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Developmental Abnormalities of the Bile Duct and Foregut

  • Masaki Iwai
  • Takahiro Mori
  • Wilson M. S. TsuiEmail author
Chapter

Abstract

Extrahepatic biliary atresia, paucity of intrahepatic bile ducts, viral hepatitis, and malformations of bile ducts are the main causes of neonatal cholestasis. Biliary atresia occurs due to inflammation and destruction of extrahepatic bile duct systems in utero and during the perinatal period. Malformations of bile ducts are associated with abnormal remodeling of embryonic bile duct plate and fibrocystic lesions are developed, and they include congenital hepatic fibrosis [1], Caroli’s disease [2], microhamartoma [3], and choledochal cyst, and they carry an increased risk of bile duct carcinoma. Differing from other cystic lesions, ciliated hepatic cyst arising from embryonic foregut is detected incidentally in adults and should be differentiated from bile duct lesions [4]. Infantile and adult types of hereditary polycystic disease cause renal failure, and hepatic failure is rare [5, 6].

Abbreviations

ADPKD

Autosomal dominant polycystic kidney disease

ADPLD

Autosomal dominant polycystic liver disease

ARPKD

Autosomal recessive polycystic kidney disease

CHF

Congenital hepatic fibrosis

Erb-B2

Erythroblastic oncogene B2

Gd-EOB-DTPA-MRI

Gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid magnetic resonance imaging

MEK

MAP (Mitogen-Activated Protein) / ERK (Extracellular Signal-Regulated Kinase) kinase

mTOR

Mechanistic target of rapamycin

SOL

Space-occupying lesion

Srk kinase

S-receptor kinase

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Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Masaki Iwai
    • 1
  • Takahiro Mori
    • 2
  • Wilson M. S. Tsui
    • 3
    Email author
  1. 1.KyotoJapan
  2. 2.HyogoJapan
  3. 3.Hong KongChina

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