Congenital Portosystemic Shunt (CPSS) is a rare condition important by the multiplicity and severity of associated complications. CPSS is venous anomaly in which blood coming from the intestines only partially passes through the liver. This leads to the accumulation of potentially toxic factors that cause systemic effects. Complications vary among the individuals, and currently, it is challenging to predict which individuals will develop severe complications. The IRCPSS registry is established with the aim of centralizing detailed clinical follow-up and biological information from participants around the world who suffer from Congenital Portosystemic Shunt (CPSS). A multidisciplinary consortium of experts is collaborating to enhance our understanding of the prevalence, natural history, individual risks, and physiopathology of the disease through the IRCPSS registry.
Study Type
OBSERVATIONAL
Enrollment
500
Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.
Yale University School of Medicine
New Haven, Connecticut, United States
Lurie Children's Hospital
Chicago, Illinois, United States
Royal Children's Hospital Melbourne
Parkville, Victoria, Australia
Universitary Clinics Saint-Luc, UCL
Brussels, Belgium
The Hospital for Sick Children
Toronto, Ontario, Canada
Beaujon University Hospital
Clichy, France
Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris.
Le Kremlin-Bicêtre, France
Hôpital Necker
Paris, France
Hannover Medical School
Hanover, Germany
Haunersche Kinderklinik - LMU Munich
Munich, Germany
...and 16 more locations
Numbers of patients with spontaneous shunt closure
Time frame: 3; 6; 12; 24 months
Number of patients developing one or more complications
Time frame: 5; 10; 20 years
Number of patients undergoing preemptive closure
Time frame: 6; 12; 24 months
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