Congenital extrahepatic portosystemic shunt

Common Name(s)

Congenital extrahepatic portosystemic shunt, Abernethy malformation

Congenital extrahepatic portosystemic shunt (CEPS), also known as Abernethy malformation, occurs when blood vessels near the liver do not form correctly during fetal development.  The hepatic portal vein delivers blood to the liver; in CEPS, the portal vein may not develop or another blood vessel may attach to the portal vein, redirecting (shunting) blood away from the liver rather than through the liver. CEPS is divided into two types based on if blood can flow through the liver. In type 1, certain blood vessels in the liver are missing. In type 2, these blood vessels are present but they have abnormal connections.  Both types can be associated with other complications, such congenital heart disease, liver disease, nodules on the liver, and hepatic encephalopathy.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Congenital extrahepatic portosystemic shunt" for support, advocacy or research.

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Abernethy Malformation | Maddi's Hope

Maddi's Hope is place for those with Abernethy malformation of any type in their life to find support and information from others that have the same problems and challenges with this liver disorder.

Last Updated: 24 Apr 2014

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Congenital extrahepatic portosystemic shunt" for support, advocacy or research.

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Abernethy Malformation | Maddi's Hope

Maddi's Hope is place for those with Abernethy malformation of any type in their life to find support and information from others that have the same problems and challenges with this liver disorder.

http://abernethymalformationtoo.blogspot.com/

Last Updated: 24 Apr 2014

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Congenital extrahepatic portosystemic shunt" returned 3 free, full-text research articles on human participants. First 3 results:

Type 1 congenital extrahepatic portosystemic shunt.
 

Author(s): Akhilesh K Sista, Roy A Filly

Journal: J Ultrasound Med. 2009 May;28(5):703-5.

 

Last Updated: 24 Apr 2009

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Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children.
 

Author(s): Akira Nii, Hiro-o Takehara, Hisako Kuyama, Mitsuo Shimada

Journal: J. Med. Invest.. 2009 Feb;56(1-2):49-54.

 

A congenital extrahepatic portosystemic shunt (CEPS) is a rare abnormality. The shunts are classified into 2 types. Of these, a type 2-shunt is a side-to-side one, which may be treated by a simple shunt division. The aim of this retrospective study was to clarify the effects of a ...

Last Updated: 5 Mar 2009

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Congenital extrahepatic portosystemic venous shunt: imaging features.
 

Author(s): H Kandpal, R Sharma, N K Arora, S D Gupta

Journal: Singapore Med J. 2007 Sep;48(9):e258-61.

 

Congenital extrahepatic portosystemic venous shunt (CEPS) is a rare anomaly. It causes metabolic derangements and is often associated with liver tumours and other anomalies. Imaging plays an important role in the diagnosis of CEPS. However, it may be misleading in determining the ...

Last Updated: 30 Aug 2007

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Congenital extrahepatic portosystemic shunt" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.