Glucocorticosteroids for infants with biliary atresia following Kasai portoenterostomy

Tyraskis, Athanasios; Parsons, Christopher; Davenport, Mark

doi:10.1002/14651858.cd008735.pub3

Cited by 12 publications

(11 citation statements)

References 59 publications

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“…Age at HPE has been investigated mainly from the viewpoint of SNL and clearance of jaundice: 57 to 90% of patients undergoing HPE within 60 days of life will have a successful reestablishment of bile flow 45 ; as a result SNL appears to be improved. 46 Impact on perioperative complications has not been clearly analyzed. Our study found the absence of correlation between age at HPE and perioperative complications.…”

Section: Factors Associated With Complicationsmentioning

confidence: 99%

Perioperative Complications after Kasai Hepatoportoenterostomy: Data from the Swiss National Biliary Atresia Registry

Calinescu¹,

Wilde²,

Korff

et al. 2019

Eur J Pediatr Surg

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Introduction Hepatoportoenterostomy (HPE) is the first-line treatment for biliary atresia (BA) patients. This study aims to describe perioperative complications after HPE and to analyze their impact on outcome. Materials and Methods Patients with HPE (Swiss National Biliary Atresia Registry, 1994–2017) were retrospectively analyzed. Perioperative complications were defined as complications occurring up to 30 days after surgery. Surgical complications were defined as directly related to the surgical act; medical complications were defined as any other deviation from the uneventful postoperative course. Results Sixty-two patients were included. Median age at HPE was 63 days (18–126). Twenty six patients out of 62 (42%) had ≥ 1 complications: 6/62 (10%) surgical, 24/62 (39%) medical, that is, we observed 7 surgical and 28 medical complications. As for medical complications, cholangitis was the most frequent: 19/28 (68%). Lower gestational age at birth correlated with more overall complications (p = 0.02). Age, weight at HPE, syndromic BA, and postoperative steroid administration were not significantly correlated. There were no perioperative deaths. Perioperative complications did not correlate with overall survival (p = 0.14) and survival with native liver (p = 0.55). Conclusion HPE is often associated with perioperative medical complications. Lower gestational age at birth was significantly associated with more complications. Perioperative complications had no impact on overall outcome.

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Section: Factors Associated With Complicationsmentioning

confidence: 99%

Perioperative Complications after Kasai Hepatoportoenterostomy: Data from the Swiss National Biliary Atresia Registry

Calinescu¹,

Wilde²,

Korff

et al. 2019

Eur J Pediatr Surg

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“…Treatment with adjuvant steroids after KPE has previously been used to assist restoring bile flow and anti-inflammation; however, the effects of steroids on BA outcome remain controversial (35,36). In several studies, no benefits of postoperative steroids on prognosis were observed (36,37), whereas other studies showed that treatment with adjuvant steroids after KPE may improve short-term (≤1 year) JC. In this study, our results revealed that patients treated with adjuvant steroids initially suffered from no postoperative bile drainage and also had a higher risk obtaining a low 2-years NLS (HR = 1.992, 95% CI = 1.419-2.797, P = 0.000).…”

Section: Correction Of Coagulation and Protection Of Blood Vessels Canmentioning

confidence: 99%

Evaluation of Perioperative Complications in the Management of Biliary Atresia

Wang

Tang

et al. 2020

Front. Pediatr.

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Purpose: To analyze the influence of perioperative complications in the management of biliary atresia (BA). Methods: A retrospective study was performed using a total of 422 BA patients who underwent Kasai portoenterostomy (KPE) in a single institution between February 2016 and May 2017. Data on patients' clinical characteristics, laboratory examinations, perioperative complications, and outcomes were collected. Unpaired two-tailed t-test and χ 2 test were employed for the comparison between BA patients with and without perioperative complications. Cox regression analysis was used to screen the risk factors for 2-years NLS in BA, and their influence on the 2-years NLS was analyzed using Kaplan-Meier survival analysis as well as the log-rank test. Results: The incidence of perioperative complications, 6-months jaundice clearance (JC) and 2-years native liver survival (NLS) rate were 60.4, 59.5, and 56.6%, respectively. Patients with perioperative complications had lower serum albumin (ALB) level, but higher aspartate aminotransferase-to-platelet ratio index (APRI) and international normalized ratio (INR) levels when compared with those without perioperative complications (ALB, P < 0.05; APRI, P < 0.01; INR, P < 0.05). Moreover, perioperative complications were correlated with glucocorticoid administration (P = 0.002). Univariate Cox regression analysis showed no relationship between perioperative complications and 2-years NLS (P > 0.05). However, multivariate Cox regression analysis indicated 6-months JC was an independent protective factor for 2-years NLS [P < 0.0001, hazard ratio (HR) = 0.074, 95% confidence interval = 0.05-0.11], and concordance index of this prediction model including age, weight, APRI, glucocorticoid, and 6-months JC was 0.811. Conclusion: Although perioperative complication is common during and after KPE, it had no influence on the prognosis of BA. However, assessment of the serum level of total bilirubin after KPE may serve as an important predictor for the outcome in BA.

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“…Thus far, no post-HPE adjuvant therapy has been shown to change outcomes in BA. Several trials showed that prednisone treatment after HPE did not promote improved bile drainage and caused significant adverse effects 26, 39, 40 . Another trial showed that IVIG administration was similarly ineffective at promoting bile drainage post-HPE 27 .…”

Section: Clinical Managementmentioning

confidence: 99%

Recent advances in understanding biliary atresia

2019

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Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.

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Glucocorticosteroids for infants with biliary atresia following Kasai portoenterostomy

Cited by 12 publications

References 59 publications

Perioperative Complications after Kasai Hepatoportoenterostomy: Data from the Swiss National Biliary Atresia Registry

Perioperative Complications after Kasai Hepatoportoenterostomy: Data from the Swiss National Biliary Atresia Registry

Evaluation of Perioperative Complications in the Management of Biliary Atresia

Recent advances in understanding biliary atresia

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