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BACKGROUND: The management of complex, intra- and extrahepatic portal vein thrombosis (PVT) after liver transplantation (LT) is challenging. Although most of the patients remain asymptomatic or oligosymptomatic in the chronic setting, some of them may develop severe portal hypertension and related complications, notably gastrointestinal (GI) bleeding. In the emergency scenario, clinical and endoscopic treatments as well as intensive support constitute the bases of conservative management, while more definitive treatment options such as surgical shunting and retransplantation are related to high morbidity rates. Transjugular intrahepatic portosystemic shunt (TIPS) was largely considered of limited role due to technical difficulties arising from extensive PVT. Recently, however, new minimally invasive image-guided techniques emerged, allowing portal vein recanalization and TIPS creation simultaneously (TIPS-PVR), even in complex PVT pretransplant patients. METHODS: Herein, we describe a novel indication for TIPS-PVR in a post-LT adolescent presenting with life-threatening, refractory GI bleeding. RESULTS: The patient presented with complete resolution of the hemorrhagic condition after the procedure, with no deterioration of hepatic function or hepatic encephalopathy. Follow-up Doppler ultrasound after TIPS-PVR showed normal hepatopetal venous flow within the stents, and no evidence of complications, including intraperitoneal or peri splenic bleeding. CONCLUSIONS: This report describes the feasibility of TIPS-PVR in the post-LT scenario complicated by extensive PVT. In this case, a complete resolution of the life-threatening GI bleeding was achieved, with no major complications. Other patients with complex chronic PVT might benefit from the use of the described technique, but further studies are required to determine the correct timing and indications of the procedure, eventually before the occurrence of life-threatening complications.

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BACKGROUND: The techniques involved in neonatal and infantile transplantation require approaches that can sculpt a left lateral segment (LLS) to the right shape and size and avoid large-for-size syndrome. The aim of this article is to describe the anterior hepatic resection (AHR) of the LLS in pediatric LDLT. METHODS: A retrospective anatomical study of preoperative image studies, description of the technique for AHR, and short-term results. RESULTS: The AHR was performed in eight cases. All donors were male, with average age, BW, and BMI of 28.3 ± 5.9 years, 74.2 ± 9.3 kg, and 24.3 ± 2.6 kg/m2, respectively. Donors were discharged at an average of 3.6 ± 0.8 days. The median recipient age and BW at transplantation were 6.9 (2.7 to 11) months and 5.9 (3.9 to 8) kg, respectively, and the recipient-to-donor body weight ratio (RDBW) was <0.1 in all but one case. The mean percentage reduction in graft weight and in the antero-posterior diameter were 33.2% ± 5.5% and 38.3% ± 12.6%, respectively. The average (SD) GRWR was 4.8% ± 1.7% before all the resections and 3.5% ± 1.0% after the procedures. Seven patients were primarily closed. CONCLUSION: After LLS resection, a nonanatomical anterior resection of the LLS was accomplished without hilar vascular dissection to segments II/III. The final liver graft allowed primary abdominal wall closure in all but one patient, with meaningful adjustments in GRWR. AHR proved to be simple, safe, reproducible, and effective in the presented case series.

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BACKGROUND: Living donor liver transplantation is a safe alternative for patients on a liver transplant list. Donor evaluation goes beyond physical variables to include social, emotional, and ethical aspects. The role of pre-donation sociopsychological evaluation of the donor candidate is as important to the success of the procedure as is the medical assessment. Success implies recovery from the operation and prompt engagement in pre-transplant professional and social activities, without leading to psychological or physical distress. Psychological profiling of potential living liver donors (PLLD) and evaluation of quality of life (QOL) can influence outcomes. AIM: To evaluate the socio-demographics and psychological aspects (QOL, depression, and anxiety) of PLLD for pediatric liver transplantation in a cohort of 250 patients. METHODS: This was a retrospective cohort study of 250 PLLD who underwent psychological pre-donation evaluation between 2015 and 2019. All the recipients were children. The Beck anxiety inventory, Beck depression inventory, and 36-item short-form health survey (SF-36) scores were used to evaluate anxiety (Beck anxiety inventory), depression (Beck depression inventory), and QOL, respectively. RESULTS: A total of 250 PLLD were evaluated. Most of them were women (54.4%), and the mean age was 29.2 ± 7.2 years. A total of 120 (48.8%) PLLD were employed at the time of evaluation for donation; however, most had low income (57% earned < 2 times the minimum wage). A total of 110 patients (44%) did not finish the donation process, and 247 PLLD answered a questionnaire to evaluate depression, anxiety, and QOL (SF-36). Prevalence of depression was of 5.2% and anxiety 3.6%. Although most of the PLLD were optimistic regarding the donation process and never had doubts about becoming a donor, some traces of ambivalence were observed: 46% of the respondents said they would feel relieved if a deceased donor became available. CONCLUSION: PLLD had a low prevalence of anxiety and depression. The foundation for effective and satisfactory results can be found in the pre-transplantation process, during which evaluations must follow rigorous criteria to mitigate potential harm in the future. Pre-donation psychological evaluation plays a predictive role in post-donation emotional responses and mental health issues. The impact of such findings on the donation process and outcomes needs to be further investigated.

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Background: The COVID-19 infection has received the attention of the scientific community due to its respiratory manifestations and association with evolution to severe acute respiratory syndrome (SARS-CoV-2). There are few studies characterizing SARS-CoV-2 in pediatric immunocompromised patients, such as liver transplanted patients. The aim of this study was to analyze the outcomes of the largest cohort of pediatric liver transplant recipients (PLTR) from a single center in Brazil who were infected with COVID-19 during the pandemic. Methods: Cross-sectional study. Primary outcomes: COVID-19 severity. The Cox regression method was used to determine independent predictors associated with the outcomes. Patients were divided into two groups according to the severity of COVID-19 disease: moderate−severe COVID and asymptomatic−mild COVID. Results: Patients categorized as having moderate−severe COVID-19 were younger (12.6 months vs. 82.1 months, p = 0.03), had a higher prevalence of transplantation from a deceased donor (50% vs. 4.3%, p = 0.02), and had a higher prevalence of COVID infection within 6 months after liver transplantation (LT) (75% vs. 5.7%, p = 0.002). The independent predictor of COVID-19 severity identified in the multivariate analysis was COVID-19 infection <6 months after LT (HR = 0.001, 95% CI = 0.001−0.67, p = 0.03). Conclusion: The time interval of less than 6 months between COVID-19 infection and LT was the only predictor of disease severity in pediatric patients who underwent liver transplantation.

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METHODS: We present a series of three patients with large hepatocellular adenoma lesions showing a central location, for which the living donor liver transplantation strategy was used as a backup procedure. RESULTS: Hepatocellular adenoma was confirmed by biopsy in all patients. Surgical resection was indicated because of the patients' symptoms and lesion size and growth. All patients had a lesion that was central or in close contact with major vessels. The final decision to proceed with the resection was made intraoperatively. A live donor was prepared for all three patients. Two patients underwent portal vein embolization associated with extended hepatectomy, and a total hepatectomy plus liver transplantation with a living donor was performed in one patient. All patients had good postoperative outcomes. CONCLUSIONS: In the treatment of hepatocellular adenomas for which complex resections are necessary and resectability can only be confirmed intraoperatively, surgical safety can be improved through the use of a living donor backup. Center expertise with living donor liver transplantation is paramount for the success of this approach.

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BACKGROUND: Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor. METHODS: This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT. RESULTS: During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation. CONCLUSION: Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.

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BACKGROUND: The impact of the COVID pandemic on liver transplant (LT) programs varied among countries. Few data are available about that impact in pediatric liver transplant (PLT) programs. This study aimed at comparing the data of our program in Brazil (2019 vs. 2020). METHODS: Retrospective cohort study. RESULTS: One hundred and seventy-four PLT were performed in the period (93% living donors). Patients were divided into two groups according to the LT date: pre-COVID-19 period (march/2019-February/2020) and COVID-19 period (March/2020-February 2021). In the pre-COVID-19 period, 97 LTs were performed, and 77 LTs were performed in the COVID-19 period. Patients in the COVID-19 period were younger (10.9 months vs. 16 months, p 0.009), had higher PELD scores (15 vs. 14, p 0.04), more ascites (66.2 vs. 51.5%, p 0.03), and more frequently hospitalized before LT (27.3 vs. 17.5%). However, there was no difference in post-LT complications, retransplantation nor survival rates. Six (6.2%) patients from pre-COVID-19 period were COVID positive at a median of 15.5 months (14-17.5), and 6 (7.8%) patients from COVID-19 period were COVID positive at a median of 3 months (20 days-6 months) from LT. There was neither mortality nor complications in those patients. Four (33%) were hospitalized, and one had prolonged intubation. Four (33%) were asymptomatic, 4 (33%) had upper airways symptoms, and the remaining had gastrointestinal symptoms. CONCLUSION: Overall, PLT was not affected during COVID-19 period. Even though patients from COVID-19 period were sicker, there was no significant impact in LT outcomes. All the recipients who tested positive for COVID had a favorable outcome.

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BACKGROUND: The impact of perioperative blood transfusion on short- and long-term outcomes in pediatric living donor liver transplantation (PLDLT) must still be ascertained, mainly among young children. Clinical and surgical postoperative complications related to perioperative blood transfusion are well described up to three months after adult liver transplantation. AIM: To determine whether transfusion is associated with early and late postoperative complications and mortality in small patients undergoing PLDLT. METHODS: We evaluated the effects of perioperative transfusion on postoperative complications in recipients up to 20 kg of body weight, submitted to PLDLT. A total of 240 patients were retrospectively allocated into two groups according to postoperative complications: Minor complications (n = 109) and major complications (n = 131). Multiple logistic regression analysis identified the volume of perioperative packed red blood cells (RBC) transfusion as the only independent risk factor for major postoperative complications. The receiver operating characteristic curve was drawn to identify the optimal volume of the perioperative RBC transfusion related to the presence of major postoperative complications, defining a cutoff point of 27.5 mL/kg. Subsequently, patients were reallocated to a low-volume transfusion group (LTr; n = 103, RBC ≤ 27.5 mL/kg) and a high-volume transfusion group (HTr; n = 137, RBC > 27.5 mL/kg) so that the outcome could be analyzed. RESULTS: High-volume transfusion was associated with an increased number of major complications and mortality during hospitalization up to a 10-year follow-up period. During a short-term period, the HTr showed an increase in major infectious, cardiovascular, respiratory, and bleeding complications, with a decrease in rejection complications compared to the LTr. Over a long-term period, the HTr showed an increase in major infectious, cardiovascular, respiratory, and minor neoplastic complications, with a decrease in rejection complications. Additionally, Cox hazard regression found that high-volume RBC transfusion increased the mortality risk by 3.031-fold compared to low-volume transfusion. The Kaplan-Meier survival curves of the studied groups were compared using log-rank tests and the analysis showed significantly decreased graft survival, but with no impact in patient survival related to major complications. On the other hand, there was a significant decrease in both graft and patient survival, with high-volume RBC transfusion. CONCLUSION: Transfusion of RBC volume higher than 27.5 mL/kg during the perioperative period is associated with a significant increase in short- and long-term postoperative morbidity and mortality after PLDLT.

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BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2  < 50 mm Hg and those with PaO2  ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.

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BACKGROUND: Maple syrup urine disease (MSUD) is an autosomal recessive inherited metabolic disease caused by deficient activity of the branched-chain α-keto acid dehydrogenase (BCKD) enzymatic complex. BCKD is a mitochondrial complex encoded by BCKDHA, BCKDHB, DBT, and DLD genes. MSUD is predominantly caused by Variants in BCKDHA, BCKDHB, and DBT genes encoding the E1α, E1ß, and E2 subunits of BCKD complex, respectively. The aim of this study was to characterize the genetic basis of MSUD by identifying the point variants in BCKDHA, BCKDHB, and DBT genes in a cohort of Brazilian MSUD patients and to describe their phenotypic heterogeneity. It is a descriptive cross-sectional study with 21 MSUD patients involving molecular genotyping by Sanger sequencing. RESULTS: Eight new variants predicted as pathogenic were found between 30 variants (damaging and non-damaging) identified in the 21 patients analyzed: one in the BCKDHA gene (p.Tyr120Ter); five in the BCKDHB gene (p.Gly131Val, p.Glu146Glnfs * 13, p.Phe149Cysfs * 9, p.Cys207Phe, and p.Lys211Asn); and two in the DBT gene (p.Glu148Ter and p.Glu417Val). Seventeen pathogenic variants were previously described and five variants showed no pathogenicity according to in silico analysis. CONCLUSION: Given that most of the patients received late diagnoses, the study results do not allow us to state that the molecular features of MSUD variant phenotypes are predictive of clinical severity.

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