Hepatology Committee

General Information

The Hepatology committee is a standing committee of ESPGHAN.


As an independent committee the Hepatology Commitee attempts to be a neutral body and aims to
a) represent the interests of hepatologists within the council and society;
b) promote high quality research in Paediatric Hepatology
c) create “consensus papers” in the field of Paediatric Hepatology 
d) to organize scientific meetings such as the Hepatology Summer School, specialized workshops, the Hepatology session during the ESPGHAN Annual Meeting and monothematic conferences and
e) work closely with the Hepatology Interest Group and the Trainee Committee to improve training in the field of Paediatric Hepatology.

The Hepatology Interest Group (HIG) is open for all ESPGHAN Members with a special interest in liver. The HIG meets each year at the ESPGHAN Annual Meeting to discuss achievements and new ideas, and to promote networking in the field of Peadiatric Hepatology. The meeting is organized by the Hepatology Committee. The activities of the committee are reported annually to the ESPGHAN Council and ESPGHAN Members.

To download the annual committee report, please click here.

 

Hepatology Interest Group

IRCPSS: International Registry of Congenital Porto-Systemic Shunts

Contact:
Valerie Mc Lin, Switzerland, email: Valerie.McLin@hcuge.ch

Description: Absence of portal flow or incomplete portal flow is known to lead to a number of systemic complications in portal hypertension secondary to cirrhosis, in pre-hepatic portal hypertension secondary to obstruction of the portal vein with cavernous transformation, and in the rare setting of congenital porto-systemic shunts (cPSS).
Although its prevalence is not known, cPSS is believed to be a relatively rare condition in humans. cPSS are associated with severe systemic complications at all ages, which is why developing a registry to understand their prevalence, clinical and pathological presentation, and natural history is of considerable clinical importance.
The potential implications of abnormal porto-venous shunting and decreased hepatic portal flow are numerous and potentially serious. In children, cPSS are increasingly sought in patients presenting with unexplained neurological or psychiatric abnormalities, severe cardiopulmonary complications such as hepatopulmonary syndrome, portopulmonary hypertension and neonatal hight-output heart failure, as well as in primary liver tumours [1].
Other less well described associations include growth retardation or overgrowth, nephropathy and coagulation abnormalities. In adults, cPSS have been reported at all ages, and are often interpreted as incidental findings, although in referral centers they are now sought as part of the work up of pulmonary hypertension [2].
Although cPSS are increasingly suspected and sought in specialized centers, much is still unknown clinically, histologically and biologically. They have been studied in some detail in other mammals including dogs, cats[3], and mice [4].
Although these models are valuable, understanding the pathophysiology and natural history in human subjects is paramount for two reasons: first, to identify patients at risk of developing complications, and second to determine the timing and type of management according to clinical presentation. At present, this rare clinical problem affecting both children and adults is managed and studied in a few specialized centers thereby lending itself well to the creation of a collaborative registry with the goals to a) characterize the natural history of cPSS b) develop a clear, consensual nomenclature c) identify subjects at risk of developing complications and d) standardize care of patients with cPSS.

Projects & Activities: to learn more about the IRCPSS, you can download information here.

Terms of Reference: to view the ToR of this Group, please click here.

GIG: Graft Injury Group

Contact Deirdre Kelly, United Kingdom, email:deirdre.kelly@bch.nhs.uk

Projects & Activities: to view all recent and upcoming activities of the Graft Injury Group, you can download the annual committee report here.

Terms of Reference: to view the ToR of this Graft Injury Group, please click here.

NITE: Network of Intestinal rehabilitation and Transplantation in Europe

Contact
Jutta Köglmeier, United Kingdom, email: jutta.koeglmeier@gosh.nhs.uk

Description:
The intestinal failure and transplantation group is established with representation from the ESPGHAN Hepatology Committee, Gastroenterology Committee and Nutrition Committee to
a) improve outcome of children with intestinal failure and intestinal transplantation
b) improve collaboration and develop a model of managed clinical network within Europe between European Intestinal Transplantation Centres and Intestinal Rehabilitation centres
c)sharing of expertise between experienced and less experienced centres with the ultimate aim of improving outcome of children with intestinal failure and those undergoing intestinal transplantation, d) close collaboration of clinical expertise will translate into collaborative research between different centres
The group was established in 2009 and has been successfully running for the last 5 years Members:
Girish Gupte
Florence Lacaille
Raanan Shamir
Sanja Kolacek
Lorenzo D’Antiga
Ekkehard Sturm
John Puntis

Projects & Activities: to view all recent and upcoming activities of the NITE Group, you can dowload the annual committee report here.

Terms of Reference: to view the ToR of this group, please click here.

ERN Rare Liver: European Reference Network Rare Liver

Contact
Ekkehard Sturm, Germany, email: ekkehard.sturm@med.uni-tuebingen.de
Samy Cadranel - Co-Chair

Despcription: 

Projects & Activities: to view all recent and upcoming activities of the ERN, you can download the annual committee report here.

Terms of Reference: to view the ToR for this group, please click here.

Alpha1-Antitrypsin Deficiency

The Alpha1 Working Group focus on liver disease related to alpha1-antitrypsin deficiency (AATD) in children, and aims to develop networking and knowledge in this field.
It works closely with ERN Rare Liver to improve care of patients affected by this rare disease. This group was established in 2019 during the ESPGHAN Annual Meeting in Glasgow, UK and the ERN meeting in Hamburg, Germany.

The aims of this working group are to:
a) implement a European registry
b) promote European pediatric research projects
c) identify modifiers associated with severe liver disease in AATD
d) collaborate with ERN – Rare Liver in order to develop a unique training and knowledge sharing resources for professionals and patients

Projects & Activities:

Members:

This working group remains open to all goodwill. If you are interested in participating, please feel free to contact us.

  • Mathias Ruiz
  • Emmanuel Gonzales
  • Ekkehard Sturm
  • Florence Lacaille
  • Lioara Restier
  • Marion Bouchecareilh
  • Alain Lachaux

Networking/Meetings:

The Alpha1 Working Group met in Paris in October 2019, in cooperation with ERN Rare Liver, in order to establish the bases of the group and the axes for further works. The proposals are as follows

Current actions:

  • Implementation and diffusion of the electronic database of alpha1antitrypsin variants (L. Restier, P. Joly, M. Ruiz, A. Lachaux)
  • An histological study in order to compare histological findings in children and adults, in cooperation with ERN (P. Strnad, M. Ruiz)
  • Identification of genetic polymorphisms involved in severe liver disease related to AATD (M. Bouchecareilh, MF. Odou, M. Balduyck, M. Ruiz, A. Lachaux)

Further projects:

  • To create a common registry with a common eCRF, based on the French cohort DEFIALPHA
  • Position paper on management of liver disease related to AATD both in adults and children, in relation with ERN Rare Liver
  • Position paper on management of liver disease related to AATD in children.