Congenital Hepatic Fibrosis

Common Name(s)

Congenital Hepatic Fibrosis

Description for this condition is not yet available.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Congenital Hepatic Fibrosis" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

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 Hepatic Fibrosis" returned 26 free, full-text research articles on human participants. First 3 results:

Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease.
 

Author(s): Meral Gunay-Aygun, Esperanza Font-Montgomery, Linda Lukose, Maya Tuchman Gerstein, Katie Piwnica-Worms, Peter Choyke, Kailash T Daryanani, Baris Turkbey, Roxanne Fischer, Isa Bernardini, Murat Sincan, Xiongce Zhao, Netanya G Sandler, Annelys Roque, Daniel C Douek, Jennifer Graf, Marjan Huizing, Joy C Bryant, Parvathi Mohan, William A Gahl, Theo Heller

Journal: Gastroenterology. 2013 Jan;144(1):112-121.e2.

 

Autosomal recessive polycystic kidney disease (ARPKD), the most common ciliopathy of childhood, is characterized by congenital hepatic fibrosis and progressive cystic degeneration of kidneys. We aimed to describe congenital hepatic fibrosis in patients with ARPKD, confirmed by detection ...

Last Updated: 24 Dec 2012

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Thrombocytopenia and splenomegaly: an unusual presentation of congenital hepatic fibrosis.
 

Author(s): Serena Botto Poala, Gianni Bisogno, Raffaella Colombatti

Journal:

 

Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disease that primarily affects the hepatobiliary and renal systems. It is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. Firm or hard hepatomegaly is present nearly in all patients, ...

Last Updated: 30 Apr 2010

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[A case of congenital hepatic fibrosis presented with recurrent acute cholangitis].
 

Author(s): Shin Young Lee, Hye Jin Joo, Young Shim Cho, Won Joong Jeon, Hee Bok Chae, Seon Mee Park, Sei Jin Youn, Rohyun Sung

Journal: Korean J Gastroenterol. 2009 Dec;54(6):404-8.

 

Acute cholangitis usually develops in congenital hepatic fibrosis (CHF), accompanied by cystic dilated bile ducts. However, it can also develop in simple CHF and may lead to critical course. A 30-year old man presented with recurrent acute cholangitis without bile duct dilatation. ...

Last Updated: 22 Dec 2009

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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 Hepatic Fibrosis" returned 4 free, full-text review articles on human participants. First 3 results:

Congenital hepatic fibrosis in autosomal recessive polycystic kidney disease.
 

Author(s): Jessica Wen

Journal: Clin Transl Sci. 2011 Dec;4(6):460-5.

 

Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis (CHF) characterized by dilated bile ducts ...

Last Updated: 3 Jan 2012

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Experience of a single center with congenital hepatic fibrosis: a review of the literature.
 

Author(s): Ali Shorbagi, Yusuf Bayraktar

Journal: World J. Gastroenterol.. 2010 Feb;16(6):683-90.

 

Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. It is one of the fibropolycystic diseases, which also include Caroli disease, autosomal ...

Last Updated: 5 Feb 2010

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Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF).
 

Author(s): Baris Turkbey, Iclal Ocak, Kailash Daryanani, Esperanza Font-Montgomery, Linda Lukose, Joy Bryant, Maya Tuchman, Parvathi Mohan, Theo Heller, William A Gahl, Peter L Choyke, Meral Gunay-Aygun

Journal: Pediatr Radiol. 2009 Feb;39(2):100-11.

 

ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical ...

Last Updated: 23 Dec 2008

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Clinical and Molecular Investigations Into Ciliopathies
 

Status: Recruiting

Condition Summary: Autosomal Recessive Polycystic Kidney Disease; Congenital Hepatic Fibrosis; Caroli's Disease; Polycystic Kidney Disease; Joubert Syndrome; Cerebro-Oculo-Renal Syndromes; COACH Syndrome; Senior-Loken Syndrome; Dekaban-Arima Syndrome; Cogan Oculomotor Apraxia; Nephronophthisis; Bardet-Biedl Syndrome; Alstrom Syndrome; Oral-Facial-Digital Syndrome

 

Last Updated: 11 Nov 2014

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UAB HRFD Core Center: Core A: The Hepato/Renal Fibrocystic Diseases Translational Resource
 

Status: Recruiting

Condition Summary: Hepato/Renal Fibrocystic Disease; Autosomal Recessive Polycystic Kidney Disease; Joubert Syndrome; Bardet Biedl Syndrome; Meckel-Gruber Syndrome; Congenital Hepatic Fibrosis; Caroli Syndrome; Oro-Facial-Digital Syndrome Type I; Nephronophthisis; Glomerulocystic Kidney Disease

 

Last Updated: 12 Jan 2014

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Liver Fibrosis in Sickle Cell Disease
 

Status: Recruiting

Condition Summary: Sickle Cell Disease

 

Last Updated: 3 Sep 2014

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