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Updated: Jun 17, 2026

Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
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Considerations in the development of the International Multicenter Pediatric Portal Hypertension Registry

Tassos Grammatikopoulos1,2, Catalina Jaramillo3, Jean Molleston4

  • 1Paediatric Liver, GI & Nutrition Centre and Mowat Labs, King's College Hospital NHS Trust, London, UK.

Journal of Pediatric Gastroenterology and Nutrition
|November 18, 2024
PubMed

Insights

This study details the creation of the International Multicenter Pediatric Portal Hypertension Registry (IMPPHR) to gather data on variceal hemorrhage in children. The registry aims to improve outcomes for pediatric portal hypertension through better understanding of hemorrhage and prophylaxis.

Area of Science:

  • Hepatology
  • Pediatric Gastroenterology
  • Clinical Research Methodology

Background:

  • Portal hypertension is a serious complication of chronic liver disease, leading to life-threatening variceal hemorrhage.
  • Managing variceal hemorrhage in children is challenging due to limited data on this rare condition.
  • Multicenter international registries are crucial for collecting data on rare diseases.

Purpose of the Study:

  • To establish the International Multicenter Pediatric Portal Hypertension Registry (IMPPHR).
  • To collect pediatric data on mortality from first variceal hemorrhage.
  • To evaluate primary and secondary prophylaxis strategies for variceal hemorrhage in children with portal hypertension.

Main Methods:

  • The IMPPHR was developed following symposia at Baveno V and VI meetings.
  • A formal executive committee initiated registry development in 2019.
  • Data collection occurred from 2020 to 2024 across 44 centers, including over 700 subjects.

Main Results:

  • The registry successfully gathered data from a significant number of pediatric patients.
  • Information on variceal hemorrhage incidence, mortality, and prophylaxis was collected.
  • The complexities and methodologies involved in developing the registry are described.

Conclusions:

  • The IMPPHR provides a valuable resource for studying pediatric portal hypertension and variceal hemorrhage.
  • This registry will facilitate evidence-based approaches to improve outcomes in children.
  • Further analysis of IMPPHR data is expected to guide clinical practice and research.

Keywords:
biliary atresiacirrhosisliver diseaseportal vein thrombosisvarices

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