Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia

Nightingale, S. L.; Stormon, Michael; O’Loughlin, Edward; Shun, Albert; Thomas, Gordon; Benchimol, Eric I; Day, Andrew S.; Adams, Susan; Shi, Enyi; Ooi, Chee Y.; Kamath, Binita M.; Fecteau, Annie; Langer, Jacob C.; Roberts, Eve A.; Ling, Simon C.; Ng, Vicky L.

doi:10.1097/mpg.0000000000001289

Cited by 48 publications

(43 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Some elements have already been low albumin levels, -and a rapid increase in the INR. 6,[35][36][37][38][39][40] The results of the comparison of three strategies (groups S1, S2, and S3; Figure 3), the fact that the two deaths that occurred after listing of the patients were in patients listed for PMD, and the results of the series (no death in any patient candidate for LRD) suggest that having access to LRD as an option is crucial for the children candidate for transplantation. This allowed clearly the patients of the S2 group, who were deteriorating rapidly on the waiting list, to opt for an alternative and get transplanted in time.…”

Section: Discussionmentioning

confidence: 99%

“…In order to do so, it will be necessary to define a set of criteria that will allow for identifying that condition. Some elements have already been suggested as possible criteria, either at the diagnosis or at the Kasai operation, such as —the severity of hepatic fibrosis at the biopsy, —the anatomical type of atresia (type 3, absence of cyst), —the presence of polysplenia syndrome, —severe portal vein hypoplasia and portal flow inversion, —BA in the preterm, as well as within the first 3 months after surgery, — an early development of —ascites, —high bilirubin and low albumin levels, —and a rapid increase in the INR …”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

A paradigm shift in the intention‐to‐transplant children with biliary atresia: Outcomes of 101 cases and a review of the literature

Goyet¹,

Grimaldi

Tuzzolino

et al. 2019

Pediatric Transplantation

View full text Add to dashboard Cite

For children with BA who do not benefit from Kasai surgery, the only therapeutic option is liver replacement and transplantation. The very decision to proceed for transplantation is a crucial point in time because it is the first step toward the preparation for the transplantation. The former time point is defined in this analysis as “intent‐to‐transplant” care pathway. In the life of every BA candidate for liver replacement, this point in time varies and mostly depends on the decision of their primary caring teams—about when to switch from supportive care to transplant, and thus to refer to a transplant center. This intent‐to‐transplant analysis of a series of 101 consecutive infants that were referred to a single transplant team showed that excellent overall outcome (97% survival) has been achieved overall. However, three deaths occurred that were clearly related to a late referral. This analysis and recent observations from other centers strongly support that the timing for referring these children to a transplant center and/or deciding to list them on the waiting list is currently too late and should be anticipated to what it is currently. This paradigm shift in the intention‐to‐transplant children is likely necessary for giving a better chance to an increased number of children and impacts positively on the general outcome. Networking and defining new tools for a rapid recognition of the infants who need early transplantation are necessary; centralization of these children may be helpful to achieve better outcomes than currently observed.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A paradigm shift in the intention‐to‐transplant children with biliary atresia: Outcomes of 101 cases and a review of the literature

Goyet¹,

Grimaldi

Tuzzolino

et al. 2019

Pediatric Transplantation

View full text Add to dashboard Cite

show abstract

“…Thus, a dosage of TB <2 mg/dL between the third and sixth month of the postoperative period may be a good predictor of better results after portoenterostomy. Furthermore, the presence of biliary flow after the Kasai procedure has been described as an important variable associated with NLS (7,29,39) .…”

Section: Discussionmentioning

confidence: 99%

Multivariate Analysis of Biliary Flow-Related Factors and Post-Kasai Survival in Biliary Atresia Patients

Ferreira

Queiroz

Vidigal

et al. 2019

Arq. Gastroenterol.

View full text Add to dashboard Cite

BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

show abstract

“…The diagnosis of BA was confirmed by intraoperative cholangiography and a histopathologic assessment of the biliary remnant. For this study, COJ was defined as plasma total bilirubin < 34 μmol/L (2 mg/dL) [15][16][17] while living with the native liver after PEostomy. Follow-up ended at the time of last follow-up liver biopsy, when survival status of the native liver was recorded.…”

Section: Patients and Study Designmentioning

confidence: 99%

“…As long-term fibrosis of the native liver has rarely been studied, no reliable means exist to predict in which patients liver fibrosis progresses, remains stable, or even resolves after clearance of jaundice (COJ), although noninvasive methods may be useful in estimating the current stage of fibrosis in the liver. 12,13 Although overall survival of the native liver after PEostomy depends strongly on the postoperative decrease in serum bilirubin to < 34 μmol/L (2 mg/dL), [14][15][16] it remains unclear how different patient-and treatment-related factors predict progression of liver fibrosis among patients who reach normal serum bilirubin levels after PEostomy. Identifying early predictors of progressive liver fibrosis would facilitate targeting intensive postoperative monitoring to children at the greatest risk for cirrhosis and its complications.…”

Section: Introductionmentioning

confidence: 99%

Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis

Hukkinen

Kerola

Jahnukainen

et al. 2019

Surgery

View full text Add to dashboard Cite

Background: Progression of fibrosis and ensuing complications determine the postoperative course of patients operated on for biliary atresia. We evaluated predictors of the progression of fibrosis in the native liver after operative treatment. Methods: Among patients whose bilirubin decreased to < 34 μmol/L after portoenterostomy (n = 41), predictors of follow-up cirrhosis and the progression of fibrosis were analyzed with logistic regression and survival of their native liver was evaluated with the Kaplan-Meier method. Areas under receiving operating characteristic were used to define optimal cutoffs. Results: After median follow-up of 5.2 years (interquartile range 1.6-10.2) after portoenterostomy, liver biopsies showed cirrhosis in 53% of patients, and the Metavir stage remained stable or decreased in 38%. The development of cirrhosis was predicted by total or conjugated bilirubin ≥170/120 μmol/L at the time of portoenterostomy (P ≤ .009); normalization of bilirubin within 1.9 months (P = .002); total or conjugated bilirubin ≥ 12.5/7.5 μmol/L (P = .002) and aspartate aminotransferase-to-platelet ratio ≥ 0.55 at 3 months postoperatively (P = .001); and total or conjugated bilirubin ≥ 7.5/2.5 μmol/L (P ≤ .001), aspartate aminotransferase-to-platelet ratio ≥ 0.63 (P = .004), and gamma glutamyl transferase ≥ 266 U/L (P = .007) at 6 months postoperatively. In multiple regression analysis, conjugated bilirubin ≥ 2.5 μmol/L at 6 months increased the risk of cirrhosis 35-fold (P = .020), and other predictors were not predictive. Total or conjugated bilirubin < 12.5/7.5 μmol/L (P ≤ .014), aspartate aminotransferase-to-platelet ratio < 0.55 at 3 months (P = .006), and total or conjugated bilirubin < 7.5/2.5 μmol/L at 6 months postoperatively (P ≤ .014) were the strongest predictors of a stable, nonprogressive fibrosis. Decreases in total or conjugated bilirubin to < 12.5/7.5 μmol/L by 3 months and to < 7.5/2.5 μmol/L by 6 months improved survival of the native liver (log-rank P ≤ .025). Age at follow-up or at portoenterostomy, anatomic type of biliary atresia, use of postoperative steroids, and episodes of cholangitis were unrelated to the progression of fibrosis or the development of cirrhosis (P = not significant). Conclusion: Among patients whose serum bilirubin normalizes after portoenterostomy, its rapid decrease to very low levels prolongs the survival of their native liver by delaying the progression of fibrosis.

show abstract

Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia

Cited by 48 publications

References 25 publications

A paradigm shift in the intention‐to‐transplant children with biliary atresia: Outcomes of 101 cases and a review of the literature

A paradigm shift in the intention‐to‐transplant children with biliary atresia: Outcomes of 101 cases and a review of the literature

Multivariate Analysis of Biliary Flow-Related Factors and Post-Kasai Survival in Biliary Atresia Patients

Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis

Contact Info

Product

Resources

About