Biliary Atresia Patients With Successful Kasai Portoenterostomy Can Present With Features of Obliterative Portal Venopathy

Patel, Kalyani R.; Harpavat, Sanjiv; Khan, Zahida; Dhingra, Sadhna; Quintanilla, Norma; Firan, Mihail; Goss, John A.

doi:10.1097/mpg.0000000000002701

Cited by 17 publications

(14 citation statements)

References 33 publications

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“…Moreover, these parameters were easily obtained and could be evaluated in various hospital settings regardless of sophisticated equipment. The majority of the 14 parameters reported in our research were nutritional status, liver laboratory function, and sonographic features, which showed consistency with the results of previous articles (25,26). BA patients are usually accompanied by poor liver function and different stages of liver fibrosis.…”

Section: Discussionsupporting

confidence: 91%

Development and Assessment of Screening Nomogram for Biliary Atresia Based on Hepatobiliary Ultrasonographic Features

Yang

Sun

et al. 2021

Front. Pediatr.

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Objectives: Biliary atresia (BA) is a rare neonatal liver disease of which the early diagnosis remains a challenge for clinicians. Our center has established a nomogram diagnostic model based on clinical characteristics and liver function characteristics. We aim to develop and validate a nomogram that includes additional ultrasound and finds hepatobiliary abnormality with better BA early screening performance.Methods: In this single-center, retrospective cohort analysis, 1,001 neonatal obstructive jaundice (NOJ) patients between 2012 and 2015 were enrolled. Multivariable analysis was used to identify clinical characteristics, laboratory liver function characteristics, and ultrasonic features that may early screen BA. A nomogram was developed to predict the probability of BA using multiple logistic regression analysis. This nomogram was subsequently validated using another cohort of 501 NOJ patients between 2015 and 2017. Calibration curve analysis and decision curve analyses were performed to evaluate and interpret the nomogram's clinical benefits.Results: Gender, direct bilirubin (DB), alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (GGT), fasting gallbladder visibility, fasting gallbladder filling, and common bile duct visibility were found to have profound statistical significance between the BA and non-BA groups (P < 0.05). The significant features were used to build the nomogram. The area under the receiver operating characteristic (ROC) curve (AUC) value of the novel nomogram (0.87) was superior to those of the former nomogram (0.83) and GGT alone (0.81) in the prediction of BA. The calibration curve revealed a close resemblance between the predicted and actual BA probabilities. Also, the net benefit from the decision curve analysis (DCA) of the nomogram (0.54) was superior to those of the former nomogram (0.49) and GGT alone (0.45) at 80% of threshold possibility.Conclusions: The nomogram has demonstrated better performance for BA screening by including additional information of the US finding, holding a promising future as a non-invasive method for BA patients.

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Section: Discussionsupporting

confidence: 91%

Development and Assessment of Screening Nomogram for Biliary Atresia Based on Hepatobiliary Ultrasonographic Features

Yang

Sun

et al. 2021

Front. Pediatr.

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“…The risk of spontaneous variceal bleeding depends primarily on the endoscopic pattern of gastroesophageal varices, regardless of the cause of liver disease (12,(24)(25)(26)(27). This may result, in part, from the presence, in children with many cirrhotic as well as noncirrhotic causes of portal hypertension, of lesions and\or obstruction of the intrahepatic branches of the portal vein (32)(33)(34)(35)(36). These lesions create a presinusoidal block and could explain why the endoscopic patterns associated with a high risk of bleeding are similar in cirrhotic and noncirrhotic children.…”

Section: Discussionmentioning

confidence: 99%

Efficacy and Safety of Endoscopic Primary Prophylaxis of Bleeding in Children With High-Risk Gastroesophageal Varices

Ackermann

Darmellah-Remil

Bernard

et al. 2022

Journal of Pediatric Gastroenterology &Amp; Nutrition

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Objectives: Primary prophylaxis of bleeding is debated in children with gastroesophageal varices; one of the reasons is the limited number of studies concerning its efficacy and safety. We report our experience with endoscopic primary prophylaxis. Methods: From 2006 to 2019, 145 children (median age, 3.5 years; cirrhosis, n = 116) with high-risk gastroesophageal varices underwent primary prophylaxis (banding, n = 114; sclerotherapy n = 31, primarily in smaller children). Results: We observed the eradication of varices in 93% of children after a mean of 6 months, at least one recurrence of varices in 45% after eradication, and gastrointestinal bleeding in 17% of children. Irrespective of the cause of portal hypertension, grade 3 esophageal varices, presence of gastric varices along the cardia and a lower composite score of endoscopic severity were associated with a worse probability of eradication, a longer time to eradication and a lower risk of a first recurrence and of bleeding following the procedure, respectively. Ten-year probabilities of overall survival and of bleeding-free survival were 95% and 75%, respectively. Conclusions: Endoscopic primary prophylaxis of variceal bleeding is reasonably effective and safe in children with high-risk gastroesophageal varices. Worse results are observed in children with more advanced endoscopic features. This pleads for endoscopic screening in children with portal hypertension and early detection of varices warranting primary prophylaxis.

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“…PHT in patients with BA after a successful Kasai procedure may not be due to advanced fibrosis, but obliterative portal venopathy. 33 EHPVO presents with clinically significant PHT with preserved liver function. 34 In fact, none of the patients in the present study was classified as Child-Pugh class C, so poor liver function might not help stratify the risk of early rebleeding after EVL.…”

Section: Discussionmentioning

confidence: 99%

Factors associated with bleeding after endoscopic variceal ligation in children

Yokoyama

Ishizu

Ishigami

et al. 2021

Pediatrics International

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Background: Endoscopic variceal ligation (EVL) is a widely accepted treatment for esophagogastric varices in patients with portal hypertension (PHT). It is used for urgent treatment and prophylactic treatment of esophagogastric varices in pediatric as well as adult patients. However, major life-threatening adverse events such as early rebleeding can occur. Although early rebleeding after EVL among children and adolescents has been reported, the risk factors remain obscure. This study evaluated the risk factors for early rebleeding after EVL in children and adolescents. Methods: The subjects were children and adolescents (<18 years) with PHT who underwent EVL for esophagogastric varices. Early rebleeding was defined as hematemesis, active bleeding, or blood retention in the stomach, confirmed by esophagogastroduodenoscopy from 2 h to 5 days after EVL. Results: A total of 50 EVL sessions on 22 patients were eligible for this study. There were four episodes of early rebleeding. No other major adverse event has occurred. Multivariate analysis showed that EVL implemented at cardiac varices just below the esophagogastric junction (EGJ), within 5 mm from the EGJ, is the independent factor for a higher risk of early rebleeding: odds ratio 18.2 (95% confidence interval: 1.40-237.0), P = 0.02. Conclusions: Children and adolescents who undergo EVL for cardiac varices just below the EGJ have a higher risk of early rebleeding than those who do not.

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Biliary Atresia Patients With Successful Kasai Portoenterostomy Can Present With Features of Obliterative Portal Venopathy

Cited by 17 publications

References 33 publications

Development and Assessment of Screening Nomogram for Biliary Atresia Based on Hepatobiliary Ultrasonographic Features

Development and Assessment of Screening Nomogram for Biliary Atresia Based on Hepatobiliary Ultrasonographic Features

Efficacy and Safety of Endoscopic Primary Prophylaxis of Bleeding in Children With High-Risk Gastroesophageal Varices

Factors associated with bleeding after endoscopic variceal ligation in children

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