CEPS
MCID: CNG092
MIFTS: 18

Congenital Extrahepatic Portosystemic Shunt (CEPS)

Categories: Rare diseases

Aliases & Classifications for Congenital Extrahepatic Portosystemic Shunt

MalaCards integrated aliases for Congenital Extrahepatic Portosystemic Shunt:

Name: Congenital Extrahepatic Portosystemic Shunt 53
Abernethy Malformation 53
Ceps 53

Classifications:



Summaries for Congenital Extrahepatic Portosystemic Shunt

NIH Rare Diseases : 53 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.

MalaCards based summary : Congenital Extrahepatic Portosystemic Shunt, also known as abernethy malformation, is related to porphyria, congenital erythropoietic and porphyria cutanea tarda. Affiliated tissues include liver, heart and kidney.

Related Diseases for Congenital Extrahepatic Portosystemic Shunt

Diseases related to Congenital Extrahepatic Portosystemic Shunt via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 37)
# Related Disease Score Top Affiliating Genes
1 porphyria, congenital erythropoietic 12.2
2 porphyria cutanea tarda 11.1
3 hepatocellular carcinoma 10.5
4 hepatopulmonary syndrome 10.3
5 heterotaxy 10.3
6 encephalopathy 10.3
7 frasier syndrome 10.2
8 porphyria 10.2
9 pneumonia 10.2
10 eosinophilic pneumonia 10.2
11 chronic eosinophilic pneumonia 10.2
12 pulmonary hypertension 10.1
13 congenital portosystemic shunt 10.1
14 fatty liver disease, nonalcoholic 1 10.1
15 inguinal hernia 10.1
16 thrombosis 10.1
17 klippel-trenaunay-weber syndrome 10.0
18 tetralogy of fallot 10.0
19 cholangiocarcinoma 10.0
20 chronic kidney failure 10.0
21 visceral heterotaxy 10.0
22 heart disease 10.0
23 nephrotic syndrome 10.0
24 hepatic coma 10.0
25 hepatic encephalopathy 10.0
26 biliary atresia 10.0
27 ventricular septal defect 10.0
28 vascular disease 10.0
29 hyperinsulinism 10.0
30 kidney disease 10.0
31 hepatoblastoma 10.0
32 weber syndrome 10.0
33 ovarian cancer 10.0
34 tropical calcific pancreatitis 10.0
35 ectopic pregnancy 10.0
36 churg-strauss syndrome 10.0
37 allergic angiitis 10.0

Graphical network of the top 20 diseases related to Congenital Extrahepatic Portosystemic Shunt:



Diseases related to Congenital Extrahepatic Portosystemic Shunt

Symptoms & Phenotypes for Congenital Extrahepatic Portosystemic Shunt

Drugs & Therapeutics for Congenital Extrahepatic Portosystemic Shunt

Search Clinical Trials , NIH Clinical Center for Congenital Extrahepatic Portosystemic Shunt

Genetic Tests for Congenital Extrahepatic Portosystemic Shunt

Anatomical Context for Congenital Extrahepatic Portosystemic Shunt

MalaCards organs/tissues related to Congenital Extrahepatic Portosystemic Shunt:

41
Liver, Heart, Kidney, Ovary

Publications for Congenital Extrahepatic Portosystemic Shunt

Articles related to Congenital Extrahepatic Portosystemic Shunt:

(show top 50) (show all 81)
# Title Authors Year
1
Catheter Embolization of Type II Congenital Extrahepatic Portosystemic Shunt with Hematochezia: A Case Series and Review of the Literature. ( 29691614 )
2018
2
Correction to: Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation). ( 29931385 )
2018
3
Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation). ( 29675774 )
2018
4
Gradual attenuation of a congenital extrahepatic portosystemic shunt with a self-retaining polyacrylic acid-silicone device in 6 dogs. ( 29697147 )
2018
5
Recanalisation of a congenital extrahepatic portosystemic shunt previously attenuated with cellophane banding in a cat. ( 30186617 )
2018
6
Erratum: Recanalisation of a congenital extrahepatic portosystemic shunt previously attenuated with cellophane banding in a cat. ( 30263145 )
2018
7
Congenital extrahepatic portosystemic shunt: description of four cases and review of the literature. ( 30357760 )
2018
8
Endovascular Closure of a Large Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation) Using Vena Cava Filter and AMPLATZER Vascular Plugs. ( 30368322 )
2018
9
Laparoscopic ligation of a congenital extrahepatic portosystemic shunt for children with hyperammonemia: a single-institution experience. ( 30392166 )
2018
10
Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature. ( 30363163 )
2018
11
Hepatocellular Carcinoma in Abernethy Malformation: A Rare Occurrence of Congenital Complete Portosystemic Shunt. ( 30502882 )
2018
12
A rare case of portosystemic shunt: Variation of Abernethy malformation. ( 29233631 )
2018
13
Confusion for Fifteen Years: A Case of Abernethy Malformation. ( 29330099 )
2018
14
Haemodynamic consequences following closure of an Abernethy malformation in a patient following a total cavopulmonary shunt. ( 29463344 )
2018
15
The Abernethy malformation with inferior caval vein hypoplasia: a tailored technique for transcatheter closure and an insight into embryological perspective. ( 29978777 )
2018
16
Abernethy Malformation Type II and Concurrent Nodular Hyperplasia in a Rare Female Case. ( 30073109 )
2018
17
Rex Shunt as a Treatment of Type II Abernethy Malformation with Severe Dysplasia of Portal Vein. ( 30081160 )
2018
18
Abernethy malformation: Single-center experience from India with review of literature. ( 30187299 )
2018
19
Abernethy malformation type 2: varied presentation, management and outcome. ( 30262201 )
2018
20
Histopathology of livers in patients with congenital portosystemic shunts (Abernethy malformation): a case series of 22 patients. ( 30357455 )
2018
21
Liver histopathology in patients with hepatic masses and the Abernethy malformation. ( 30528201 )
2018
22
Prepared for the Unexpected: Accessory Ovaries with Abernethy Malformation. ( 30553034 )
2018
23
A Type II Congenital Extrahepatic Portosystemic Shunt: A Rare Cause of Abdominal Pain Diagnosed by Point-of-Care Ultrasound. ( 28162834 )
2017
24
Congenital Extrahepatic Portosystemic Shunt Embolization with the Use of a Duct Occluder in a Neonate with Liver Dysfunction and Hyperammonemia. ( 28841939 )
2017
25
Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) With Associated Hepatocellular Carcinoma: Case Report and Literature Review. ( 28727971 )
2017
26
Rare association of progressive congenital extrahepatic portosystemic shunt with development of multifocal hepatocellular carcinoma. ( 30363309 )
2017
27
Transplantation for Large Liver Tumors in the Setting of Abernethy Malformation. ( 28302006 )
2017
28
Three-Dimensional Printing Facilitates Successful Endovascular Closure of a Type II Abernethy Malformation Using an Amplatzer Atrial Septal Occluder Device. ( 28502889 )
2017
29
Abernethy malformation associated with Caroli's syndrome in a patient with a PKHD1 mutation: a case report. ( 28814334 )
2017
30
Congenital Extrahepatic Portosystemic Shunt in a Child With Caroli's Syndrome. ( 28119942 )
2016
31
Congenital Extrahepatic Portosystemic Shunt with Multiple Visceral Aneurysms. ( 26781025 )
2016
32
Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) with Associated Hepatocellular Carcinoma: Case report and Literature Review. ( 27116469 )
2016
33
Role of ultrasonography in early diagnosis of congenital extrahepatic portosystemic shunt. ( 30363630 )
2016
34
Multiple regenerative nodular hyperplasia in the left infrarenal vena cava accompanied by Abernethy malformation. ( 25813917 )
2016
35
The Abernethy malformation-myriad imaging manifestations of a single entity. ( 27857464 )
2016
36
Abernethy malformation type I (congenital absence of the portal vein) in a patient with chronic kidney disease. ( 28010000 )
2016
37
Congenital extrahepatic portosystemic shunt. ( 26140564 )
2015
38
Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma. ( 26459734 )
2015
39
Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome. ( 26311567 )
2015
40
Premature pubarche, hyperinsulinemia and hypothyroxinemia: novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. ( 25676144 )
2015
41
Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. ( 25624897 )
2015
42
Surgical ligation of portosystemic shunt to resolve severe hematuria and hemafecia caused by type II abernethy malformation. ( 25770392 )
2015
43
Prenatal diagnosis of Abernethy malformation by three-dimensional ultrasonography. ( 26062719 )
2015
44
Focal nodular hyperplasia in an individual with Abernethy malformation type 1b. ( 26341965 )
2015
45
Abernethy Malformation. ( 26371585 )
2015
46
Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. ( 26385342 )
2015
47
Abernethy malformation with Inferior Vena Cava stenosis: A cryptic cause of cyanosis. ( 26702682 )
2015
48
Single Stage Endovascular Treatment of a Type 2 Abernethy Malformation: Successful Nonsurgical Outcome in a Case Report. ( 26770860 )
2015
49
An unusual presentation of type II Abernethy malformation. ( 24530574 )
2014
50
Porto-caval shunt or Abernethy malformation. ( 24794522 )
2014

Variations for Congenital Extrahepatic Portosystemic Shunt

Expression for Congenital Extrahepatic Portosystemic Shunt

Search GEO for disease gene expression data for Congenital Extrahepatic Portosystemic Shunt.

Pathways for Congenital Extrahepatic Portosystemic Shunt

GO Terms for Congenital Extrahepatic Portosystemic Shunt

Sources for Congenital Extrahepatic Portosystemic Shunt

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