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Objective: Unusual clinical course Background: Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise ursodeoxycholic acid (UDCA), partial external biliary diversion (PEBD), and liver transplantation (LTx). The role and timing of LTx in PFIC-1 remains debated. We present 2 case reports of male siblings with PFIC-1 who benefited from different treatments. Case Report: Both siblings harbored a homozygous truncating mutation in ATP8B1 characteristic for PFIC-1 and both underwent PEBD after unsuccessful UDCA treatment at the age of 7 and 5 months, respectively. The older brother, after initial improvement of symptoms, developed severe pruritus, cholestasis, and diarrhea 9 months after PEBD and underwent LTx at the age of 16 months. Chronic diarrhea and abnormal transaminases activity appeared soon after transplantation. A liver biopsy was performed 3 months after LTx and showed severe macrovesicular steatosis (95%). Sixteen months after LTx, total biliary diversion was performed, with rapid relief from diarrhea and significant regression of graft steatosis by <30%. In his brother we observed persistent severe pruritus and cholestasis after PEBD, but we decided to postpone LTx due to lack of a living related donor and risk of graft steatosis. Eight months after PEBD, bilirubin and bile acids significantly decreased and pruritus disappeared completely. Currently, in 5-year follow-up, liver function is stable and he has no pruritus. Conclusions: The good effect of PEBD may be delayed in PFIC-1, even in severe mutation; thus, the decision to perform LTx should be made cautiously. Total biliary diversion is an efficient procedure in case of persistent symptoms after LTx and can reverse graft steatosis in children with PFIC-1. [ABSTRACT FROM AUTHOR]

Background: Calcineurin inhibitors (CNI) may increase the risk of cardiovascular (CV) events. This prospective study aimed to determine cardiovascular risk factors in pediatric patients after living related liver transplantation (LRLTx) 12 months after the conversion from cyclosporine (CS) to tacrolimus (TAC)., Material/methods: The study group consisted of 7 children (5 females and 2 males) after LRLTx performed at the median age of 3 years (range 0.8-7.2), who received CS monotherapy for at least 5 years before it was switched to TAC. The median age at conversion was years 13.1 years (range 10.1-18). Weight BMI Z-score, 24-h ABPM (ambulatory blood pressure monitoring), renal function assessment, and fasting lipid and oxidative stress profiles were performed before and 12 months after conversion., Results: Within 1-year follow-up, TAC was well tolerated and we did not observe any drug-related adverse effects or severe infections. Renal function, blood pressure, and lipid parameters did not differ after the conversion. Before the conversion, there was lower median glutathione (GSH) levels (748 vs. 776 [µmol/l]) and glutathione peroxidase (GPx) activity (31.4 vs. 32.4 [U/gHb]), but statistical significance was not reached (p>0.05). Asymmetric dimethylarginine (ADMA) levels were higher before conversion to TAC (0.93 vs. 0.69 [µmol/l], p=0.01), as were oxidized LDL (oxyLDL) levels (317 vs. 264 [mU/ml], p=0.04)., Conclusions: There was no significant difference between CS and TAC in risk factors for CV events. Potential benefits in oxidative stress profile resulting from CS to TAC conversion may add another important area for further research.

Background: Amoxicillin/clavulanic acid-induced liver injury is a well recognized complication. Presentation and outcome may vary, which is related to individual liver maturity, genetic predisposition, enzyme heterogeneity, intensity of treatment, and drug interactions. In most cases withdrawing the drug is sufficient treatment; however, cases of progressive liver damage leading to liver transplantation have been reported., Case Report: We present the case of an 8-year-old patient after liver transplantation who developed drug induced liver injury (DILI) after amoxicillin/clavulanic acid treatment for upper respiratory tract infection. Jaundice appeared 2 days after cessation of treatment. Clinical presentation and liver biopsy were consistent with DILI. Because of rapidly increasing bilirubin levels, we used 3 boluses of methylprednisolone and ursodeoxycholic acid. The treatment reversed progression of the cholestasis and full recovery was achieved in 3 months., Conclusions: In most cases of DILI, withdrawing the toxic drug is sufficient treatment, but we must be aware of a possible fatal outcome in case of progressive cholestasis. Corticosteroids may have beneficial effects in these patients.,

Background: Children with biliary atresia and polysplenia syndrome (BA-PS) have always been considered as high risk liver transplant recipients due to technical problems during transplant surgery. We report single-center experience with liver transplantation in children with this syndrome., Material/methods: Between 2000 and 2010, 401 liver transplantations were performed in 358 children, including 6 patients with BA-PS, who underwent living (5 patients) or deceased (1 patient) donor liver transplantation. Patients demonstrated various malformations: absence of retrohepatic vena cava (3), intestinal malrotation (3), preduodenal portal vein (1), hepatic artery anomalies (3), cardiac anomalies (2), and situs inversus (1). Transplantations were performed at the patient age of 8 months to 11 years., Results: There were no serious technical problems during the operations, and we did not have to use vascular conduits for graft revascularization in any case. All patents were alive at follow-up between 14 and 123 months after transplantation (mean 75 months). We observed, however, increased incidence of PV thrombosis and biliary complications in these patients, which did not influence patient and graft survival. In 1 child with graft failure due to chronic rejection after discontinuation of immunosuppression due to PTLD, retransplantation was performed., Conclusions: Results of liver transplantation in children with BA-PS are as good as for other indications and non-syndromic BA in an experienced pediatric liver transplant center. Although there were no serious technical problems during deceased or living related donor transplantation in these children, close observation for possible vascular complications should be the routine in the postoperative period.

Liver transplant recipients are at increased risk of oxidative stress due to pre-existing liver failure, ischemia-reperfusion injury, functional impairment of graft, rejections and immunosuppression. Despite the use of several methods of measurement, it is still unclear how oxidative stress correlates with tissue injury and clinical complications. In this review we discuss pathogenesis, methods of measurement, possible complications and antioxidant treatment in liver transplant recipients.

Background: Aim of the study was to analyze the effect of living related liver transplantation on selected parameters of bone formation and resorption in children with liver cirrhosis caused by biliary atresia., Material/methods: 20 children (13F/7M) with biliary atresia aged from 6 month to 2.4 years were enrolled into the study 4-9 days before liver transplantation. Osteocalcin, procollagen 1 aminoterminal propeptide, collagen type 1 crosslinked C-telopeptide, parathyroid hormone and metabolites of vitamin D: 25(OH)D3, 1,25(OH)2D3 were measured before, 3, 6 and 12 months after liver transplantation., Results: Three months after living related liver transplantation statistically significant increase of osteocalcin, collagen type 1 crosslinked C-telopeptide, parathyroid hormone and 1,25(OH)2D3 levels were found. We didn't observe further increase of these parameters during the next 9 months after liver transplantation. There was no difference in 25(OH)D3 levels in patients before and after liver transplantation., Conclusions: In children after successful living related liver transplantation we observed improvement of selected parameters of bone formation and resorption which indicate stimulation of growing processes and mechanisms of bone geometry modelling.