Search Results (6)

Background/Objectives: Biliary atresia (BA) is the most common etiology for pediatric liver transplantation (LT). However, whether a previous Kasai hepatoportoenterostomy (KP) and its timing influence the outcomes of BA patients who undergo LT remains controversial. Methods: Pediatric patients with BA who underwent LT at Beijing Friendship Hospital, Capital Medical University, between June 2013 and November 2022 were recruited. The patients were divided into non-KP, early-KP (before 90 days of life), and late-KP subgroups. The clinical data were compared among the groups. A nomogram to predict the 1-, 3-, and 5-year graft survival probabilities based on a multivariate Cox model was constructed and validated. Results: Among the 475 BA patients, the no-KP group accounted for 31.8%, the early KP for 60.4%, and the late KP for 7.8%, respectively. The incidences of LT complications were comparable among the groups. From the multivariate Cox analyses, an intensive care unit (ICU) stay and bleeding were identified as the independent risk factors for postoperative patient survival, and the LT type, graft type, vascular complications, and biliary complications were those for graft survival. A nomogram for graft survival was constructed, with a C-index of 0.82, and areas under the curves (AUCs) of 0.829, 0.824, and 0.824 for the 1-, 3-, and 5-year survival nomograms, respectively. The calibration and decision curve analysis (DCA) curves showed good discrimination ability and clinical applicability. A risk classification system was further developed, and the Kaplan–Meier curves demonstrated high discrimination between the high- and low-risk groups (p < 0.0001). Conclusions: A previous KP has no impact on patients or graft survival after LT in BA patients. The established nomogram may be helpful for counseling BA patients about their clinical prognosis after LT. Full article

Epidemiological evidence suggests that thrombophilic factors, including male sex, non-O blood type, MTHFRnt677TT mutation, factor V Leiden G1691A mutation, and prothrombin G20210A polymorphism, may contribute to the progression of fibrosis and occurrence of portal vein thrombosis in liver disease. We retrospectively investigated the effect of potentially thrombophilic factors on native liver survival as a patient-relevant endpoint of disease progression in a cohort of 142 children being followed up for biliary atresia at Hannover Medical School from April 2017 to October 2019. No significant association could be determined. There was no evidence for relevant differences in native liver survival for the Factor V Leiden G1691A mutation (hazard ratio [HR] = 0.86, 95% confidence interval [CI] 0.38–1.98, p = 0.73), prothrombin G20210A polymorphism (HR = 0.96, 95%CI 0.24–3.65, p = 0.96), non-O blood type (HR = 0.79, 95%CI 0.51–1.21, p = 0.28) or MTHFRnt677TT mutation (HR = 1.24, 95%CI 0.60–2.56, p = 0.56). A certain, albeit not strong, evidence of reduced native liver survival in male patients after Kasai hepatoportoenterostomy, particularly during the first 2000 days (42%; HR = 1.41, 95%CI 0.92–2.18, p = 0.11) was found. All children with pre-transplant portal vein thrombosis (n = 7) had non-O blood types. Larger multi-centre studies are necessary to show if the male sex or other thrombophilic factors could be potentially associated with reduced native liver survival. Full article

Balancing post-operative adequate pain control, respiratory depression, and return of bowel function can be particularly challenging in infants receiving the Kasai procedure (hepatoportoenterostomy). We performed a retrospective chart review of all patients who underwent the Kasai procedure from a single surgeon at Children’s Healthcare of Atlanta from 1 January 2018, to 1 September 2022. 12 patients received the Kasai procedure within the study period. Average weight was 4.47 kg and average age was 7.4 weeks. Most patients received multimodal pain management including dexmedetomidine and/or ketorolac along with intravenous opioids. A balance of colloid and crystalloids were used for all patients; 57% received blood products as well. All patients were extubated in the OR and transferred to the general surgical floor without complications. Return of bowel function occurred in all patients by POD2, and enteral feeds were started by POD3. One patient had a presumed opioid overdose while admitted requiring a rapid response and brief oxygen supplementation. Simultaneously optimizing pain control, respiratory safety, and bowel function is possible in infants receiving the Kasai procedure. Based on our experience and the current pediatric literature, we propose an enhanced recovery protocol to improve patient outcomes in this fragile population. Larger, prospective studies implementing an enhanced recovery protocol in the Kasai population are required for stronger evidence and recommendations. Full article

(1) Background: In patients with biliary atresia (BA) liver nodules can be identified either by pre-transplant imaging or on the explant. This study aimed to (i) analyze the histopathology of liver nodules, and (ii) to correlate histopathology with pretransplant radiological features. (2) Methods: Retrospective analysis of liver nodules in explants of BA patients transplanted in our center (2000–2021). Correlations with pretransplant radiological characteristics, patient age at liver transplantation (LT), time from Kasai hepatoportoenterostomy (KPE) to LT, age at KPE and draining KPE. (3) Results: Of the 63 BA-patients included in the analysis, 27/63 (43%) had nodules on explants. A majority were benign macroregenerative nodules. Premalignant (low-grade and high-grade dysplastic) and malignant (hepatocellular carcinoma) nodules were identified in 6/63 and 2/63 patients, respectively. On pretransplant imaging, only 13/63 (21%) patients had liver nodules, none meeting radiological criteria for malignancy. The occurrence of liver nodules correlated with patient age at LT (p < 0.001), time KPE-LT (p < 0.001) and draining KPE (p = 0.006). (4) Conclusion: In BA patients, pretransplant imaging did not correlate with the presence of liver nodules in explants. Liver nodules were frequent in explanted livers, whereby 25% of explants harboured malignant/pre-malignant nodules, emphasizing the need for careful surveillance in BA children whose clinical course may require LT. Full article

(1) Background: Acute cholangitis during the first year after Kasai hepatoportoenterostomy (HPE) has a negative impact on patient and native liver survival. There are no consistent guidelines for the definition, treatment, and prophylaxis of cholangitis after HPE. The aim of this study was to develop definition, treatment, and prophylaxis guidelines to allow for expeditious management and for standardization in reporting. (2) Methods: the Delphi method, an extensive literature review, iterative rounds of surveys, and expert panel discussions were used to establish definition, treatment, and prophylaxis guidelines for cholangitis in the first year after HPE. (3) Results: Eight elements (pooled into two groups: clinical and laboratory/imaging) were identified to define cholangitis after HPE. The final proposed definitions for suspected and confirmed cholangitis are a combination of one element, respectively, two elements from each group; furthermore, the finding of a positive blood culture was added to the definition of confirmed cholangitis. The durations for prophylaxis and treatment of suspected and confirmed cholangitis were uniformly agreed upon by the experts. (4) Conclusions: for the first time, an international consensus was found for guidelines for definition, treatment, and prophylaxis for cholangitis during the first year after Kasai HPE. Applicability will need further prospective multicentered studies. Full article

Background and objectives: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management. This study aimed to analyze the evolution and survival of patients with BA referred for HPE (Kasai operation) in our department. Materials and Methods: A retrospective analysis was performed on fourteen patients with BA, diagnosed in the pediatric department and further referred for HPE in our surgical department between 2010 and 2016. After HPE, the need for transplantation was assessed according to patients cytomegalovirus (CMV) status, and histological and biochemical analysis. Follow-up results at 1–4 years and long term survival were assessed. Results: Mean age at surgery was 70 days. Surgery in patients younger than 60 days was correlated with survival. Jaundice’s clearance rate at three months was 36%. Total and direct bilirubin values had a significant variation between patients with liver transplants and native liver (p = 0.02). CMV was positive in eight patients, half with transplant need and half with native liver survival. Smooth muscle actin (SMA) positivity was proof of advanced fibrosis. The overall survival rate was 79%, with 75% for native liver patients and an 83% survival rate for those with liver transplantation. Transplantation was performed in six patients (43%), with a mean of 10 months between HPE and transplantation. Transplanted patients had better survival. Complications were diagnosed in 63% of patients. The mean follow-up period was six years. Conclusions: HPE, even performed in advanced cirrhosis, allows a significant survival, and ensures an essential time gain for patients requiring liver transplantation. A younger age at surgery is correlated with a better outcome, despite early CMV infection. Full article