interlobular bile duct paucity

小葉間胆管減少症

WordNet

1. a continuous tube formed by a row of elongated cells lacking intervening end walls
2. an enclosed conduit for a fluid
3. 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
4. a digestive juice secreted by the liver and stored in the gallbladder; aids in the digestion of fats (同)gall
5. between lobes or lobules

PrepTutorEJDIC

1. 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
2. (…を)少量,少数…欠乏《+『of』+『名』》
3. 胆汁(たんじゅう) / かんしゃく;不きげん

UpToDate Contents

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• 1. 肝内胆管の消失および胆管消失症候群 hepatic ductopenia and vanishing bile duct syndrome
• 2. 総胆管損傷の修復 repair of common bile duct injuries
• 3. 総胆管診査 common bile duct exploration
• 4. 小児における胆道疾患のERCP ercp for biliary disease in children
• 5. 腹腔鏡下胆嚢摘出術の合併症 complications of laparoscopic cholecystectomy

English Journal

• Characteristics and Outcome of Liver Transplantation in Children with Alagille Syndrome: A Single-Center Experience.

• Lee CN, Tiao MM, Chen HJ, Concejero A, Chen CL, Huang YH.Author information Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan; Department of Pediatrics, Zuoying Branch of Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.AbstractBACKGROUND: This study was carried out in Chang Gung Memorial Hospital-Kaohsiung Medical Center, Taiwan, with the aim of reviewing the characteristics and the outcome of liver transplantation (LT) in children with Alagille syndrome (AGS).
• Pediatrics and neonatology.Pediatr Neonatol.2013 Oct 7. pii: S1875-9572(13)00152-6. doi: 10.1016/j.pedneo.2013.09.001. [Epub ahead of print]
• BACKGROUND: This study was carried out in Chang Gung Memorial Hospital-Kaohsiung Medical Center, Taiwan, with the aim of reviewing the characteristics and the outcome of liver transplantation (LT) in children with Alagille syndrome (AGS).METHODS: We performed a retrospective analysis of transplant r
• PMID 24113225

• An unusual case of trisomy 18 associated with paucity of bile ducts.

• Kahramaner Z, Erdemir A, Cosar H, Turkoglu E, Sutcuoglu S, Turelik O, Cumurcu S, Bayol U, Ozer E.Author information Department of Pediatrics, Division of Neonatology, Ministry of Health, Izmir Tepecik Research and Teaching Hospital, Turkey.AbstractA case of neonatal cholestasis associated with Trisomy 18 (Edward's syndrome) is presented. A 3-day-old boy was referred to our clinic due to respiratory distress, elevated serum direct bilirubin levels, a systolic heart murmur, growth restriction and micrognathia. Liver biopsy and chromosomal analysis revealed paucity of intrahepatic bile ducts and Trisomy 18. Extrahepatic biliary atresia was reported in only a few patients with Trisomy 18. To our knowledge, we described for the first time a patient with Trisomy 18 and neonatal cholestasis associated with paucity of interlobular bile ducts.
• Fetal and pediatric pathology.Fetal Pediatr Pathol.2013 Oct;32(5):337-40. doi: 10.3109/15513815.2013.768736. Epub 2013 Feb 19.
• A case of neonatal cholestasis associated with Trisomy 18 (Edward's syndrome) is presented. A 3-day-old boy was referred to our clinic due to respiratory distress, elevated serum direct bilirubin levels, a systolic heart murmur, growth restriction and micrognathia. Liver biopsy and chromosomal analy
• PMID 23421547

• Alagille syndrome: Experience of a tertiary care center in North India.

• Srivastava A, Goel D, Bolia R, Poddar U, Yachha SK.Author information Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, 226 014, India.AbstractAlagille syndrome (AGS) is an autosomal dominant disorder of chronic cholestasis characterized by paucity of interlobular bile ducts. The condition has been described only as isolated case reports in India. We describe clinical profile and outcome of nine subjects (six infants and three older children) with AGS. Cholestasis and characteristic facies were present in all, followed by congenital heart disease, vertebral anomalies, and posterior embryotoxon in seven, five, and four cases, respectively. Pruritus was the commonest symptom which was refractory to medical treatment in one third of cases. Two cases developed decompensated liver disease on follow up. High index of suspicion for this multisystemic condition is essential for correct diagnosis and management.
• Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology.Indian J Gastroenterol.2013 Sep 6. [Epub ahead of print]
• Alagille syndrome (AGS) is an autosomal dominant disorder of chronic cholestasis characterized by paucity of interlobular bile ducts. The condition has been described only as isolated case reports in India. We describe clinical profile and outcome of nine subjects (six infants and three older childr
• PMID 24222371

Japanese Journal

• 肝内胆管減少症,進行性家族性肝内胆汁うっ滞症 (小児の治療指針) -- (消化器)

• 小児科診療 73(-), 602-604, 2010
• NAID 40017118235

• アラジール症候群と診断され長期経過観察中の患者に発生した肝細胞癌の1例

• 日本消化器外科学会雑誌 41(10), 1803-1808, 2008-10-01
• NAID 110006980015

• アラジール症候群と診断され長期経過観察中の患者に発生した肝細胞癌の１例

• 日本消化器外科学会雑誌 41(10), 1803-1808, 2008
• NAID 130004344771

Related Links

• Paucity of interlobular bile ducts.

1. Semin Diagn Pathol. 1992 Feb;9(1):24-30. Paucity of interlobular bile ducts. Hadchouel M(1). Author information: (1)U 347 Inserm, Le Kremlin-Bicêtre, France. Paucity of interlobular bile ducts (PIBD) is ...

• Bile duct paucity ("intrahepatic atresia").

1. Perspect Pediatr Pathol. 1982;7:185-201. Bile duct paucity ("intrahepatic atresia"). Witzleben CL. Reduction of the number of interlobular bile ducts (BDP) has potentially significant physiologic consequences. Most of ...

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★リンクテーブル★

リンク元	「小葉間胆管減少症」
関連記事	「paucity」「duct」「pauci」

「小葉間胆管減少症」

　　[★]

英

paucity of interlobular bile duct, interlobular bile duct paucity

関

小葉間胆管、アラジール症候群 Alagille症候群

「paucity」

　　[★]

• n.

• 少量、少数、不足

関

dearth、deficiency、deficient、deficit、depletion、deprivation、deprive、in short、insufficiency、insufficient、lack、scarce、scarcity、short、shortage、shortness、starve

　

「duct」

　　[★]

• n.

• 管、(植物)導管

関

canal、conduit、ductal、ductus、meatus、pipe、tubal、tube、vas、vessel

　 　 　 　

「pauci」

　　[★]

• comb form.

• 少数の、少しの。(latin paucus = "few")