Good outcomes after repeated pediatric liver retransplantations: A justified procedure even in times of organ shortage.

BACKGROUND: Pediatric liver transplantations generally represent advanced surgery for selected patients. In case of acute or chronic graft failure, biliary or vessel complications, a retransplantation (reLT) can be necessary. In these situations massive adhesions, critical patient condition or lack of good vessels for anastomosis often are problematic. METHODS: Between 2008 and 2021, 208 pediatric patients received a liver transplantation at our center. Retrospectively, all cases with at least one retransplantation were identified and stored in a database. Indication, intra- and postoperative course and overall survival (OS) were analyzed. RESULTS: Altogether 31 patients (14.9%) received a reLT. In 22 cases only one reLT was done, 8 patients received 2 reLTs and 1 patient needed a fourth graft. Median age for primary transplantation, first, second and third reLT was 14 (range: 1-192 months), 60.5 (range: 1-215 months), 58.5 (range: 14-131 months) and 67 months, respectively. Although biliary atresia (42%) and acute liver failure (23%) represented the main indications for the primary liver transplantation, acute and chronic graft failure (1st reLT: 36%, 2nd reLT: 38%), hepatic artery thrombosis (1st reLT: 29%, 2nd reLT: 25%, 3rd reLT: 100%) and biliary complications (1st reLT: 26%, 2nd reLT: 37%) were the most frequent indications for reLT. OS was 81.8% for patients with 1 reLT, 87.5% with 2 reLTs and 100% with 3 reLTs. CONCLUSION: Pediatric liver retransplantation is possible with a good outcome even after multiple retransplantations in specialized centers. Nevertheless, careful patient and graft selection, as well as good preoperative conditioning, are essential.

Biological abdominal wall expansion in pediatric liver recipients after transplantation with large-for-size organs.

BACKGROUND: After pediatric split liver transplantation, intra-abdominal loss of domain due to large-for-size left lateral grafts is a frequent problem for fascial closure and potentially leads to reduced liver perfusion and abdominal compartment syndrome. Therefore, delayed fascial closure with the use of temporary silastic meshes and reoperation or alternative fascial bridging procedures are necessary. METHODS: Between March 2019 and October 2021, biologic meshes were used for abdominal wall expansion in 6 cases of pediatric split liver transplantation. These cases were analyzed retrospectively. RESULTS: One male and 5 female children with median age of 6 months (range: 0-57 months) and weight of 6 kg (range: 3.5-22 kg) received a large-for-size left lateral graft. Graft-to-recipient weight ratio (GRWR) was 4.8% (range: 1.5%-8.5%) in median. Biologic mesh implantation for abdominal wall expansion was done in median 7 days (range: 3-11 days) after transplantation when signs of abdominal compartment syndrome with portal vein thrombosis in 3 and of the liver artery in 1 case occurred. In 2 cases, bovine acellular collagen matrix and 4 cases ovine reinforced tissue matrix was used. Median follow-up was 12.5 months (range: 4-28 months) and showed good liver perfusion by sonography and normal corporal development without signs of ventral hernia. One patient died because of fulminant graft rejection and emergency re-transplantation 11 months after the initial transplantation. CONCLUSIONS: Biologic meshes can be used as safe method for abdominal wall expansion to achieve fascial closure in large-for-size liver transplant recipients. Usage for primary fascial closure can be considered in selected patients.

Predictors of portal vein complications after pediatric liver transplantation: A German center experience.

BACKGROUND: Portal vein complications (PVCs) after pediatric liver transplantation (LT) are sometimes asymptomatic, especially in the early phase, and can threaten both the graft and patient's survival. Therefore, the purpose of this study is to analyze the risk factors for portal vein thrombosis (PVT) and portal vein stenosis (PVS) after pediatric LT. METHODS: All pediatric patients (n = 115) who underwent primary LT at Regensburg University Hospital between January 2010 and April 2017 were included in this study. The pre-, intra-, and postoperative parameters of all patients were retrospectively reviewed and risk factors for both PVT and PVS were analyzed. RESULTS: Of the 115 patients, living donor LT was performed on 57 (49.5%) patients, and biliary atresia was the primary diagnosis in 65 patients (56%). After pediatric LT, 9% of patients developed PVT, and 16.5% developed PVS. Patient weight ≤7 kg [odds ratio (OR) 9.35, 95% confidence interval (CI) 1.03-84.9, p = .04] and GRWR >3% (OR 15.4, 95% CI 1.98-129.5, p = .01) were the independent risk factors for the development of PVT and PVS, respectively upon multivariate analysis. The overall patient survival rates at 1, 3, and 5 years were 91%, 90%, and 89%, respectively, and there was no difference in patient survival among those with and without PVCs. CONCLUSIONS: Pediatric patients with body weight <7 kg and/or receiving a graft with GRWR >3% may develop PVCs and so require certain surgical modifications, close follow-up, and prophylactic anticoagulant therapy following transplant.

Nutritional interventions for liver-transplanted patients.

BACKGROUND: Malnutrition is a common problem for patients waiting for orthotopic liver transplantation and a risk factor for post-transplant morbidity. The decision to initiate enteral or parenteral nutrition, to which patients and at which time, is still debated. The effects of nutritional supplements given before or after liver transplantation, or both, still remains unclear. OBJECTIVES: The aim of this review was to assess the beneficial and harmful effects of enteral and parenteral nutrition as well as oral nutritional supplements administered to patients before and after liver transplantation. SEARCH METHODS: We searched the Cochrane Hepato-Biliary Group Controlled Trials Register (March 2012), the Cochrane Central Register of Controlled Trials (Issue 2 of 12, 2012) in The Cochrane Library, MEDLINE (January 1946 to March 2012), EMBASE (January 1974 to March 2012), Science Citation Index Expanded (January 1900 to March 2012), Social Science Citation Index (January 1961 to October 2010), and reference lists of articles. Manufacturers and experts in the field have also been contacted and relevant journals and conference proceedings were handsearched (from 1997 to October 2010). SELECTION CRITERIA: Randomised clinical trials of parallel or cross-over design evaluating the beneficial or harmful effects of enteral or parenteral nutrition or oral nutritional supplements for patients before and after liver transplantation were eligible for inclusion. DATA COLLECTION AND ANALYSIS: Two authors independently assessed the risk of bias of the trials and extracted data. Dichotomous data were reported as odds ratios (OR) and continuous data as mean differences (MD) along with their corresponding 95% confidence intervals (CI). Meta-analysis was not possible due to clinical heterogeneity of included interventions. MAIN RESULTS: Thirteen trials met the inclusion criteria. Four publications did not report outcomes pre-defined in the review protocol, or other clinically relevant outcomes and additional data could not be obtained. Nine trials could provide data for the review. Most of the 13 included trials were small and at high risk of bias. Meta-analyses were not possible due to clinical heterogeneity of the interventions.No interventions that were likely to be beneficial were identified.For interventions of unknown effectiveness,postoperative enteral nutrition compared with postoperative parenteral nutrition seemed to have no beneficial or harmful effects on clinical outcomes. Parenteral nutrition containing protein, fat, carbohydrates, and branched-chain amino acids with or without alanyl-glutamine seemed to have no beneficial effect on the outcomes of one and three years survival when compared with a solution of 5% dextrose and normal saline. Enteral immunonutrition with Supportan® seemed to have no effect on occurrence of immunological rejection when compared with enteral nutrition with Fresubin®.There is weak evidence that, compared with standard dietary advice, adding a nutritional supplement to usual diet for patients during the waiting time for liver transplantation had an effect on clinical outcomes after liver transplantation. The combination of enteral nutrition plus parenteral nutrition plus glutamine-dipeptide seemed to be beneficial in reducing length of hospital stay after liver transplantation compared with standard parenteral nutrition (mean difference (MD) -12.20 days; 95% CI -20.20 to -4.00). There is weak evidence that the use of parenteral nutrition plus branched-chain amino acids had an effect on clinical outcomes compared with standard parenteral nutrition, but each was beneficial in reducing length of stay in intensive care unit compared to a standard glucose solution (MD -2.40; 95% CI -4.29 to -0.51 and MD -2.20 days; 95% CI -3.79 to -0.61). There is weak evidence that adding omega-3 fish oil to parenteral nutrition reduced the length of hospital stay after liver transplantation (mean difference -7.1 days; 95% CI -13.02 to -1.18) and the length of stay in intensive care unit after liver transplantation (MD -1.9 days; 95% CI -1.9 to -0.22).For interventions unlikely to be beneficial, there is a significant increased risk in acute rejections in malnourished patients with a history of encephalopathy and treated with the nutritional supplement Ensure® compared with usual diet only (MD 0.70 events per patient; 95% CI 0.08 to 1.32). AUTHORS' CONCLUSIONS: We were unable to identify nutritional interventions for liver transplanted patients that seemed to offer convincing benefits. Further randomised clinical trials with low risk of bias and powerful sample sizes are needed.

[Play therapy in hospital]. / Spieltherapie im Krankenhaus.

The following article presents an overview of current research studies on play therapy in the hospital. It highlights individual diagnoses for which play therapy has shown reasonable success. The aim of this review is to describe the current status of the scientific debate on play therapy for sick children in order to allow conclusions regarding the indications for which play therapy is or might be useful.

Pulse Wave Velocity for Risk Stratification of Patients with Aortic Aneurysm.

Background: Patients with an aortic aneurysm are at high cardiovascular risk. Pulse wave velocity (PWV) is used as a parameter for risk stratification but may be affected by aortic disease (AoD). This study aimed to investigate the dependence of PWV on treated or untreated AoD and to identify modifiable factors of PWV. Methods: The measurement of PWV with the Mobil-O-Graph was performed fully automatically in a collective of 381 patients (75.6% male and 24.4% female). Of all patients, 53.8% had nonaortic atherosclerotic vascular disease (AVD), 28.9% had treated AoD, and 17.3% had untreated AoD. Results: There was a statistically significant effect of age (R2 = 0.838) and current systolic blood pressure (SBP) on PWV (page corrected < 0.05). After correction for age, no statistically significant difference was found between the PWV of men and women, patients with different body weights or degrees of chronic kidney disease, diabetics and nondiabetics, and smokers and nonsmokers. Comparison between patients with nonaortic AVD and treated or untreated AoD revealed no statistically significant differences (PWVnonaortic AVD 10.0 ± 1.8 m/s, PWVtreated AoD 10.0 ± 1.5 m/s, PWVuntreated AoD 9.8 ± 1.6 m/s; page corrected > 0.05). Conclusions: PWV determined with the Mobil-O-Graph correlated with age and current SBP. Neither aortic disease versus nonaortic AVD, its treatment, nor other cardiovascular risk factors had a significant effect on PWV. Successful blood pressure control is crucial to avoid high PWV and thus an increase in cardiovascular events.

Phenotypic spectrum and diagnostic pitfalls of ABCB4 deficiency depending on age of onset.

Genetic variants in the adenosine triphosphate-binding cassette subfamily B member 4 (ABCB4) gene, which encodes hepatocanalicular phosphatidylcholine floppase, can lead to different phenotypes, such as progressive familial intrahepatic cholestasis (PFIC) type 3, low phospholipid-associated cholelithiasis, and intrahepatic cholestasis of pregnancy. The aim of this multicenter project was to collect information on onset and progression of this entity in different age groups and to assess the relevance of this disease for the differential diagnosis of chronic liver disease. Clinical and laboratory data of 38 patients (17 males, 21 females, from 29 families) with homozygous or (compound) heterozygous ABCB4 mutations were retrospectively collected. For further analysis, patients were grouped according to the age at clinical diagnosis of ABCB4-associated liver disease into younger age (<18 years) or adult age (≥18 years). All 26 patients diagnosed in childhood presented with pruritus (median age 1 year). Hepatomegaly and splenomegaly were present in 85% and 96% of these patients, respectively, followed by jaundice (62%) and portal hypertension (69%). Initial symptoms preceded diagnosis by 1 year, and 13 patients received a liver transplant (median age 6.9 years). Of note, 9 patients were misdiagnosed as biliary atresia, Alagille syndrome, or PFIC type 1. In the 12 patients with diagnosis in adulthood, the clinical phenotype was generally less severe, including intrahepatic cholestasis of pregnancy, low phospholipid-associated cholelithiasis, or (non)cirrhotic PFIC3. Conclusion: ABCB4 deficiency with onset in younger patients caused a more severe PFIC type 3 phenotype with the need for liver transplantation in half the children. Patients with milder phenotypes are often not diagnosed before adulthood. One third of the children with PFIC type 3 were initially misdiagnosed, indicating the need for better diagnostic tools and medical education. (Hepatology Communications 2018;2:504-514).