Treatment Policy and Liver Histopathology Predict Biliary Atresia Outcomes: Results after National Centralization and Protocol Biopsies

Hukkinen, Maria; Kerola, Anna; Heikkilä, Päivi; Merras‐Salmio, Laura; Jahnukainen, Timo; Koivusalo, Antti; Jalanko, Hannu; Pakarinen, Mikko P.

doi:10.1016/j.jamcollsurg.2017.09.009

Cited by 42 publications

(73 citation statements)

References 48 publications

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“…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%

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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

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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.

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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

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“…Biliary atresia (BA) is a progressive fibroinflammatory cholangiopathy presenting exclusively in newborns and accounting for the majority of pediatric liver transplantations (LTs) worldwide . Currently, clearance of jaundice (COJ) rates after portoenterostomy (PE) exceed 50%, and up to 25% of patients survive with their native liver (NL) until adulthood . Although patients who still have cholestasis after PE rapidly progress to end‐stage liver disease and require LT for survival, the disease course is far more variable after COJ.…”

mentioning

confidence: 99%

“…Currently, clearance of jaundice (COJ) rates after portoenterostomy (PE) exceed 50%, and up to 25% of patients survive with their native liver (NL) until adulthood . Although patients who still have cholestasis after PE rapidly progress to end‐stage liver disease and require LT for survival, the disease course is far more variable after COJ. Following a successful PE, most patients survive with their NLs for many years .…”

mentioning

confidence: 99%

“…Although patients who still have cholestasis after PE rapidly progress to end‐stage liver disease and require LT for survival, the disease course is far more variable after COJ. Following a successful PE, most patients survive with their NLs for many years . However, during their disease course, the majority of children gradually progress to cirrhosis and develop symptoms of portal hypertension (PH), whereas others remain free from liver disease complications during childhood …”

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confidence: 99%

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Noninvasive Evaluation of Liver Fibrosis and Portal Hypertension After Successful Portoenterostomy for Biliary Atresia

et al. 2019

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We investigated noninvasive follow‐up markers for histologic liver fibrosis and portal hypertension (PH) in patients with biliary atresia after successful portoenterostomy (PE). Among children with bilirubin <20 µmol/L after PE (n = 39), Metavir fibrosis stage was evaluated at PE and in follow‐up protocol liver biopsies (n = 83). PH was defined as endoscopically confirmed esophageal varices or thrombocytopenia associated with splenomegaly. The accuracy of liver biochemistry and stiffness in detecting liver fibrosis and PH was analyzed by the area under the receiving operating characteristic curve (AUROC) and multiple regression models. During a median native liver survival of 8.3 years (interquartile range 2.5‐10.8 years), cirrhosis (Metavir F4) had developed in 51% of patients and PH in 54% of patients. Cirrhosis was equally common in all age tertiles of 1.2‐2.1 years (n = 10/27), 3.9‐5.8 years (n = 12/28), and 9.0‐14 years (n = 12/28). In the two oldest age tertiles, histologic liver fibrosis had progressed further in patients with PH than without PH (P < 0.001). PH was accurately predicted by the aspartate aminotransferase‐to‐platelet ratio index (APRI) (cutoff, 0.70; AUROC, 0.92), bile acids (cutoff, 49 µmol/L; AUROC, 0.91), and liver stiffness (cutoff, 16.9 kPa; AUROC, 0.89; P < 0.001 each) across all age tertiles. Liver stiffness was the most accurate predictor of cirrhosis overall (AUROC, 0.82; P < 0.001), whereas bilirubin was >11 µmol/L in the youngest tertile (AUROC, 0.91; P < 0.001), bile acids was >80 µmol/L in the middle tertile (AUROC, 0.81; P = 0.009), and liver stiffness was >24 kPa in the oldest age tertile (AUROC, 0.96; P = 0.002). Conclusion: After successful PE, development of PH associates with progression of liver fibrosis and can be accurately detected by APRI and stiffness. Liver stiffness most accurately identified cirrhosis in older children, whereas biochemical markers of cholestasis closely reflected histologic cirrhosis in younger children.

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Biliary atresia: a scientometric analysis of the global research architecture and scientific developments

Friedmacher

Ford

Davenport

2019

J Hepato Biliary Pancreat

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Biliary atresia (BA) is a rare cholangiopathy of largely unknown etiology and unpredictable outcome. There has been an increasing number of BA-related publications, which may challenge researchers to determine their actual scientific value. This study aimed to evaluate the global research activity and developments relating to BA using a combination of scientometric methodologies and visualization tools. A comprehensive search strategy for the Web of Science TM database was designed to obtain bibliographic data on scientific BA publications for the timespan 1900-2018. Research output of countries, institutions, individual authors and collaborative networks was analyzed. Semi-qualitative research measures including citation rate and h-index were assessed. Choropleth mapping and network diagrams were used to visualize results. In total, 4,459 publications on BA were identified (88.5% in English), originating from 63 countries. The largest number was published by the USA (n = 991; 22.2%), Japan (n = 667; 15.0%) and the UK (n = 294; 6.6%). The USA combined the highest number of cooperation articles (n = 140). The most productive collaborative network was established between the USA and Canada (n = 17). Scientific papers from the UK received the highest average citation rate (16.7), whereas the USA had the highest country-specific h-index (59). Eighty-eight (2.0%) items were published under the auspices of multicenter consortiums and registries. The most productive institutions and authors were based in the USA, the UK, Japan, France, Canada and Taiwan. BArelated research has constantly been progressing, becoming more multidisciplinary but with main research endeavors concentrated in a few high-income countries. Studies into pathogenesis of BA remain uncommon, but are sorely needed to foster true scientific progress with this rare disease. Hence, international collaborative and translational research should be strengthened to allow further evolution in this field.

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Treatment Policy and Liver Histopathology Predict Biliary Atresia Outcomes: Results after National Centralization and Protocol Biopsies

Cited by 42 publications

References 48 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

Noninvasive Evaluation of Liver Fibrosis and Portal Hypertension After Successful Portoenterostomy for Biliary Atresia

Biliary atresia: a scientometric analysis of the global research architecture and scientific developments

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