WordNet

pertaining to or affecting the liver; "hepatic ducts"; "hepatic cirrhosis"
present at birth but not necessarily hereditary; acquired during fetal development (同)inborn, innate
development of excess fibrous connective tissue in an organ

PrepTutorEJDIC

肝臓の;肝臓にきく,肝臓のような色の
(病気・身体的欠陥など)生まれつきの,先天的な
線維症(線維性結合線維が増殖し過ぎる症状)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/10/21 02:16:10」(JST)

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Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension.

Overview

The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation.^[1]

Congenital hepatic fibrosis has an autosomal recessive pattern of inheritance.

Embryogenically, congenital hepatic fibrosis is due to malformation of the duct plate, a round structure appearing in the eighth week of gestation that is formed by primitive hepatocytes, which differentiate into cholangiocytes.^[2] Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include Cholangitis, hepatomegaly and signs of portal hypertension.

External links

GeneReviews/NCBI/NIH/UW entry on Congenital Hepatic Fibrosis Overview

References

^ "eMedicine - Congenital Hepatic Fibrosis : Article by Hisham Nazer, MBBCh, FRCP". Retrieved 2007-06-30.
^ Jorge OA, Jorge AD (2006). "Congenital hepatic fibrosis associated with von Recklinghausen's disease". Revista española de enfermedades digestivas : organo oficial de la Sociedad Española de Patología Digestiva 98 (9): 693–7. PMID 17092201.

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1. カロリー病 caroli disease
2. 肝線維症の病因 pathogenesis of hepatic fibrosis
3. 小児における常染色体劣性多発性嚢胞腎 autosomal recessive polycystic kidney disease in children
4. 肝内胆管の消失および胆管消失症候群 hepatic ductopenia and vanishing bile duct syndrome
5. 小児における常染色体優性多発性嚢胞腎 autosomal dominant polycystic kidney disease in children

English Journal

Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease.

Srinath A, Shneider BL.SourceDivision of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
Journal of pediatric gastroenterology and nutrition.J Pediatr Gastroenterol Nutr.2012 May;54(5):580-7.
OBJECTIVES: : The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD).METHODS: : A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V c
PMID 22197937

Portal hypertension in children.

Grimaldi C, de Ville de Goyet J, Nobili V.SourceDepartment of Paediatric Surgery and Transplantation Center, Bambino Gesù Childrens Hospital, Roma, Italy.
Clinics and research in hepatology and gastroenterology.Clin Res Hepatol Gastroenterol.2012 Apr 20. [Epub ahead of print]
The main causes of intrahepatic portal hypertension in children are cirrhosis and congenital hepatic fibrosis. Non cirrhotic portal hypertension in children is mostly due to extrahepatic portal vein obstruction. In half of cases, no underlying disorder is found. The meso-Rex bypass is the preferred
PMID 22521554

Japanese Journal

症例成人発症の先天性肝線維症の1例

野崎太希,村石懐,不破相勲 [他]
臨床放射線 56(9), 1108-1111, 2011-09
NAID 40018978081

CONGENITAL HEPATIC FIBROSIS WITHOUT ANY SYMPTOMS AS DIAGNOSED BY LAPAROSCOPY

KISAKA YOSHIYASU,ABE MASANORI,TOKUMOTO YOSHIO,HIROOKA MASASHI,SHIGEMATSU SHUICHIRO,KOIZUMI YOHEI,MURAKAMI HIDEHIRO,MATSUURA BUNZO,HIASA YOICHI,ONJI MORIKAZU
Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy Society 22(4), 357-359, 2010-10-01
NAID 10028187503