WordNet
- a continuous tube formed by a row of elongated cells lacking intervening end walls
- an enclosed conduit for a fluid
- a bodily passage or tube lined with epithelial cells and conveying a secretion or other substance; "the tear duct was obstructed"; "the alimentary canal"; "poison is released through a channel in the snakes fangs" (同)epithelial duct, canal, channel
- a pattern of symptoms indicative of some disease
- a complex of concurrent things; "every word has a syndrome of meanings"
- a digestive juice secreted by the liver and stored in the gallbladder; aids in the digestion of fats (同)gall
- a sudden disappearance from sight
- a sudden or mysterious disappearance
PrepTutorEJDIC
- 送水管;(建物・船などの)通気管;(地下ケーブルなどの)ダクト / (体液を運ぶ)導管,(植物が水・空気を運ぶ)導管
- (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
- 胆汁(たんじゅう) / かんしゃく;不きげん
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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/10/05 04:48:41」(JST)
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Vanishing bile duct syndrome (or "ductopenia") is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.[1]
Contents
- 1 Etiology
- 1.1 Congenital/Developmental
- 1.1.1 Atretic causes
- 1.1.2 Fibrocystic causes
- 1.1.3 Chromosomal associations
- 1.1.4 Genetic associations
- 1.2 Immunologic associations
- 1.3 Other causes
- 2 Clinical presentation
- 3 Treatment
- 4 References
- 5 External links
Etiology
Congenital/Developmental
In fetal and neonatal life the ductal plates are remodeled. The malformations can be atretic or fibrocystic.
Atretic causes
- Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450)
- Extrahepatic bile duct atresia
Fibrocystic causes
- Autosomal recessive polycystic kidney disease
- Congential hepatic fibrosis
- Caroli's disease
- Von Meyenburg complex
Chromosomal associations
Genetic associations
- Cystic fibrosis
- Alpha 1 antitrypsin deficiency
- Trihydroxycoprostanic acidemia
- Byler's disease
Immunologic associations
Bile duct injury and loss can result from autoimmune destruction. T cells recognize biliary epithelial cell antigens causing injury and eventual atresia.
Other causes
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Hodgkin's lymphoma [2]
- Chronic graft-versus-host disease
- Drugs/Toxins
- Ischemia
Clinical presentation
The presentation is dependent upon the underlying etiology. The course can be rapid or chronic.
Symptoms
- Fatigue
- Anorexia
- Abdominal pain
- Weight loss
- Pruritus
Signs
- Hyperlipidemia
- Malabsorption
- Fat-soluble vitamin deficiencies
- Elevated alkaline phosphatase
- Elevated gamma-glutamyltransferase
- Elevated conjugated bilirubin
Treatment
Treatment is dependent upon the underlying etiology. Treatment is supportive as it is not possible to induce regrowth of lost ducts.
Medical therapies
- Ursodeoxycholic acid
- Immunosuppression
- General consensus is that more studies are needed before this can be considered
- Organ transplant
References
- ^ Reau NS, Jensen DM (February 2008). "Vanishing bile duct syndrome". Clin Liver Dis 12 (1): 203–17, x. doi:10.1016/j.cld.2007.11.007. PMID 18242505.
- ^ Rossini, M. S.; Lorand-Metze, I; Oliveira, G. B.; Souza, C. A. (2000). "Vanishing bile duct syndrome in Hodgkin's disease: Case report". Sao Paulo medical journal = Revista paulista de medicina 118 (5): 154–7. PMID 11018850.
External links
- Uptodate:Hepatic ductopenia and vanishing bile duct syndrome
UpToDate Contents
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English Journal
- Vanishing Bile Duct Syndrome in Hodgkin's Lymphoma: A Single Center Experience and Clinical Pearls.
- Bakhit M1, McCarty TR, Park S, Njei B, Cho M, Karagozian R, Liapakis A.
- Journal of clinical gastroenterology.J Clin Gastroenterol.2016 May 19. [Epub ahead of print]
- PMID 27203429
- Hydroxycut-related Vanishing Bile Duct Syndrome.
- Adike A1, Smith M2, Chervenak A1, Vargas HE1.
- Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association.Clin Gastroenterol Hepatol.2016 May 2. pii: S1542-3565(16)30141-0. doi: 10.1016/j.cgh.2016.04.028. [Epub ahead of print]
- PMID 27151488
- Vanishing bile duct syndrome associated with diffuse large B-cell lymphoma.
- Dachy G1, Connerotte T2.
- British journal of haematology.Br J Haematol.2016 May;173(4):505. doi: 10.1111/bjh.14004. Epub 2016 Mar 14.
- PMID 26988022
Japanese Journal
- Co-occurrence of cytomegalovirus-induced vanishing bile duct syndrome with papillary stenosis in HIV infection
- TYAGI Ila,PURI Amarender S.,SAKHUJA Puja,MAJUMDAR Kaushik,LUNIA Manish,GONDAL Ranjana
- Hepatology research : the official journal of the Japan Society of Hepatology 43(3), 311-314, 2013-03-01
- NAID 10031183472
- 肝組織像の経過を追えた中毒性表皮壊死症に合併した胆管消失症候群の1例
- 隅田 幸佑,道免 和文,相島 慎一,岩坂 翔,久本 仁美,原田 由紀子,三宅 典子,下野 信行,下田 慎治
- 肝臓 54(1), 67-73, 2013-01-25
- NAID 10031144322
Related Links
- Description. Vanishing bile duct syndrome (VBDS) is a rare but serious outcome and complication of drug induced liver injury marked clinically by chronic cholestasis and histologically by loss of intrahepatic bile ducts.
- 1. Clin Liver Dis. 2008 Feb;12(1):203-17, x. doi: 10.1016/j.cld.2007.11.007. Vanishing bile duct syndrome. Reau NS, Jensen DM. Author information: Center for Liver Diseases, Section of Gastroenterology, Department of Medicine ...
★リンクテーブル★
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- 英
- vanishing bile duct syndrome
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- 関
- canal、conduit、ductal、ductus、meatus、pipe、tubal、tube、vas、vessel
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