Factors predicting health‐related quality of life in pediatric liver transplant recipients in the functional outcomes group

Data from 997 pediatric LT recipients were used to model demographic and medical variables as predictors of lower levels of HRQOL. Data were collected through SPLIT FOG project. Patients were between 2 and 18 yr of age and survived LT by at least 12 months. Parents and children (age ≥ 8 yr) completed PedsQL? 4.0 Generic Core and CF Scales at one time point. Demographic and medical variables were obtained from SPLIT. HRQOL scores were categorized as “poor” based on lower 25% of scores for each measure. Logistic regression models were generated. Single‐parent households (OR 1.94, CI 1.13–3.33, p = 0.017), anti‐seizure medications (OR 3.99, CI 1.26–12.70, p = 0.019), and number of days hospitalized (OR 1.03, CI 1.01–1.06, p = 0.0067) were associated with lower self‐reported HRQOL. Parent data identified increasing age at transplant, age 5–12 yr at survey, hospitalization >21 days at LT, re‐operations, diabetes, and growth failure at LT as additional predictors of generic HRQOL. Male gender, single‐parent households, higher bilirubin levels at LT, and use of anti‐seizure medication predicted lower cognitive function scores. HRQOL following pediatric LT is related to medical and demographic variables.     

Health‐related quality of life in pediatric intestinal transplantation

To determine HRQOL after pediatric intestinal transplantation. Thirty‐four IT survivors from 1999 to 2012 were asked to complete age‐specific HRQOL non‐disease‐specific questionnaires: TAPQOL (0–4 yr), KINDL‐R (5–7 yr; 8–12 yr; 13–17 yr), and SF‐36v2 (>18 yr), all validated with Spanish population. Primary caregiver completed a SF‐36 questionnaire and CBI. Thirty‐one participants were included. Median age was 10.2 yr (1–29) and time after transplant 4.4 yr (0–13). Overall patient scores were 78.2 ± 10.6 (n = 8), 83.3 ± 9.7 (n = 6), 72.2 ± 9.21 (n = 6), 80.5 ± 12.4 (n = 7), and 82.2 ± 12.4 (n = 4) for each age group. Highest scores were obtained for vitality (group I), self‐esteem (group IV), and physical and social functioning and emotions (group V). Lowest scores were obtained in appetite and behavior (I), family and school (III), and chronic disease perception (III, IV). No significant differences were found between caregivers and their children. CBI showed stress in 52%. SF‐36 for caregivers was lower than general population. No significant differences were found depending on relevant clinical and sociodemographic data. HRQOL was acceptable and improved with age and time since transplantation. Parents had a slighter own QOL and worse perception of health than their children. When successful, intestinal transplantation allows a normal life in most patients and can be offered as an attractive option.     

Health‐related quality of life after combined liver and kidney transplantation in children

While reduced HRQOL following isolated organ transplantation has been previously reported, there are no data in the context of children following CLKT. Twenty‐three children who underwent CLKT at our institution were included in the study. The indication for CLKT was PH1 in 13 patients and ARPKD in 10 patients. Quantification of HRQOL was facilitated through the use of the PedsQL 4.0 Generic Core Scale. The results of the study were compared to healthy children and published data of children who had undergone LTx or KTx. The CLKT samples' child self‐report showed good HRQOL. No statistically significant difference was found between the patients with PH1 and patients with ARPKD (P=.4). Compared to healthy children, a significant difference in the total scale score, the physical health score, and the school functioning was reported. HRQOL did not differ significantly when compared to patients following isolated LTx or KTx. To improve HRQOL after CLKT, a focus on patients' physical health, educational performances, and overall quality of life is crucial. Thus, coordinated medical care across disciplines and psychological and social support is essential to achieve this goal.     

Tacrolimus‐related seizure after pediatric liver transplantation – A single‐center experience

To identify the risk factors for new‐onset seizures after pediatric LT and to assess their clinical implications and long‐term prognosis. The clinical and laboratory data of 27 consecutive children who underwent LT from January 2007 to December 2010 in our center were analyzed retrospectively. Patients were divided into seizures group and a non‐seizures group. Pre‐operative, intra‐operative, and post‐operative data were collected. Seizures occurred in four children, an incidence of 14.8%. All exhibited generalized tonic–clonic seizures within the first two wk after LT. Univariate analysis showed that the risk factors associated with seizures after pediatric LT included gender, pediatric end‐stage liver disease score before surgery, Child–Pugh score before surgery, serum total bilirubin after surgery, and trough TAC level. Multivariate analysis showed that trough TAC level was the only independent risk factor associated with the seizures. All children who experienced seizures survived with good graft function and remained seizure‐free without anti‐epileptic drugs over a mean follow‐up period of 33.7 ± 14.6 months. High trough TAC level was the predominant factor that contributed to seizures in the early post‐operative period after pediatric LT. High PELD and Child‐Pugh scores before LT and high post‐operative serum Tbil may be contributory risk factors for TAC‐related seizures.     

The effects of gender on health‐related quality of life in pediatric live‐donor kidney transplantation: A single‐center experience in a developing country

El‐Husseini A, Hassan R, Sobh M, Ghoneim M. The effects of gender on health‐related quality of life in pediatric live‐donor kidney transplantation: A single‐center experience in a developing country.
Pediatr Transplantation 2010:14:188–195. © 2009 John Wiley & Sons A/S. Abstract: To evaluate the effects of gender on HRQOL and overall health status in our pediatric kidney transplants. We performed a cross‐sectional study in 77 children who received living renal allo‐transplants in our center between 1981 and 2003. The patients were given a questionnaire at a post‐transplant visit. After completing, the patients returned it in a closed envelope. The questionnaire included demographic questions plus 57 multiple‐choice questions designed to analyze various aspects of post‐transplant life. Overall, the patients show satisfactory HRQOL. Most of the patients lived with their parents (79.2%). The current health status did not cause difficulties at work in 70.1% and did not interfere with the social life in 62.3% of patients. Physical and sexual growth was delayed in 48% and 85.7% of patients, respectively. A total of 67.5% of patients had normal health life or minor symptoms with normal activity. There was no significant effect of gender on HRQOL except for onset of puberty, sexual function, practicing sports, and obesity. Overall, the patients show satisfactory HRQOL. There was mild significant effect of gender on HRQOL. These findings may help health care professionals to develop gender‐specific interventions to optimize HRQOL of kidney transplants.     

Intraoperative blood loss and transfusion during primary pediatric liver transplantation: A single‐center experience

Children undergoing liver transplantation are at a significant risk for intraoperative hemorrhage and thrombotic complications, we aim to identify novel risk factors for massive intraoperative blood loss and transfusion in PLT recipients and describe its impact on graft survival and hospital LOS. We reviewed all primary PLTs performed at our institution between September 2007 and September 2016. Data are presented as n (%) or median (interquartile range). EBL was standardized by weight. Massive EBL and MT were defined as greater than the 85th percentile of the cohort. 250 transplantations were performed during the study period. 38 (15%) recipients had massive EBL, and LOS was 31.5 (15‐58) days compared to 11 (7‐21) days among those without massive EBL (P < 0.001). MT median LOS was 34 (14‐59) days compared to 11 (7‐21) days among those without MT (P = 0.001). Upon backward stepwise regression, technical variant graft, operative time, and transfusion of FFP, platelet, and/or cryoprecipitate were significant independent risk factors for massive EBL and MT, while admission from home was a protective factor. Recipient weight was a significant independent risk factor for MT alone. Massive EBL and MT were not statistically significant for overall graft survival. MT was, however, a significant risk factor for 30‐day graft loss. PLT recipients with massive EBL or MT had significantly longer LOS and increased 30‐day graft loss in patients who required MT. We identified longer operative time and technical variant graft were significant independent risk factors for massive EBL and MT, while being admitted from home was a protective factor.     

Management of late‐onset portal vein complications in pediatric living‐donor liver transplantation

The purpose of this study was to evaluate retrospectively the results of PTA for late‐onset PV complications after pediatric LDLT and to assess whether a meso‐Rex shunt is a viable option for treating restenosis of the PV after PTA in selected cases. Seventy‐five children who underwent adult‐to‐child LDLT were included in this study, and there were six late‐onset PV complications (8.0%). The initial therapeutic approach was PTA, with or without stent: PTA with balloon dilation for three children, PTA with stent placement for one child, and failure to cannulate the occluded PV for two children. A meso‐Rex shunt was performed in the two children after failed PTA: One suffered complete obstruction of the main PV, and the other, restenosis with total thrombosis after PTA with stent. The PTA was a technical and clinical success in four with PV stenosis of the six patients (66.7%), and successful application of a meso‐Rex shunt in the other two children resulted in restoration of PV flow. In conclusion, PTA is a safe and effective procedure for treating late‐onset PV stenosis after pediatric LDLT. However, in growing pediatric recipients with restenosis of the PV after PTA or chronic PV thrombosis, a meso‐Rex shunt may be a better choice for late‐onset PV complications.     

Urological complications following kidney transplantation in pediatric age: A single‐center experience

Surgical complications during kidney transplantation can seriously affect renal outcomes. We assess occurrence, risk factors, and results of all urological complications in a series of renal transplants in a single center. Children who underwent renal transplant between January 2008 and December 2014 were retrospectively evaluated. Postoperative urological complications were reviewed. Demographic details, cause of ESRD, donor type, and surgical procedures at transplant were analyzed. For statistical analysis, the chi‐square test or Fisher's exact test were used as appropriate. One hundred and twenty‐one kidney transplants were performed in 117 children (median age 12 yr). Sixty‐two of 121 (53%) had an underlying urological malformation. At a median follow‐up of three yr, 28 urological complications were recorded (23%): 12 lymphocele (10%), 10 ureteral obstruction (8%), three urinary leakage (2.5%), two symptomatic VUR (1.7%), and one hydropyonephrosis. When lymphocele was excluded, the complication incidence rate dropped to 13%. Ureteral obstruction mostly occurred late after transplant (more than six months). Presence of urological malformation was the only factor related to increased occurrence of urological complication (p = 0.007) and, in particular, ureteral obstruction (p = 0.018). Children with urological malformations presented a statistically significant risk of developing urological complications after kidney transplantation, ureteral obstruction being the most common complication.     

Outcomes of single kidney transplantation from pediatric donors: A single‐center experience

Kidneys from pDDs are increasingly used to narrow the huge gap between incremental demand and static supply. However, there is still controversy on the clinical outcome of SKT from pDDs. We conducted a retrospective cohort study of 452 adult recipients in our center between March 2012 and February 2017. Outcomes of 3 groups, transplants with organs from pDDs (n=50), aDDs (n=207), and LDs (n=195), were compared. The mean age and weight of pDDs were 8.98 years (range 8 months‐17 years) and 30.05 kg (range 8.2‐55 kg), respectively. There was no difference in 1‐year (96.0%, 98.1%, and 99.0%, respectively, P=.277) and 3‐year patient survival (96.0%, 98.1%, and 99.0%, respectively, P=.277) or in 1‐year (96.0%, 96.6%, and 98.5%, P=.307) and 3‐year (96.0%, 96.6% and 97.9%, P=.437) graft survival. SCr, eGFR, and allograft size were similar among the 3 groups at 6th month post‐transplant and thereafter. Incidence of DGF was higher in patients of the aDD group than those in the pDD group (22.7% vs 10.0%, P<.001), but there was no difference in AR and infection. SKT from pDDs to adult recipients is effective and safe with acceptable outcomes, and it will be a promising expansion to the donor pool.     

Diaphragmatic hernias after pediatric liver transplantation: Experience of a high‐volume transplant center

Diaphragmatic hernias (DHs) are rare complications after pediatric liver transplantation (PLT). It is now widely accepted that DHs after liver transplantation (LT) is a pediatric related condition. PLTs (under of age 18) performed between January 2013 and June 2019 at Malatya Inonu University Institute of Liver Transplantation were retrospectively scanned. Study group consisting DHs and a control group were compared. Among 280 PLTs, 8 of them were complicated with DHs (%2.9). Median age of the patients with DH was 3.0 (0.8‐9.5) years. Median graft recipient weight ratio was 2.5 (0.9‐4.4). Five patients were below 5th percentiles in terms of pediatric weight growth chart at the time of LT. Also, 6 patients were below 5th percentiles in terms of pediatric height growth chart. There was no statistical difference between study and control groups. There are many risk factors mentioned in literature that may be primarily responsible for DHs after PLT. These factors are left lobe and large‐for‐size grafts, malnutrition, trauma or diathermy of diaphragmatic nerve and vessels and immunosuppressants. In our study, we could not specify any reason that differs in DHs. In our aspect, narrow diaphragma and thorax are exposed to high intra‐abdominal pressure from abdomen. Large‐for‐size grafts, which are specific to children, also may contribute to this affect. Excessive diathermy and trauma to diaphragmatic collaterals may aggravate the risk of DH. More patients are needed to make an exact conclusion, in order to evaluate with comparable study on this aspect.     

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.     

Liver transplantation for urea cycle disorders in pediatric patients: A single‐center experience

LT has emerged as a surgical treatment for UCDs. We hypothesize that LT can be safely and broadly utilized in the pediatric population to effectively prevent hyperammonemic crises and potentially improve neurocognitive outcomes. To determine the long‐term outcomes of LT for UCDs, charts of children with UCD who underwent LT were retrospectively reviewed at an academic institution between July 2001 and May 2012. A total of 23 patients with UCD underwent LT at a mean age of 3.4 yr. Fifteen (65%) patients received a whole‐liver graft, seven patients (30%) received a reduced‐size graft, and one patient received a living donor graft. Mean five‐yr patient survival was 100%, and allograft survival was 96%. Mean peak blood ammonia (NH₃) at presentation was 772 μmol/L (median 500, range 178–2969, normal <30–50). After transplantation, there were no episodes of hyperammonemia. Eleven patients were diagnosed with some degree of developmental delay before transplantation, which remained stable or improved after transplantation. Patients without developmental delay before transplantation maintained their cognitive abilities at long‐term follow‐up. LT was associated with the eradication of hyperammonemia, removal of dietary restrictions, and potentially improved neurocognitive development. Long‐term follow‐up is underway to evaluate whether LT at an early age (<1 yr) will attain improved neurodevelopmental outcomes.     

Cognitive abilities,behaviour and quality of life in children after liver transplantation

Kaller T, Boeck A, Sander K, Richterich A, Burdelski M, Ganschow R, Schulz KH. Cognitive abilities, behaviour and quality of life in children after liver transplantation.
Pediatr Transplantation 2010: 14:496–503. © 2010 John Wiley & Sons A/S. Abstract: Aims: We investigated interrelations between cognitive abilities, behavioural problems, quality of life and disease‐related variables of children after LTX. Methods: Our sample consisted of 25 children. They were 8.5/2.8 (M/SD) years old and had received the transplant 5.5/3.1 years previously. For assessment we used well‐established instruments. Results: Liver transplanted children scored below the population mean on the cognitive as well as on the behavioural instrument and showed scores below average in the scales Self‐esteem, Friends and Total Score regarding QoL. Behavioural problems were associated with poorer cognitive performance (r=?0.38 to ?0.63). QoL regarding physical well‐being was correlated with sequential processing (r=0.41). Lower sequential processing scores were associated with lower QoL. Also between behavioural parameters and QoL correlations could be determined. Children with more behavioural problems experienced lower QoL (r=?0.40 to r=?0.76). Age at onset of disease showed correlations with behavioural and QoL parameters (r=?0.49 resp. r=0.44). Cognitive functioning was associated with medical complications (r=?0.44). Conclusions: High interrelations between cognitive functioning, behavioural deficits and QoL were obtained. Especially noticeable are correlations between sequential processing and internalized behavioural functions as both are associated with left lateralized brain functioning. This relationship could indicate differential effects on brain development during the preoperative phase.     

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.     

Outcomes of liver transplantation in pediatric recipients with cardiovascular disease

LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low‐risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD. CVD patients had more abnormal EKG (32.4% vs 14.5%, P < .001), abnormal chest X‐ray (11.8% vs 1.4%, P < .001), and altered echocardiography (89.7% vs 15.4%, P < .001) findings compared with the No‐CVD group pretransplant. Post‐transplant, significant differences between groups were observed related to abnormal EKG (14.7% vs 7.0%, P = .03) and echocardiography (48.5% vs 3.2%, P < .01) findings. Pretransplant ASD spontaneously closed in 22 patients. At 1 and 5 years post‐transplant, there was no difference in the survival rate between groups (P = .96). The prevalence of CVD in recipients of LT was high, and its presence was associated with significantly higher cardiac decompensation before and after LT. Minor and moderate cardiovascular disease did not impact the long‐term survival.     

Long‐term outcomes of living‐related small intestinal transplantation in children: A single‐center experience

Pediatric patients with irreversible intestinal failure present a significant challenge to meet the nutritional needs that promote growth. From 2002 to 2013, 13 living‐related small intestinal transplantations were performed in 10 children, with a median age of 18 months. Grafts included isolated living‐related intestinal transplantation (n=7), and living‐related liver and small intestine (n=6). The immunosuppression protocol consisted of induction with thymoglobulin and maintenance therapy with tacrolimus and steroids. Seven of 10 children are currently alive with a functioning graft and good quality of life. Six of the seven children who are alive have a follow‐up longer than 10 years. The average time to initiation of oral diet was 32 days (range, 13‐202 days). The median day for ileostomy takedown was 77 (range, 18‐224 days). Seven children are on an oral diet, and one of them is on supplements at night through a g‐tube. We observed an improvement in growth during the first 3 years post‐transplant and progressive weight gain throughout the first year post‐transplantation. Growth catch‐up and weight gain plateaued after these time periods. We concluded that living donor intestinal transplantation potentially offers a feasible, alternative strategy for long‐term treatment of irreversible intestinal failure in children.     

Long‐term outcomes of pediatric living donor liver transplantation at a single institution

Oh SH, Kim KM, Kim DY, Lee YJ, Rhee KW, Jang JY, Chang SH, Lee SY, Kim J‐S, Choi BH, Park S‐J, Yoon CH, Ko G‐Y, Sung K‐B, Hwang G‐S, Choi K‐T, Yu E, Song G‐W, Ha T‐Y, Moon D‐B, Ahn C‐S, Kim K‐H, Hwang S, Park K‐M, Lee Y‐J, Lee S‐G. Long‐term outcomes of pediatric living donor liver transplantation at a single institution.
Pediatr Transplantation 2010: 14:870–878. © 2010 John Wiley & Sons A/S. Abstract: There have only been a few studies on the long‐term outcomes and prognostic factors after pediatric LDLT. We conducted a retrospective, single‐center assessment of the outcomes as well as the demographic and clinical factors that influenced the poor outcomes in 113 children aged <16 (median age 21 months; 6 months–15.5 yr) who underwent 115 LDLTs, predominantly for biliary atresia (60.9%) and FHF (14.8%), between 1994 and 2006 at Asan Medical Center. Left lateral segment or left lobe grafts were implanted into most of these children (86.9%) according to routine procedures. The overall rates of graft survival at one, five, and 10 yr were 89.6%, 83.0%, and 81.5%, respectively, and the overall rates of patient survival were 92.9%, 86.3%, and 84.8%, respectively. Virus‐related disease (41.2%) and chronic rejection (29.4%) were the major causes of mortality. On multivariate analysis, UNOS status 1a and 1b and chronic rejection were significant risk factors for both graft and patient loss, whereas the PELD score >25 was a significant risk factor for graft loss. Patient and graft survival may be related not only to post‐operative complications, but also to the patient’s preoperative clinical condition.     

Fatigue in pediatric liver transplant recipients and its impact on their quality of life

The aim of the study was to investigate the occurrence of fatigue in 100 pediatric liver transplant recipients aged 2‐18 years and its impact on their health‐related quality of life (HRQL). HRQL and fatigue were measured using the PedsQL 4.0 Inventory and the PedsQL Multidimensional Fatigue Scale, which encompasses three subscales: general fatigue, sleep/rest fatigue, and cognitive fatigue. The impact of the different domains of fatigue and of clinical and sociodemographic factors on the HRQL was identified with stepwise multiple regression analyses. Parent proxy‐reports were available for all 100 participants (2‐18 years), and child self‐reports were available for 71 patients (8‐18 years). Across all domains, participants and their parents reported significantly more fatigue than healthy peers in a large PedsQL validation study. Thirty‐seven percent of patients and 57% of parents scored clinically relevant levels of fatigue. In the multiple regression analyses, none of the clinical and sociodemographic factors contributed to the HRQL for child self‐report. Only general and cognitive fatigue were significant predictors of patients' HRQL, explaining 66% of the variance in the PedsQL total score. For parent proxy‐report, general and cognitive fatigue also significantly predicted child's HRQL. Further predictors were child's age and family income. The regression model explained 65% of the variance. These findings demonstrate the importance of assessing fatigue during regular follow‐up examinations. Further research is urgently needed to better understand the underlying mechanisms of fatigue. Improvement of fatigue symptoms is essential for better HRQL, for cognitive functioning, and for school achievement.