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Atretic gallbladder

MedGen UID:
479575
Concept ID:
C3277945
Finding
Synonym: Gallbladder atresia
 
HPO: HP:0011984

Definition

Failure of formation of the lumen of the gallbladder, often associated with gallbladder hypoplasia. [from HPO]

Term Hierarchy

Conditions with this feature

Extrahepatic biliary duct atresia
MedGen UID:
1621383
Concept ID:
C4520983
Congenital Abnormality
Biliary atresia is a disorder of infants in which there is progressive obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction of bile flow. Untreated, the resulting cholestasis leads to progressive conjugated hyperbilirubinemia, cirrhosis, and hepatic failure (Bates et al., 1998). Most patients require liver transplantation within the first year of life (Leyva-Vega et al., 2010). See also Alagille syndrome (118450), which includes biliary atresia as a feature.

Professional guidelines

PubMed

Huang FC, Hwang KP
Acta Paediatr Taiwan 2006 Jul-Aug;47(4):175-80. PMID: 17180784

Recent clinical studies

Etiology

Goneidy A, Ong EGP
J Pediatr Surg 2018 Feb;53(2):274-276. Epub 2017 Dec 6 doi: 10.1016/j.jpedsurg.2017.11.018. PMID: 29221637
Tang ST, Li SW, Ying Y, Mao YZ, Yong W, Tong QS
J Laparoendosc Adv Surg Tech A 2009 Dec;19(6):827-30. doi: 10.1089/lap.2008.0432. PMID: 19961368
Huang FC, Hwang KP
Acta Paediatr Taiwan 2006 Jul-Aug;47(4):175-80. PMID: 17180784
Molmenti EP, Squires RH, Nagata D, Roden JS, Molmenti H, Fasola CG, Prestidge C, D'Amico L, Casey D, Sanchez EQ, Goldstein RM, Levy MF, Benser M, McPhail W, Andrews W, Andersen JA, Klintmalm GB
Pediatr Transplant 2003 Apr;7(2):93-7. doi: 10.1034/j.1399-3046.2003.00021.x. PMID: 12654048
Hay SA, Soliman HE, Sherif HM, Abdelrahman AH, Kabesh AA, Hamza AF
J Pediatr Surg 2000 Dec;35(12):1706-9. doi: 10.1053/jpsu.2000.19217. PMID: 11101719

Diagnosis

Goneidy A, Ong EGP
J Pediatr Surg 2018 Feb;53(2):274-276. Epub 2017 Dec 6 doi: 10.1016/j.jpedsurg.2017.11.018. PMID: 29221637
Tang ST, Li SW, Ying Y, Mao YZ, Yong W, Tong QS
J Laparoendosc Adv Surg Tech A 2009 Dec;19(6):827-30. doi: 10.1089/lap.2008.0432. PMID: 19961368
Okazaki T, Miyano G, Yamataka A, Kobayashi H, Koga H, Lane GJ, Miyano T
Pediatr Surg Int 2006 Feb;22(2):140-3. Epub 2005 Dec 8 doi: 10.1007/s00383-005-1609-0. PMID: 16341535
Senyüz OF, Yeşildağ E, Emir H, Tekant G, Bozkurt P, Sarimurat N, Söylet Y
J Pediatr Surg 2001 Mar;36(3):463-5. doi: 10.1053/jpsu.2001.21621. PMID: 11226997
Hay SA, Soliman HE, Sherif HM, Abdelrahman AH, Kabesh AA, Hamza AF
J Pediatr Surg 2000 Dec;35(12):1706-9. doi: 10.1053/jpsu.2000.19217. PMID: 11101719

Prognosis

Goneidy A, Ong EGP
J Pediatr Surg 2018 Feb;53(2):274-276. Epub 2017 Dec 6 doi: 10.1016/j.jpedsurg.2017.11.018. PMID: 29221637
Tang ST, Li SW, Ying Y, Mao YZ, Yong W, Tong QS
J Laparoendosc Adv Surg Tech A 2009 Dec;19(6):827-30. doi: 10.1089/lap.2008.0432. PMID: 19961368
Huang FC, Hwang KP
Acta Paediatr Taiwan 2006 Jul-Aug;47(4):175-80. PMID: 17180784

Clinical prediction guides

Tang ST, Li SW, Ying Y, Mao YZ, Yong W, Tong QS
J Laparoendosc Adv Surg Tech A 2009 Dec;19(6):827-30. doi: 10.1089/lap.2008.0432. PMID: 19961368
Huang FC, Hwang KP
Acta Paediatr Taiwan 2006 Jul-Aug;47(4):175-80. PMID: 17180784

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