Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding

Duché, Mathieu; Ducot, B.; Ackermann, O.; Guérin, Florent; Jacquemin, Emmanuel; Bernard, Olivier

doi:10.1016/j.jhep.2016.09.006

Cited by 92 publications

(92 citation statements)

References 31 publications

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“…In clinical practice, recognizing significant PH is of particular importance as it influences patient well‐being and may warrant endoscopic interventions for esophageal varices . Primary prophylaxis of high‐risk varices among children has been recommended due to the mortality and morbidity related to acute gastrointestinal bleeding episodes . In our series, apart from 1 patient with PH who underwent LT at a young age, esophageal varices were observed in others soon after detection of PH.…”

Section: Discussionmentioning

confidence: 80%

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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Section: Discussionmentioning

confidence: 80%

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

et al. 2019

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“…To the best of our knowledge, publications even in the recent past in regard to screening high-risk varices with PHT in children have not dealt with Baveno VI criteria. [26][27][28][29][30] However, our foremost weakness was the scarcity of the cACLD cases below 18 years, thus restricting our sample size, which meant that the plan had to be earmarked as a pilot study only. Six children (18.18%), comprising four infants with EHBA and one toddler with PFIC, continued to have high TSB, even after delivering available treatment, at 3 months of enrollment, thus acting as an effect modifier of TE values.…”

Section: Discussionmentioning

confidence: 99%

<p>Validation of Revised Baveno VI Criteria for Screening of Varices Needing Treatment in Children with Cirrhosis</p>

Samanta¹,

Biswas²,

Ghosh³

et al. 2020

CEG

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Background: Baveno VI criteria for screening varices needing treatment (VNT) have not yet been validated in an exclusive pediatric and adolescent set of the population, in whom baseline parameters differ in relation to adults. Therefore, our primary objective was to validate Baveno VI and its expanded form in children below 18 years of age. The secondary aim was to elicit whether any revision of the above criteria with a target of not missing more than 5% VNT could be more accurate for this age group. Materials and Methods: The work was carried out in two medical institutes, over a span of 3 years. Consecutively enrolled patients below 18 years of age, with compensated cirrhosis confirmed by liver biopsy, were evaluated for related blood parameters, transient elastography (TE) and esophagogastroduodenoscopy. Results: Out of the 33 recruited patients, five (15.15%) met the criteria for VNT. The sensitivity, specificity, PPV and NPV of Baveno VI and Expanded Baveno VI were observed as 60%, 92.3%, 60% and 92.3%, and 20%, 100%, 100% and 88%, respectively. We found that the Revised Baveno VI criteria with TE <19 kPa and platelet count of >175×10 9 cells/L, with sensitivity 100%, specificity 79%, PPV 45%, NPV 100% and accuracy of 82%, are more appropriate for this age group. Conclusion: We propose that further multicentrer studies with a larger sample size should be conducted before incorporating Revised Baveno VI criteria for high-risk varices in patients below 18 years in future guidelines.

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“…We read with interest the recent study by Dr Duché et al 1 about primary prophylaxis. They answer several questions raised by the Baveno consensus workshop.…”

Section: Primary Prophylaxis Of Oesophageal Variceal Bleeding In Chilmentioning

confidence: 99%

Primary prophylaxis of oesophageal variceal bleeding in children by ligation is safe and as efficient as secondary prophylaxis

Galand

Ley

Coopman

et al. 2018

Journal of Hepatology

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Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding

Cited by 92 publications

References 31 publications

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

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

<p>Validation of Revised Baveno VI Criteria for Screening of Varices Needing Treatment in Children with Cirrhosis</p>

Primary prophylaxis of oesophageal variceal bleeding in children by ligation is safe and as efficient as secondary prophylaxis

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