studies

820A AASLD ABSTRACTS HEPATOLOGY, October, 2015
Disclosures:
Satheesh Nair - Advisory Committees or Review Panels: Jansen; Grant/Research
Support: Gilead; Speaking and Teaching: Abbvie, Valeant, BMS
Sanjaya K. Satapathy - Advisory Committees or Review Panels: Gilead, Intercept;
Grant/Research Support: Genfit, Gilead, Biotest
The following authors have nothing to disclose: Cheri Ogwo, Jason Vanatta,
Vinaya Rao, Chibuzor Iwelu, James Eason
1234
Long-Term Outcome of Extrahepatic Biliary Atresia into
adult life
Javaid Sadiq 1 , Aditi Kumar 4 , Hifsa Sohail 3 , Carla Lloyd 3 , James
W. Ferguson 4 , Darius Mirza 2 , Khalid Sharif 1 , Gideon Hirschfield 4 ,
Deirdre A. Kelly 3 ; 1 Paediatric Hepatobiliary Transplant Surgery,
Birmingham Children’s Hospital Birmingham UK, Birmingham,
United Kingdom; 2 Liver Surgery, University Hospital Birmingham,
Birmingham, United Kingdom; 3 Liver unit, Birmingham Children’s
Hospital, Birmingham, United Kingdom; 4 Liver unit, University Hospital
Birmingham, Birmingham, United Kingdom
Background:Biliary Atresia(BA) is the single commonest cause
of neonatal cholestasis leading to cirrhosis,portal hypertension
and liver failure and is the main indication for pediatric liver
transplant(LT). Aim:Evaluate the long-term outcome of children
with BA transitioning to adult life Subjects & Methods:Records
of patients of BA managed over a period of 34 years(1980-
2014) at a single institution were retrospectively reviewed
.Patients with more than 10 years of follow-up were included in
the study.Data collection included demographics,age at Kasai
Portoenterostomy(KPE),associated malformations, survival with
native liver or post-LT,mortality, current education/work/marital/family
status. Results: 493 BA patients were managed
during this period(260 F & 233 M).Median age at kasai was
53 days(range:7-183 days). 92 % had isolated BA while 8 %
had BA polysplenia malformation syndrome.332 patients were
included in this study (1980 – 2004). 11 patients were lost to
follow-up. Median patient survival is 17.3 yrs(0.32- 34.6) &
median survival with native liver is 2.25 yrs(0.07-34.6). 53
patients(16.5%) died in pediatric care; 26 with their native
livers & 27 after LT.135 patients(50.3%) are still in pediatric
care(Group A).57 are surviving with their native liver(A1)
while 78 children have been transplanted(A2).7 patients are
awaiting transplant in Group A1.133(49.6 %) patients were
transferred to adult services(Group B); 49 with native livers(B1)
and 84 after LT(B2). 28 patients in group B1 had portal hypertension(PH);20
treated with beta blockers,esophageal banding
or shunts. 9 patients transferred to adult services with native
liver(B1) subsequently required LT & 7 are listed for LT due to
decompensated liver disease.6 patients in group B2 required
retransplant. After transfer to adult care, 3 patients in Group B1
died( one due to ruptured splenic aneurysm & 2 due to decompensated
liver disease) while 5 patients in Group B2 died from
post-transplant lymphoproliferative disorders(PTLD),Hepatopulmonary
syndrome, ruptured psoas cyst and bleeding & chronic
rejection).Out of 268 patients in this series, majority participated
in normal school education while 32(12 %) required
special needs support. 29 transferred went to university, 18
obtained non-vocational qualifications and 33 joined various
training courses. Conclusion: Improved medical and surgical
techniques have improved the outcome and quality of life for
patients with BA, allowing them to live into adult life, complete
their education & function as useful members of the society
Disclosures:
James W. Ferguson - Advisory Committees or Review Panels: Astellas, Novartis
Gideon Hirschfield - Advisory Committees or Review Panels: Intercept Pharma;
Consulting: Dignity Sciences, GSK, NGM Bio, Lumena, J & J; Grant/Research
Support: BioTie; Speaking and Teaching: Falk Pharma
Deirdre A. Kelly - Consulting: Sanofi Pasteur; Grant/Research Support: BMS,
Astellas, Acitllion, MSD, Roche
The following authors have nothing to disclose: Javaid Sadiq, Aditi Kumar, Hifsa
Sohail, Carla Lloyd, Darius Mirza, Khalid Sharif
1235
Assessment of Energy Metabolism in Liver Transplant
Recipients
Ajay Singhvi 1 , H. Steven Sadowsky 2 , Ayelet Cohen 1 , Alysen
Demzik 1 , Mary E. Rinella 1 , Lisa B. VanWagner 1 , Josh Levitsky 1 ;
1 Division of Gastroenterology & Hepatology, Northwestern Memorial
Hospital, Chicago, IL; 2 Physical Therapy & Human Movement
Sciences, Feinberg School of Medicine, Chicago, IL
Background: The reasons for accelerated weight gain and metabolic
syndrome following liver transplantation (LT) are not
well understood. We hypothesized that this may be related
to ineffective metabolic rates compared to healthy patients,
particularly during exercise. The purpose of this pilot study was
to assess energy expenditure during exercise in LT recipients.
Methods: We consented ten subjects who were transplanted for
NASH or cryptogenic cirrhosis (>1 year post) to undergo analysis
of body composition, resting energy expenditure (REE), and
peak oxygen uptake (VO 2 max
). The latter was conducted using
a ramped-Bruce protocol. VO 2 max
was assessed by standard
parameters, including respiratory exchange ratio and a rating
of perceived exertion. Predicted VO 2
was determined using the
Wasserman-Hansen equations and REE was determined using
the Harris-Benedict equation. Measured VO 2 max
values were
then compared to data from the NHANES database for ageand
sex-matched healthy individuals. Results: Subjects (50%
male) were mean age 60.1±4.3 years, BMI 30.7±3.22 kg/
m 2 , time post-LT 5.2±2.6 years. Average percent body fat was
22.6±8.4% in males and 28.5±2.9% in females. Mean REE
was 97.3% of predicted REE in males and 77.9% of predicted
REE in females. Average VO 2 max
(mL/kg/min) in LT males was
16.9±5.0 compared to a predicted value of 21.0±3.1 (80.4%
of predicted VO 2 max
, p=0.04). LT females had a mean VO 2
max
(mL/kg/min) of 15.1±2.2 compared to a mean predicted
value of 20.1±1.1 (75.4% of predicted VO 2 max
, p=0.004).
These values were lower than those of historical healthy controls
who had a mean VO 2 max
(mL/kg/min) of 31 in males
and 23 in females (Figure 1). Discussion: Our results suggest
that LT recipients, particularly females, have lower resting and
exercise energy expenditure compared to predicted values and
healthy controls. This may contribute to post-LT weight gain and
inability to lose adequate weight with diet and exercise. Further
study is necessary to investigate the pathophysiology of this
phenomenon, the metabolic change from pre- to post-LT, and