International Registry of Congenital Portosystemic Shunt (IRCPSS)

Conditions

• CPSS (Congenital Portosystemic Shunt)
• Congenital Portosystemic Shunt

Eligibility Criteria

Sex

ALL

Age

1 Day - N/A

Healthy Volunteers

Not accepted

Interventions

• Shunt Closure — PROCEDURE
  Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.

Study Details

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.

Key Dates

Start date

Apr 26, 2018

Status verified

Dec 2024

Primary completion

Oct 26, 2026

Completion

Dec 31, 2028

Study Design

Enrollment

500 participants (estimated)

Arms

• Arm: Neonate, Children, Adult with CPSS

Primary Outcome Measure

Numbers of patients with spontaneous shunt closure [ Time Frame: 3; 6; 12; 24 months ]

Locations (2)

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