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Endoscopic retrograde cholangiopancreatography in adult patients with biliary atresia: PROCESS-compliant case series.

Abstract Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood.
PMID
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Authors

Mayor MeshTerms

Cholangiopancreatography, Endoscopic Retrograde

Keywords
Journal Title medicine
Publication Year Start




PMID- 29718863
OWN - NLM
STAT- MEDLINE
DCOM- 20180508
LR  - 20180508
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 97
IP  - 18
DP  - 2018 May
TI  - Endoscopic retrograde cholangiopancreatography in adult patients with biliary
      atresia: PROCESS-compliant case series.
PG  - e0603
LID - 10.1097/MD.0000000000010603 [doi]
AB  - INTRODUCTION: Biliary atresia is a progressive inflammatory disease of the bile
      duct that eventually results in biliary cirrhosis. It is a rare neonatal disease 
      that mandates treatment within the first 2 years of life in order for the infant 
      to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when
      Kasai portoenterostomy has been performed in a timely manner, progression is
      still inevitable. In fact, the majority of patients require curative liver
      transplantation at a later stage before reaching adulthood. METHODS: Two
      jaundiced biliary atresia patients who have lived well beyond 20 years with their
      native liver after undergoing Kasai portoenterostomy and underwent endoscopic
      retrograde cholangiopancreatography (ERCP) were identified. The data on patients'
      clinical information, procedures performed, and outcomes were retrospectively
      collected by chart review. RESULTS: Presence of a long Roux limb and acute
      angulation from external adhesions along with ductal anomaly from disease itself 
      rendered ERCP challenging, and intraoperative ERCP had to be performed in 1
      patient. As enteroscopes had to be used, availability of accessory devices was
      limited. CONCLUSION: Management of adult biliary atresia patients with biliary
      obstruction with ERCP is feasible, at times, through multidisciplinary means.
FAU - Hyun, Jong Jin
AU  - Hyun JJ
AD  - Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA.
AD  - Department of Internal Medicine, Korea University College of Medicine, Seoul,
      Korea.
FAU - Irani, Shayan S
AU  - Irani SS
AD  - Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA.
FAU - Kozarek, Richard A
AU  - Kozarek RA
AD  - Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
SB  - AIM
SB  - IM
MH  - Adult
MH  - Biliary Atresia/*surgery
MH  - *Cholangiopancreatography, Endoscopic Retrograde
MH  - Female
MH  - Humans
MH  - Male
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - Young Adult
EDAT- 2018/05/03 06:00
MHDA- 2018/05/09 06:00
CRDT- 2018/05/03 06:00
PHST- 2018/05/03 06:00 [entrez]
PHST- 2018/05/03 06:00 [pubmed]
PHST- 2018/05/09 06:00 [medline]
AID - 10.1097/MD.0000000000010603 [doi]
AID - 00005792-201805040-00031 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2018 May;97(18):e0603. doi: 10.1097/MD.0000000000010603.