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2016 ; 20
(6
): 756-69
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gab.com Text
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English Wikipedia
Liver transplantation for pediatric inherited metabolic disorders: Considerations
for indications, complications, and perioperative management
#MMPMID27329540
Oishi K
; Arnon R
; Wasserstein MP
; Diaz GA
Pediatr Transplant
2016[Sep]; 20
(6
): 756-69
PMID27329540
show ga
LT is an effective therapeutic option for a variety of IEM. This approach can
significantly improve the quality of life of patients who suffer from severe
disease manifestations and/or life-threatening metabolic decompensations despite
medical/dietary management. Due to the significant risks for systemic
complications from surgical stressors, careful perioperative management is vital.
Even after LT, some disorders require long-term dietary restriction, medical
management, and monitoring of metabolites. Successful liver transplant for these
complex disorders can be achieved with disease- and patient-specific strategies
using a multidisciplinary approach. In this article, we review indications,
complications, perioperative management, and long-term follow-up recommendations
for IEM that are treatable with LT.