60 Days in Biliary Atresia: A Historical Dogma Challenged

Tessier, Mary Elizabeth M.; Shneider, Benjamin L.

doi:10.1002/cld.843

Cited by 10 publications

(9 citation statements)

References 17 publications

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“…In our case, ultrasonography, hepatobiliary scintigraphy, and liver biopsy confirmed the diagnosis of biliary atresia. Early diagnosis, ideally earlier than 30–45 days, followed by the Kasai procedure, results in better outcomes 12 . In our case, the Kasai operation was performed on the 40th day of life.…”

Section: Discussionmentioning

confidence: 70%

Biliary atresia and posterior fossa bleed: Chance or causality. A case report and review of the literature

Karki,

Chand,

Parajuli

et al. 2023

Clinical Case Reports

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Key Clinical MessageA newborn with a rare form of biliary atresia had posterior fossa bleed and subarachnoid hemorrhage despite vitamin K prophylaxis, indicating biliary atresia is a causality rather than chance.AbstractBiliary atresia frequently causes surgical jaundice, resulting in delayed vitamin K deficiency. We report a 28‐day‐old newborn diagnosed with a rare form of biliary atresia presented with an unusual association of posterior fossa bleed and subarachnoid hemorrhage despite vitamin K prophylaxis. Thus, biliary atresia remains causality rather than chance.

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Section: Discussionmentioning

confidence: 70%

Biliary atresia and posterior fossa bleed: Chance or causality. A case report and review of the literature

Karki,

Chand,

Parajuli

et al. 2023

Clinical Case Reports

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“…Patients from the most recent cohort in that study (2010 to 2015) had 87% 5-year survival, an improvement from 72% in the first part of the study (1986 to 1996). This trend is naturally expected with accumulated experience over the years, and the awareness that the 60-day paradigm for KPE must be shifted to early intervention in the course of the disease ( 23 ).…”

Section: Discussionmentioning

confidence: 89%

In vitro fertilization: an unexpected finding in a cohort of patients with biliary atresia

Costa

Porta

Miura

et al. 2023

Braz J Med Biol Res

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“…In our group of patients, except for a patient operated at 196 days of age, the mean age at surgery was 58 days (42–84 days), which is concordant with the European guidelines [ 6 ]. Even though 60 days is considered a cut-off value for a successful surgery, the best results are thought to be obtained in patients aged between 45 and 60 days [ 3 ]. Some studies suggested surgery may be performed even before the age of 30 days, achieving jaundice clearance in all patients [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…This was a reason to change the focus toward a faster diagnostic, given that younger patients age have a less advanced stage of the disease, with consecutively, better results. The ideal time for surgery is currently considered between the first 30–45 days of life, although when performed at 45–60 days it seemed to be associated with better results [ 3 , 4 ]. An important tool for the early detection of BA would be a screening program in which the efficiency of stool color cards is highlighted [ 5 ] together with case centralization in high volume centers [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Biological and Histological Assessment of the Hepatoportoenterostomy Role in Biliary Atresia as a Stand-Alone Procedure or as a Bridge toward Liver Transplantation

et al. 2020

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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.

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60 Days in Biliary Atresia: A Historical Dogma Challenged

Abstract: http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/15-s1-reading-tessier a video presentation of this article

Cited by 10 publications

References 17 publications

Biliary atresia and posterior fossa bleed: Chance or causality. A case report and review of the literature

Biliary atresia and posterior fossa bleed: Chance or causality. A case report and review of the literature

In vitro fertilization: an unexpected finding in a cohort of patients with biliary atresia

Biological and Histological Assessment of the Hepatoportoenterostomy Role in Biliary Atresia as a Stand-Alone Procedure or as a Bridge toward Liver Transplantation

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