Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review

Teru Kumagi; Joost P H Drenth; Orlee Guttman; Vicky Ng; Les Lilly; George Therapondos; Yoichi Hiasa; Kojiro Michitaka; Morikazu Onji; Yuji Watanabe; Sambit Sen; William Griffiths; Eve Roberts; Jenny Heathcote; Gideon M Hirschfield

doi:10.1111/j.1478-3231.2011.02668.x

Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review

Teru Kumagi, Joost P H Drenth, Orlee Guttman, Vicky Ng, Les Lilly, George Therapondos, Yoichi Hiasa, Kojiro Michitaka, Morikazu Onji, Yuji Watanabe, Sambit Sen, William Griffiths, Eve Roberts, Jenny Heathcote, Gideon M Hirschfield

Research output: Contribution to journal › Article › peer-review

26 Citations (Scopus)

Abstract

BACKGROUND: Biliary atresia is a progressive biliary injury which occurs only in infants.

AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood.

METHODS: A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation.

RESULTS: Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up.

CONCLUSIONS: Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.

Original language	English
Pages (from-to)	510-8
Number of pages	9
Journal	Liver International
Volume	32
Issue number	3
DOIs	https://doi.org/10.1111/j.1478-3231.2011.02668.x
Publication status	Published - Mar 2012

Keywords

Adolescent
Adult
Bile Ducts, Intrahepatic
Biliary Atresia
Cholangiopancreatography, Magnetic Resonance
Cholangitis, Sclerosing
Cholestasis
Female
Humans
Hypertension, Portal
Male
Middle Aged
Portoenterostomy, Hepatic
Survivors
Treatment Outcome

Access to Document

10.1111/j.1478-3231.2011.02668.x

Cite this

Kumagi, T., Drenth, J. P. H., Guttman, O., Ng, V., Lilly, L., Therapondos, G., Hiasa, Y., Michitaka, K., Onji, M., Watanabe, Y., Sen, S., Griffiths, W., Roberts, E., Heathcote, J., & Hirschfield, G. M. (2012). Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review. Liver International, 32(3), 510-8. https://doi.org/10.1111/j.1478-3231.2011.02668.x

@article{e8dce8d83ed04484a825a633f356b854,

title = "Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review",

abstract = "BACKGROUND: Biliary atresia is a progressive biliary injury which occurs only in infants.AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood.METHODS: A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation.RESULTS: Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up.CONCLUSIONS: Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.",

keywords = "Adolescent, Adult, Bile Ducts, Intrahepatic, Biliary Atresia, Cholangiopancreatography, Magnetic Resonance, Cholangitis, Sclerosing, Cholestasis, Female, Humans, Hypertension, Portal, Male, Middle Aged, Portoenterostomy, Hepatic, Survivors, Treatment Outcome",

author = "Teru Kumagi and Drenth, {Joost P H} and Orlee Guttman and Vicky Ng and Les Lilly and George Therapondos and Yoichi Hiasa and Kojiro Michitaka and Morikazu Onji and Yuji Watanabe and Sambit Sen and William Griffiths and Eve Roberts and Jenny Heathcote and Hirschfield, {Gideon M}",

note = "{\textcopyright} 2011 John Wiley & Sons A/S.",

year = "2012",

month = mar,

doi = "10.1111/j.1478-3231.2011.02668.x",

language = "English",

volume = "32",

pages = "510--8",

journal = "Liver International",

issn = "1478-3223",

publisher = "Wiley",

number = "3",

}

Kumagi, T, Drenth, JPH, Guttman, O, Ng, V, Lilly, L, Therapondos, G, Hiasa, Y, Michitaka, K, Onji, M, Watanabe, Y, Sen, S, Griffiths, W, Roberts, E, Heathcote, J & Hirschfield, GM 2012, 'Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review', Liver International, vol. 32, no. 3, pp. 510-8. https://doi.org/10.1111/j.1478-3231.2011.02668.x

TY - JOUR

T1 - Biliary atresia and survival into adulthood without transplantation

T2 - a collaborative multicentre clinic review

AU - Kumagi, Teru

AU - Drenth, Joost P H

AU - Guttman, Orlee

AU - Ng, Vicky

AU - Lilly, Les

AU - Therapondos, George

AU - Hiasa, Yoichi

AU - Michitaka, Kojiro

AU - Onji, Morikazu

AU - Watanabe, Yuji

AU - Sen, Sambit

AU - Griffiths, William

AU - Roberts, Eve

AU - Heathcote, Jenny

AU - Hirschfield, Gideon M

PY - 2012/3

Y1 - 2012/3

N2 - BACKGROUND: Biliary atresia is a progressive biliary injury which occurs only in infants.AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood.METHODS: A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation.RESULTS: Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up.CONCLUSIONS: Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.

AB - BACKGROUND: Biliary atresia is a progressive biliary injury which occurs only in infants.AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood.METHODS: A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation.RESULTS: Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up.CONCLUSIONS: Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.

KW - Adolescent

KW - Adult

KW - Bile Ducts, Intrahepatic

KW - Biliary Atresia

KW - Cholangiopancreatography, Magnetic Resonance

KW - Cholangitis, Sclerosing

KW - Cholestasis

KW - Female

KW - Humans

KW - Hypertension, Portal

KW - Male

KW - Middle Aged

KW - Portoenterostomy, Hepatic

KW - Survivors

KW - Treatment Outcome

UR - http://www.ncbi.nlm.nih.gov/pubmed/22098694

U2 - 10.1111/j.1478-3231.2011.02668.x

DO - 10.1111/j.1478-3231.2011.02668.x

M3 - Article

C2 - 22098694

SN - 1478-3223

VL - 32

SP - 510

EP - 518

JO - Liver International

JF - Liver International

IS - 3

ER -

Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review

Abstract

Keywords

Access to Document

Fingerprint

Cite this