[109-111] An example of the potential advantage of LDLT over LRLT

[109-111] An example of the potential advantage of LDLT over LRLT would be in recipients with genetic hepatopathies (e.g., Alagille

Inhibitor Library concentration syndrome) when the donor may be an asymptomatic relative and not a good candidate if they share common alleles. To consider LDLT, LT must 1) be the only therapeutic option, or 2) deceased donor LT is not an option, or 3) a deceased donor organ has not become available. Furthermore, for the LDLT to be ethically appropriate, the likelihood the recipient will survive following LDLT should be high, the mortality risk to the donor low, and the donor is well informed of the risks to his/her short- and long-term health.[108] Considerable pressure is placed on the potential donor from both internal and external sources to save the life of a child or relative. These pressures should be addressed throughout the donor evaluation process to ensure the donor’s “free will” to proceed with liver donation

and have the ability to confidentially remove him or herself from consideration at any time. Consideration of LDLT for a child with acute liver failure has raised concerns Ganetespib that the emergent clinical environment might be coercive to a potential donor and impede honest informed consent. While coercion is difficult to assess, postoperative evaluation of donors have found positive emotional and psychological outcomes regardless of the outcome for the patient.[112] Pediatric patients with acute liver failure who received LDLT had decreased wait times to LT, decreased cold ischemia time, and improved survival compared to a group who received a cadaveric donation.[113] In addition PRKACG to the standard evaluation requirements to assess general health status, surgical risks, volume of the segments to be removed, and evidence of a transmissible virus, the

potential donor will require additional assessments that include psychological assessment and social support systems. If the potential recipient has an inherited metabolic disease, the feasibility of a parent wishing to serve as an LRLT donor should be determined in the context of the child’s genetic condition.[114] LRLT has been successfully performed using heterozygote donors for conditions such as Crigler-Najjar syndrome type 1,[115] Wilson’s disease,[116] carbamoyl-phosphate synthase 1 deficiency,[117] propionic acidemia,[118] arginosuccinic aciduria,[119] progressive familial intrahepatic cholestasis,[120] alpha-1 antitrypsin deficiency,[121] tyrosinemia,[121] Alagille syndrome,[122] and others. In patients with Alagille syndrome receiving LRLT, poor recipient outcomes or technical failure due to bile ducts being too small to utilize are reported if the donor has bile duct hypoplasia.[122] Children receiving an LRLT for arginosuccinic aciduria may still require arginine supplementation during periods of physiological stress or fasting due to persistent deficiency in extrahepatic tissues.[119] 30.

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