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.     

Using pediatric liver grafts (≤6 yr) for adult recipients: A considerable option?

In LT, the common policy is to allocate pediatric liver grafts to pediatric recipients. Pediatric organs are also offered to adults if there is no pediatric recipient. However, they are rarely accepted for adult recipients. So far, there is no information available reporting outcome of LT in adult recipients using pediatric livers from donors ≤6 yr. In this study, we included nine adult recipients (seven females and two males) who received grafts from children ≤6 yr from January 2008 to December 2013. We evaluated the graft quality, the GBWR and analyzed the recipients’ perioperative course. Laboratory samples and graft perfusion were analyzed. Nine adults with a median age of 49 yr (range: 25–65) and a median weight of 60 kg (range: 48–64) underwent LT with a pediatric donor graft. Median donor age was five yr (range: 3–6). Median GBWR was 1.02 (range: 0.86–1.45). After a median follow‐up of 3.9 yr (range: 11 months–6.6 yr), patient survival was 100%; graft survival was 89%. One patient needed re‐transplantation on the second postoperative day due to PNF. Eight recipients were discharged from the ICU after 2–9 days with a regular graft function. Doppler scans revealed regular flow patterns at any time. Only if denied for pediatric recipients, the use of pediatric livers from donors ≤6 yr for adult recipients is a considerable option.     

Prope tolerance after pediatric liver transplantation

pT, under mono‐ and infratherapeutic calcineurin inhibition, may constitute an optimal condition combining graft acceptance with low IS load and minimal IS‐related toxicity. We reviewed 171 pediatric (<15.0 yr) survivors beyond one yr after LT, transplanted between April 1999 and June 2007 under tacrolimus‐based regimens (median follow‐up post‐LT: 6.0 yr, range: 0.8–9.5 yr). Their current status regarding IS therapy was analyzed and correlated with initial immunoprophylaxis. pT was defined as tacrolimus monotherapy, with mean trough blood levels <4 ng/mL during the preceding year of follow‐up, combined with normal liver function tests. The 66 children transplanted before April 2001 received a standard tacrolimus–steroid regimen. Beyond April 2001, 105 patients received steroid‐free tacrolimus–basiliximab or tacrolimus–daclizumab immunoprophylaxis. In the latter group, 43 (41%) never experienced any acute rejection episode and never received steroids. In the long term, a total of 79 recipients (47%) developed pT (n = 73) or IS‐free operational tolerance (n = 6), 27 of them belonging to the 43 steroid‐free patients (63%). In contrast, only 52/128 (41%) children treated with steroids subsequently developed prope/operational tolerance (p = 0.012). Steroid‐free tacrolimus‐based IS seems to promote long‐term graft acceptance under minimal/no IS. These results constitute the first evidence that minimization of IS, including steroid avoidance, might be tolerogenic in the long term after pediatric LT.     

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.     

Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach

LT for PFIC type 1 is often complicated by postoperative diarrhea and recurrent graft steatosis. A 26‐month‐old female child with cholestatic jaundice, pruritus, diarrhea, and growth retardation revealed total bilirubin 9.1 mg/dL, gamma‐glutamyl transpeptidase 64 IU/L, and TBA 295.8 μmol/L. Genetic analysis confirmed ATP8B1 defects. A LT (segment 2, 3 graft) from the heterozygous father was performed. Biliary diversion was performed by a 35‐cm jejunum conduit between the graft hepatic duct and the mid‐transverse colon. Stools became pigmented immediately. Follow‐up at 138 days revealed resolution of jaundice and pruritus and soft‐to‐hard stools (6–8 daily). Radioisotope hepato‐biliary scintigraphy (days 26, 68, and 139) confirmed unobstructed bile drainage into the colon (t_1/2 34, 27, and 19 minutes, respectively). Contrast meal follow‐through at day 62 confirmed the absence of any colo‐jejuno‐hepatic reflux. At 140 days, contrast follow‐through via the biliary stent revealed patent jejuno‐colonic anastomosis and satisfactory transit. Graft biopsy at LT, 138 days, and 9 months follow‐up revealed comparable grades of macrovesicular steatosis (<20%). TIBD during LT may be a clinically effective stoma‐free biliary diversion and may prevent recurrent graft steatosis following LT for PFIC type 1.     

Transient elastography for non‐invasive evaluation of post‐transplant liver graft fibrosis in children

As graft survival in pediatric LT is often affected by progressive fibrosis, numerous centers carry out protocol liver biopsies. Follow‐up biopsy protocols differ from center to center, but all biopsies are progressively spaced out, as time from transplant increases. Therefore, there is a need for non‐invasive techniques to evaluate graft fibrosis progression in those children who have no clinical or serological signs of liver damage. Indirect markers, such as the APRI, should be relied on with caution because their sensitivity in predicting fibrosis can be strongly influenced by the etiology of liver disease, severity of fibrosis, and patient age. A valid alternative could be TE, a non‐invasive technique already validated in adults, which estimates the stiffness of the cylindrical volume of liver tissue, 100‐fold the size of a standard needle biopsy sample. The aims of this study were to evaluate the reliability of TE in children after LT and to compare both the TE and the APRI index results with the histological scores of fibrosis on liver biopsies. A total of 36 pediatric LT recipients were studied. All patients underwent both TE and biopsy within a year (median interval ‐0.012 months) at an interval from LT of 0.36 to 19.47 years (median 3.02 years). Fibrosis was assessed on the biopsy specimens at histology and staged according to METAVIR. There was a statistically significant correlation between TE stiffness values and METAVIR scores (P = .005). The diagnostic accuracy of TE for the diagnosis of significant fibrosis (F ≥ 2) was measured as the area under the curve (AUROC = 0.865), and it demonstrated that the method had a good diagnostic performance. APRI was not so accurate in assessing graft fibrosis when compared to METAVIR (AUROC = 0.592). A liver stiffness cutoff value of 5.6 kPa at TE was identified as the best predictor for a significant graft fibrosis (METAVIR F ≥ 2) on liver biopsy, with a 75% sensitivity, a 95.8% specificity, a 90% positive predictive value, and an 88.5% negative predictive value. These data suggest that TE may represent a non‐invasive, reliable tool for the assessment of graft fibrosis in the follow‐up of LT children, alerting the clinicians to the indication for a liver biopsy, with the aim of reducing the number of protocol liver biopsies.     

Living donor liver transplantation in Alagille syndrome—Single center experience from south Asia

To analyze the clinical characteristics and the outcomes of living donor liver transplantation in children with Alagille syndrome (AGS). Clinical data of children with AGS who underwent liver transplantation between July 2009 and May 2019 in our unit were retrospectively analyzed. Primary end‐points were patient and graft survival. Ten children with AGS underwent living donor liver transplantation at a median age of 28 months (range, 12‐84 months). Jaundice was the most common initial symptom and was noted after a median duration of 20 days after birth (range, 7‐60 days). Two patients had undergone Kasai porto‐enterostomy for misdiagnosis of biliary atresia. The most common indication for transplantation was severe pruritus with poor quality of life. Explant livers in three children showed cirrhosis with early well‐differentiated hepatocellular carcinoma. We have 100% patient and graft survival at a mean follow‐up of 32 months (range 3‐72 months). The median z‐score for weight and height at liver transplantation was ?2.66 (range: ?6.44 to ?0.9) and ?3.6 (range: ?7.96 to ?0.93) while at follow‐up was ?1.7 (range: ?3.4 to ?0.35) and ?2.1 (range: ?3.9 to ?1.4), respectively. The estimated glomerular filtration rate was normal pretransplant and follow‐up. This is the first series of LDLT for Alagille syndrome in the Indian sub‐continent. We report excellent post‐transplant outcomes in contrast to outcomes reported from Western literature.     

Late allograft fibrosis in pediatric liver transplant recipients: Assessed by histology and transient elastography

Late allograft fibrosis in LT recipients can cause graft dysfunction and may result in re‐transplantation. TE is a non‐invasive tool for the assessment of liver fibrosis. We aimed to evaluate the prevalence of allograft fibrosis in pediatric LT recipients, identify factors associated with allograft fibrosis, and determine the diagnostic value of TE, compared to histology. All children who underwent LT for ≥3 years were included. TE was performed for LSM in all patients. LSM of ≥7.5 kPa was considered as abnormal and suggestive of allograft fibrosis. Percutaneous liver biopsy was performed when patients had abnormal LSM and/or abnormal LFTs. Histological fibrosis was diagnosed when METAVIR score ≥F1 or LAF scores ≥1. TE was performed in 43 patients and 14 (32.5%) had abnormal LSM suggestive of allograft fibrosis. Histological fibrosis was identified in 10 of the 15 patients (66.7%) who underwent percutaneous liver biopsy and associated findings included chronic active HBV infection (n = 3), and late acute rejection (n = 3). Multivariate analysis showed that graft age was significantly associated with allograft fibrosis (OR = 1.22, 95% CI: 1.05‐1.41, P = 0.01). In conclusion, late allograft fibrosis is common in children undergoing LT for ≥3 years and associated with graft age. HBV infection and late acute rejection are common associated findings. Abnormal TE and/or LFTs may guide physicians to consider liver biopsy for the detection of late allograft fibrosis in LT children.     

Liver transplantation for pediatric inherited metabolic disorders: Considerations for indications,complications, and perioperative management

LT is an effective therapeutic option for a variety of IEM. This approach can significantly improve the quality of life of patients who suffer from severe disease manifestations and/or life‐threatening metabolic decompensations despite medical/dietary management. Due to the significant risks for systemic complications from surgical stressors, careful perioperative management is vital. Even after LT, some disorders require long‐term dietary restriction, medical management, and monitoring of metabolites. Successful liver transplant for these complex disorders can be achieved with disease‐ and patient‐specific strategies using a multidisciplinary approach. In this article, we review indications, complications, perioperative management, and long‐term follow‐up recommendations for IEM that are treatable with LT.     

Survival outcomes scores (SOFT,BAR, and Pedi‐SOFT) are accurate in predicting post‐liver transplant survival in adolescents

SOFT and BAR scores utilize recipient, donor, and graft factors to predict the 3‐month survival after LT in adults (≥18 years). Recently, Pedi‐SOFT score was developed to predict 3‐month survival after LT in young children (≤12 years). These scoring systems have not been studied in adolescent patients (13–17 years). We evaluated the accuracy of these scoring systems in predicting the 3‐month post‐LT survival in adolescents through a retrospective analysis of data from UNOS of patients aged 13–17 years who received LT between 03/01/2002 and 12/31/2012. Recipients of combined organ transplants, donation after cardiac death, or living donor graft were excluded. A total of 711 adolescent LT recipients were included with a mean age of 15.2±1.4 years. A total of 100 patients died post‐LT including 33 within 3 months. SOFT, BAR, and Pedi‐SOFT scores were all found to be good predictors of 3‐month post‐transplant survival outcome with areas under the ROC curve of 0.81, 0.80, and 0.81, respectively. All three scores provided good accuracy for predicting 3‐month survival post‐LT in adolescents and may help clinical decision making to optimize survival rate and organ utilization.     

The use of hepatitis B immunoglobulin with or without hepatitis B vaccine to prevent de novo hepatitis B in pediatric recipients of anti‐HBc–positive livers

Prophylactic measures are used to reduce DNHB after HBsAg‐negative patients receive anti‐HBc–positive liver grafts. This study investigated the incidence of DNHB and clinical outcomes in pediatric LT recipients under HBIG prophylaxis, with or without hepatitis B vaccination. Between 1995 and 2013, 51 HBsAg‐negative pediatric recipients underwent living‐donor LT from anti‐HBc–positive donors. The median (range) age was 4 (0.1‐17) years, 23 (45%) were male, and 71% were negative for both anti‐HBc and anti‐HBc. During a median follow‐up of 12.1 (0.06‐19.9) years, 13 (25.4%) developed DNHB; 7 of the 13 achieved HBsAg seroconversion after administration of LAM or ETV. Among studied patients, 20 (39%) received hepatitis B vaccination, and 2 of them (10%) developed DNHB. At last follow‐up, 41% (21/51) discontinued HBIG either after successful HBV vaccination (n = 17) or retransplantation with anti‐HBc–negative grafts (n = 4). In conclusion, pediatric LT recipients of anti‐HBc–positive grafts, most of them were naïve to HBV infection, were at high risk of DNHB, and consistent monitoring for the early detection of DNHB was necessary. A combination use of post‐LT vaccination is promising prophylactic strategy against DNHB.     

Pretransplant trends in α‐fetoprotein levels as a predictor of recurrence after living donor liver transplantation for unresectable hepatoblastoma: A single‐institution experience

LT is a practical therapeutic alternative for unresectable hepatoblastoma; however, deciding when to perform LT is difficult. The aim of this study was to optimize the timing of LT for hepatoblastoma using pretransplant trends in AFP levels. Trends in pretransplant AFP levels and their influence on post‐transplant outcomes were retrospectively evaluated. All patients who underwent living donor LT for hepatoblastoma in our institution since 2002 were included. Variables analyzed included history of prior tumor resection, pretransplant AFP responses to chemotherapy, metastatic disease at diagnosis, and post‐transplant chemotherapy. Eight patients (seven boys and one girl; median age, 35 months; range, 15 months‐12 years) were transplanted. The overall post‐transplant recurrence‐free survival rate was 62.5% (5/8) with a mean follow‐up of 77 months. Patients with post‐transplant recurrence showed a 0.573 log increase in AFP levels after the last chemotherapy session before LT. This was significantly higher than the 0.279 log decrease observed in patients without post‐transplant recurrence (P = .024). Because the AFP response cannot be accurately predicted before each cycle of chemotherapy, it may be appropriate to perform LT when AFP levels do not decrease after the last cycle and before they are found to be elevated again.     

Late T cell–mediated rejection may contribute to poor outcomes in adolescents and young adults with liver transplantation

Although poor long‐term graft survival in LT in AYA is recognized, detailed epidemiological data are still lacking. L‐TCMR may have poor outcomes. This study aimed to provide a detailed, epidemiological assessment of the association between AYA age and rejection. L‐TCMR was defined in this study as TCMR with central vein or perivenular inflammation occurring later than 3 months after LT. A total of 342 patients who survived for at least 3 months after LT between 2005 and 2015 were enrolled. The AYA group (10‐24 years) was compared with the C group (less than 10 years), and the incidence and outcomes of L‐TCMR were analyzed. In total, 342 patients had LT; 38 of these were AYA with the mean follow‐up period of 6.7 years. A total of 304 patients in C group had a mean follow‐up period of 6.3 years (P = .28). The incidence of L‐TCMR in AYA group was significantly higher than in C group (15.8% vs 4.6%, P = .006). The time to L‐TCMR after LT was significantly shorter in AYA group (P = .01). Neither patient survival nor the incidence of non‐adherence differed significantly between the groups (P = .18 and P = .89). The number of additional immunosuppressants after L‐TCMR was significantly higher in the AYA group (P = .04). A high incidence of L‐TCMR was observed in AYA group irrespective of non‐adherence. AYA patients with L‐TCMR should be followed carefully due to the poor results of post‐treatment biopsy and the need for intensive immunosuppressive therapy.     

Basiliximab treatment for steroid‐resistant rejection in pediatric patients following liver transplantation for acute liver failure

An IL‐2 receptor antagonist, basiliximab, decreases the frequency of ACR in liver transplant (LT) recipients as induction therapy. The aim of this study was to evaluate the effectiveness of basiliximab against SRR as rescue therapy in pediatric LT patients with ALF. Forty pediatric ALF patients underwent LT between November 2005 and July 2013. Among them, seven patients suffering from SRR were enrolled in this study. The median age at LT was 10 months (6–12 months). SRR was defined as the occurrence of refractory rejection after more than two courses of steroid pulse therapy. Basiliximab was administered to all patients. The withdrawal of steroids without deterioration of the liver function was achieved in six patients treated with basiliximab therapy without patient mortality, although one patient developed graft loss and required retransplantation for veno‐occlusive disease. The pathological examinations of liver biopsies in the patients suffering from SRR revealed severe centrilobular injuries, particularly fibrosis within one month after LT. We demonstrated the effectiveness and safety of rescue therapy consisting of basiliximab for SRR in pediatric LT recipients with ALF.     

Persistence of post‐operative color Doppler abnormalities is linked to reduced graft survival in pediatric patients after liver transplantation

Color Doppler US is a readily available imaging modality for the evaluation of liver transplants. The aim of our study was to evaluate the temporal course of color Doppler US findings in children after LTX and to investigate the effect of resolving and persisting abnormalities during follow‐up on long‐term outcome. All children who underwent LTX during January 2000 until December 2003 (155 LTX in 137 patients, 75 male and 62 female; mean age at LTX 4.1 ± 4.8 years; range, 0.1‐16.3 years) were retrospectively evaluated. Following a predefined ultrasound protocol with prospective documentation, intraoperative, post‐operative, and follow‐up examinations were evaluated for color Doppler abnormalities. The time of occurrence and temporal course of the findings were recorded. Graft survival rates and graft survival times were compared. Abnormal color Doppler US examinations were noted in 98 of 155 grafts during the entire observational period (63.2%). In 57 of 98 grafts (58.2%), abnormalities were limited to the perioperative period (<30 days after LTX). Survival of grafts with transient perioperative abnormalities was similar to transplantations with regular color Doppler US examinations (graft survival rates, 80.7% vs 84.2%, P = .622; mean graft survival time, 2596.92 vs 2511.40 days, P = .67). Grafts with persisting color Doppler US abnormalities in the follow‐up period (>30 days after LTX; 37/98 LTX, 37.8%) showed reduced survival compared with regular courses (graft survival rate 62.2% vs 80.7%, P = .047), indicating underlying organ‐specific alterations. Standardized longitudinal evaluation during the perioperative and the follow‐up period can enhance the prognostic capabilities of color Doppler US in children following LTX.     

Individualized concept for the treatment of autosomal recessive polycystic kidney disease with end‐stage renal disease

Management of children with autosomal recessive polycystic kidney disease (ARPKD) who develop end‐stage renal disease (ESRD) remains challenging because of concomitant liver disease. Patients with recurrent cholangitis are candidates for liver‐kidney transplantation, while the treatment for patients with splenomegaly and pancytopenia due to portal hypertension is controversial. Herein, we report 7 children who were treated using an individualized treatment strategy stratified by liver disease. Two patients with recurrent cholangitis underwent sequential liver‐kidney transplantation, while 4 patients with splenomegaly and pancytopenia but without recurrent cholangitis underwent splenectomy followed by isolated kidney transplantation. The remaining patient, who did not have cholangitis and pancytopenia, underwent isolated kidney transplantation. Blood cell counts were normalized after splenectomy was performed at the median age of 8.7 (range, 7.4‐11.7) years. Kidney transplantation was performed at the median age of 8.8 (range, 1.9‐14.7) years in all patients. Overwhelming post‐splenectomy infections and cholangitis did not occur during the median follow‐up period of 6.3 (range, 1.0‐13.2) years. The estimated glomerular filtration rate at the last follow‐up was 53 (range, 35‐107) mL/min/1.73 m². No graft loss occurred. Our individualized treatment strategy stratified by recurrent cholangitis and pancytopenia can be a feasible strategy for children with ARPKD who develop ESRD and warrants further evaluation.     

ABO‐incompatible pediatric liver transplantation in very small recipients: Birmingham’s experience

Gelas T, McKiernan PJ, Kelly DA, Mayer DA, Mirza DF, Sharif K. ABO‐incompatible pediatric liver transplantation in very small recipients: Birmingham’s experience.
Pediatr Transplantation 2011: 15: 706–711. © 2011 John Wiley & Sons A/S. Abstract: Liver transplantation (LT) for very small recipients is challenging but in experienced centres, good results can be achieved. Despite the risk of antibody‐mediated acute rejection, some studies have demonstrated the safety of ABO incompatible liver transplantation (ILT) in children and particularly in infants. The aim of our study was to describe the outcome of liver transplantation in infants <5 kg and the safety of using ILT in this group. All LT performed between 1991 and 2010 in children <5 kg were reviewed. Twenty‐nine patients were included, five of whom had an ILT. Acute liver failure was encountered in 20 cases. The recipient age and weight at transplantation were respectively 63 days (range: 14–268 days) and 4 kg (range: 2.4–5 kg). The graft‐to‐recipient ratio was 6.1% (range 2.3–9%). An aortic conduit and delayed abdominal closure were used respectively in 76% and 81% of the procedures. The ABO compatible liver transplantation (CLT) and ILT groups were similar regarding recipient’s demographics, graft types or technical transplantation data. The one‐ and five‐yr patient and graft survival were respectively 62%, 62% and 62%, 57.9% with a median follow‐up of 95 months. Vascular complications occurred in six cases (21.4%) and biliary complications were encountered in five patients (17%). Acute and chronic rejection developed respectively in 37% and 26% of the recipients. The five patients undergoing ILT are all alive without graft lost after a median follow‐up of 34 months (range 7–55 months). When compared with the CLT group, no significant differences were found regarding patient or graft survival, vascular or biliary complications and rejection rates. In our experience, ILT in small infants has short and long term outcomes comparable to ABO‐compatible grafts and excellent results can be achieved with a standard immunosuppressive protocol. To avoid mortality on the waiting list for neonatal recipients, ABO‐incompatible liver grafts can be used safely.     

Perioperative renal transplantation management in small children using adult‐sized living or deceased donor kidneys: A single‐center experience

Kidney transplantation remains the treatment of choice for children with ESRD. Optimal perioperative management is critical in small recipients of ASK to assure adequate graft perfusion. We present a single‐center experience outlining management for patients weighing <20 kg who underwent primary renal transplantation with ASKs between 2007 and 2016. Sixty‐three patients met study criteria and underwent 34 living‐related, six living‐unrelated, and 23 deceased donor kidney transplants. Median age and weight at transplant were 25 months (IQR 18‐37 months; range 11 months‐6 years) and 11.0 kg (IQR 9.2‐14.5 kg; range 7.1‐19.5 kg). Eighty‐nine percent of patients required vasoactive agents intra‐operatively, with twenty patients requiring prolonged vasoactive agents post‐operatively. Intra‐operatively, patients received 51.9 mL/kg of crystalloid, 27.3 mL/kg of 5% albumin, and 13.6 mL/kg of packed red blood cells. Most (93.7%) patients were extubated on POD#0. Weights peaked on post‐operative days three through five. Over a median follow‐up of 49 months (IQR 31‐86 months; range 0‐130 months), four grafts were lost, two due to thrombosis and two secondary to chronic rejection. There was one patient death six months post‐transplant due to causes unrelated to transplantation. Graft survival at 1, 5, and 10 years was 98.4%, 96.6%, and 84.2%, respectively. Of surviving allografts, the median 1, 5, and 10 years post‐transplant eGFR was 122.9, 90.0, and 59.2 mL/min/1.73 m² as determined by the 2009 Schwartz formula. Renal transplantation in small children using ASKs requires meticulous perioperative management including adequate fluid resuscitation and judicious use of pressors to assure adequate graft perfusion. The use of ASKs from living or deceased donors results in satisfactory short and long‐term outcomes.     

Efficacy of repeated balloon venoplasty for treatment of hepatic venous outflow obstruction after pediatric living‐donor liver transplantation: A single‐institution experience

HVOO is a rare complication after LT and an important cause of graft failure. Balloon venoplasty is the first‐line treatment for HVOO, but the effect of repeated balloon venoplasty and stent placement for HVOO recurrence after pediatric LDLT remains unclear. Between 1998 and 2016, 147 pediatric patients underwent LDLT in our institution. Among them, the incidence of HVOO and the therapeutic strategy were retrospectively reviewed. Ten patients were diagnosed with HVOO. All the patients underwent LLS grafts. Median age at the initial endovascular intervention was 2.7 years (range, 5 months‐8 years). The median interval between the LDLT and the initial interventional radiology was 2.7 months (range, 29 days‐35.7 months). Four patients experienced no recurrence after a single balloon venoplasty; 6 underwent balloon venoplasty more than 3 times because of HVOO recurrence; and 2 underwent stent placement due to the failure of repeated balloon venoplasty. All patients are alive with no symptoms of HVOO. The HVOO recurrence‐free period after the last intervention ranged from 20 days to 15.5 years (median, 8.9 years). Repeated balloon venoplasty may prevent unnecessary stent placement to treat recurrent HVOO after pediatric LDLT.     

Risk factors for neurological complications and their correlation with survival following pediatric liver transplantation

Despite the improved outcomes of LT, post‐operative NCs remain a significant cause of morbidity and mortality. The aim of the study was to identify the incidence of and risk factors for NCs in children who underwent LT. The medical records of pediatric patients who underwent LT at Asan Medical Center Children's Hospital between January 1994 and December 2010 were retrospectively analyzed. The onset and types of NC and pretransplant variables associated with NC were evaluated. We identified 190 children (85 boys [44.7%], 105 girls [55.3%]) of mean age 4.1 ± 4.7 yr, who underwent LT. Forty‐six NCs occurred in 41 (21.6%) patients after LT, the most common being seizures (n = 13, 28.3%) and encephalopathy (n = 10, 21.7%). Of the 46 NCs, 24 (52.2%) occurred within three months after LT. Multivariate analysis showed that primary liver disease, preoperative neurological problems, preoperatively higher serum creatinine concentration, and graft failure were significant risk factors for NCs. The survival rate was significantly lower for patients with NCs than for those without (p < 0.001). NCs after pediatric LTs were common and associated with a higher mortality rate in our study. Close monitoring and appropriate risk management may improve the long‐term outcomes of pediatric patients who undergo LT.