL scores. 2. Concordance between child self-report and parent proxy-report scores.
Results:   No statistically significant difference between treatment and control groups was observed for any domain of HRQOL. Intraclass concordance was good for the PedsQL CP Module Daily Activities, and Speech and Communication scores ( P  = 0.0005).
Conclusion:   This pilot work adds to the emerging evidence that UL BTX-A therapy has no statistically significant effect on the HRQOL of children with hemiplegic CP. With the increasing use of this therapy in children with CP, further research across the broader CP population is needed to identify whether this therapy is indicated in other target populations. Both child and parent proxy reports should be collected when assessing HRQOL in this population.     

The reliability of the Health Related Quality Of Life questionnaire PedsQL 3.0 Diabetes Module™ for Swedish children with type 1 diabetes

Aim: The overall aim of the study was to assess reliability and accomplish a limited validation of the Pediatric Quality of Life Inventory 3.0 Diabetes Module Scales (PedsQL 3.0), Swedish version in a sample of Swedish children diagnosed with Type 1 diabetes (T1DM). A secondary aim was to assess whether the children’s Health Related Quality of Life (HRQOL) was associated with children’s gender and age and whether the child self‐ and parent proxy reports were consistent. Methods: One hundred and thirty families from four diabetes centres participated in this study. The Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL 4.0) and the PedsQL 3.0 were administered to 108 children (aged 5–18 years) with T1DM and 130 parents (of children with T1DM aged 2–18 years). Results: The internal consistency of the PedsQL 3.0, Swedish version, reached or exceeded Cronbach’s alpha values of 0.70 for both child self‐ and proxy reports‐ and parent proxy‐reports. The PedsQL 4.0 and PedsQL 3.0 were highly correlated (r = 0.76), indicating convergent validity. The parents reported lower diabetes‐specific HRQOL than the children themselves (p < 0.01). The girls in the study reported lower psychological functioning and treatment adherence compared with the boys (p < 0.05). The oldest children (between 13 and 18 years of age) reported significantly lower diabetes‐specific HRQOL, as compared with younger children (p < 0.05). Conclusions: PedsQL 3.0 Diabetes Module can be used as a valuable tool for measuring diabetes‐specific HRQOL in child populations, both in research and in clinical practice.     

The PedsQL Brain Tumor Module: initial reliability and validity

BACKGROUND: Brain tumors (BT) are second only to acute lymphoblastic leukemia as the most prevalent form of pediatric cancer, with BT 5-year survival rates approaching 70%. With increased survival, quality of life has emerged as an essential health outcome. This investigation examines the internal consistency reliability and construct validity of the Pediatric Quality of Life Inventory (PedsQL) Brain Tumor Module. METHODS: The PedsQL 4.0 Generic Core Scales, PedsQL Multidimensional Fatigue Scale, and PedsQL Brain Tumor Module were administered to 99 families. The average age of the 56 boys and 43 girls was 9.76 years (range=2-18 years). The sample included children with tumors located in the posterior fossa/brainstem (N=62, 62.6%), supratentorial (N=15, 15.2%), and midline (N=22, 22.2%). Children were on treatment (N=46, 46.5%), off treatment<12 months (N=19, 19.2%), or off treatment>12 months/long-term survivor (N=34, 34.3%). Treatment included radiation (N=61, 61.6%), surgery (N=83, 83.8%), chemotherapy (N=87, 87.9%), and bone marrow transplant (N=5, 5.1%). RESULTS: Internal consistency reliability was demonstrated for the 24-item PedsQL Brain Tumor Module (average alpha=0.78-0.92, parent proxy-report, n=99; average alpha=0.76-0.87, child self-report, n=51). Construct validity for the PedsQL Brain Tumor Module was supported through an analysis of the intercorrelations with the Generic Core Scales and Fatigue Scale. CONCLUSIONS: The findings provide support for the measurement properties of the PedsQL Brain Tumor Module.     

Pediatric health-related quality of life after intestinal transplantation

As outcomes after ITx improve, greater emphasis is needed on HRQOL. The primary aims of this study were to (i) assess the feasibility of measuring HRQOL in pediatric ITx recipients, (ii) measure HRQOL using validated instruments, and (iii) compare HRQOL in ITx recipients to healthy normal (NL) children. The CHQ and Pediatric Quality of Life (PedsQL4.0) instruments were administered to both patients and parents at outpatient visits. All 24 eligible patients were enrolled. The median age at study enrollment was 6.0 yr (range: 2-18 yr), and the median time from transplant to study enrollment was 2.8 yr (range: 0.5-11.8 yr). The majority of subjects were male (58%), Latino (58%), and liver-inclusive (92%) recipients. For CHQ and PedsQL4.0, parental responses were significantly lower in multiple categories including physical health and social functioning compared to healthy norms. Patient responses were not different from NL using CHQ but using PedsQL4.0 were significantly lower in the school functioning subcategory and psychosocial health summary score. HRQOL as reported by children and families after ITx is significantly lower in multiple categories compared to NL.     

Prevalence and subtypes of BK virus in pediatric renal transplant recipients in Russia

BKV reactivation is associated with impaired graft function in kidney transplant patients. The objective of our study was to determine the prevalence of BKV infection in consecutive pediatric kidney transplant recipients at our center. Fifty-eight pediatric kidney transplant recipients were studied. The mean age at screening was 9.4 ± 2.8 yr, and samples were obtained at a median of 2.4 ± 1.4 yr after transplantation. BKV-DNA was analyzed in urine and plasma by quantitative PCR. Occurrences of BK-DNAuria and BK-DNAemia did not change in the first two yr after transplantation in children and amounted to 21-23% and 7-8%, respectively (p > 0.05). In the third year, the occurrences of BK-DNAuria and BK-DNAemia increased insignificantly to 27% and 9% in the pediatric patients. We also determined the subtypes and subgroups of BK virus isolated from Russian renal transplant recipients and found that BKV isolates were composed of subtypes Ib-2 and IV/c2. The data we obtained indicate that although only 5% of BKVAN cases occurred between years two and five post-transplantation, it seems necessary to regularly monitor pediatric patients for BKV infection through the third year after transplantation.     

“Inverted” positioning of renal allograft during kidney transplantation in children and adolescents: A single‐institution comparative analysis

Renal transplantation is the treatment of choice in children with end‐stage renal failure. Limitations in patient anatomy or a short donor renal vein may necessitate intraoperative inversion of the kidney. There is little evidence to support the use of this surgical technique, and no evidence in the pediatric population. This study identifies the perioperative and post‐operative outcomes of inverted renal transplants in pediatric patients. We reviewed all patients having a renal transplant between January 2012 and December 2016 and collected short‐ and long‐term outcomes of patients who received an inverted allograft. Early graft function was defined as the time to reach creatinine nadir. During this time, our hospital performed 81 transplants, and 50 (62%) were from deceased donors, including the 6 (12%) patients who received inverted renal grafts. Half (3/6) were female, 5/6 (83%) were dialysis‐dependent, and the median age at surgery was 13 years (range 9‐16 years). There was no significant difference in mean creatinine nadir values (P = 0.518) and the time to creatinine nadir mean values (P = 0.190) between the upright and inverted renal transplant groups. There were also no significant differences in rates of post‐operative complications between the upright and inverted allograft recipients. Inversion of renal allografts in pediatric patients is a viable surgical technique to compensate for shortcomings in patient anatomy or in special cases of renal transplantation involving a short donor renal vein. Future research should focus on outcomes of a larger group of pediatric inverted renal transplant patients.     

An institutional experience of pre‐emptive liver transplantation for pediatric primary hyperoxaluria type 1

Primary hyperoxaluria type 1 (PH1) is an inherited metabolic disease that culminates in ESRF. Pre‐emptive liver transplantation (pLTx) treats the metabolic defect and avoids the need for kidney transplantation (KTx). An institutional experience of pediatric PH1 LTx is reported and compared to the literature. Between 2004 and 2015, eight children underwent pLTx for PH1. Three underwent pLTx with a median GFR of 40 (30–46) mL/min/1.73 m² and five underwent sequential combined liver‐kidney transplantation (cLKTx); all were on RRT at the time of cLKTx. In one case of pLTx, KTx was required eight and a half yr later. pLTx was performed in older (median 8 vs. 2 yr) and larger children (median 27 vs. 7.75 kg) that had a milder PH1 phenotype. In pediatric PH1, pLTx, ideally, should be performed before renal and extrarenal systemic oxalosis complications have occurred, and pLTx can be used “early” or “late.” Early is when renal function is preserved with the aim to avoid renal replacement. However, in late (GFR < 30 mL/min/1.73 m²), the aim is to stabilize renal function and delay the need for KTx. Ultimately, transplant strategy depends on PH1 phenotype, disease stage, child size, and organ availability.     

De novo development of antibodies to kidney‐associated self‐antigens angiotensin II receptor type I,collagen IV,and fibronectin occurs at early time points after kidney transplantation in children

Chronic rejection is the leading cause of graft loss following pediatric kidney transplantation. Our group and others have demonstrated an association between the development of Abs to self‐antigens and chronic rejection following adult lung and heart transplantation. The goal of this study was to determine whether Abs to kidney‐associated self‐antigens develop following pediatric renal transplantation. We investigated post‐transplant development of Abs to kidney‐associated self‐antigens angiotensin II receptor type I, Fn, and collagen IV in a pediatric cohort. Using ELISA, we measured Abs to kidney‐associated self‐antigens in serum. Our cohort included 29 subjects with samples collected pretransplant and for 12 months post‐transplant. No samples had Abs to kidney‐associated self‐antigen pretransplant. In contrast, 50% (10/20) of subjects developed Abs to one or more kidney‐associated self‐antigen post‐transplantation. The median time to antibody appearance and duration of persistence were 103 and 61 days, respectively. Development of Abs did not correlate with graft function. Half of subjects developed Abs to kidney‐associated self‐antigens angiotensin II receptor type I, Fn, or collagen IV in the first year after kidney transplantation—a higher rate of early antibody development than expected. In this small study, Abs did not correlate with worse clinical outcomes.     

Quality of life in children with spinal cord injury.

PURPOSE: To compare reports of the child's quality of life (QOL) between children with spinal cord injury (SCI) and their parents using the Pediatric Quality of Life 4.0 Generic Scales (PedsQL), and assess agreement between parent and child responses. To examine the influence of level of injury on QOL and internal consistency reliability of the PedsQL in pediatric SCI. METHODS: Twenty-eight children (17 male children and 11 female children) between five and 13 years and their parents completed the PedsQL. RESULTS: Children rated their QOL better than their parents; however, there was good to excellent parent-child agreement. No differences were noted between children with tetraplegia and paraplegia. Low internal consistency reliability was obtained for various domains. CONCLUSIONS: In addition to using summary scores, specific ratings may raise important points for clinical decision-making. Results on internal consistency reliability suggest the need for condition-specific questionnaires for children with SCI.     

Post-transplantation lymphoproliferative disorder in pediatric kidney-transplant recipients - A national study

Cleper R, Ben Shalom E, Landau D, Weissman I, Krause I, Konen O, Rahamimov R, Mor E, Bar‐Nathan N, Frishberg Y, Davidovits M. Post‐transplantation lymphoproliferative disorder in pediatric kidney‐transplant recipients – A national study. Abstract: PTLD is the most common malignancy in pediatric kidney‐transplant recipients. We examined the prevalence, clinical features, and outcome of PTLD in Israel. Twelve (4.4%) of 272 pediatric (<19 yr) kidney‐transplant recipients retrieved from a search of the NIKTR for 1991–2008 had acquired PTLD at a median of 3.2 yr post‐transplantation. PTLD‐affected patients were younger at transplantation (4.2 vs. 12.5 yr, p = 0.02), had a higher rate of OKT3 therapy for acute rejection (25% vs. 4%, p = 0.015), and 5/12 were EBV‐seropositive at transplantation. Graft dysfunction was the presenting sign in six (50%). PTLD was predominantly abdominal (83%) and B‐cell type (67%); T‐cell PTLD occurred exclusively in EBV‐seropositive patients. Treatment consisted of immunosuppression cessation (6/12, 50%), antiviral agents (7/12, 58%), anti‐CD20 monoclonal antibodies (4/12, 33%), and chemotherapy (6/12, 50%). Survival was 100% in the EBV‐naïve patients and 40% in the EBV‐seropositive patients. Graft loss occurred in three of eight survivors (37.5%). PTLD‐associated mortality risk was older age: 11.2 vs. 3.4 yr, longer dialysis: 15 vs. 6.5 months, T‐cell type disease (75%), later PTLD onset: 6.35 vs. 1.9 yr post‐transplantation and era of transplantation (43% mortality before vs. 20% after 2001). Pretransplantation EBV‐seronegative status might confer a survival benefit with early detected PTLD. EBV‐seropositive patients are at risk for aggressive late‐onset lethal PTLD.