raffinose and alpha-1-Antitrypsin-Deficiency

Appropriate hypothermic packaging techniques are an essential part of organ procurement. We present a case in which deviation from standard packaging practice may have caused sub-zero storage temperatures during transport, resulting in a clinical picture resembling PNF. An 18-month-old male with alpha-1-antitrypsin deficiency underwent liver transplant from a size-matched pediatric donor. Upon arrival at the recipient hospital, ice crystals were noted in the UW solution. The transplant proceeded uneventfully with short ischemia times. Surprisingly, transaminases, INR, and total bilirubin were markedly elevated in the postoperative period but returned to near normal by discharge. Follow-up of over five yr has demonstrated normal liver function. Upon review, it was discovered that organ packaging during recovery included storage in the first bag with only 400 mL of UW solution, and pure ice in the second bag instead of slush. This suggests that the postoperative delayed graft function was related to sub-zero storage of the graft during transport. This is the first report of sub-zero cold injury, or frostbite, following inappropriate packaging of an otherwise healthy donor liver. The clinical picture closely resembled PNF, perhaps implicating this mechanism in other unexpected cases of graft non-function.

Topics: Adenosine; Allopurinol; alpha 1-Antitrypsin Deficiency; Bilirubin; Cold Temperature; Delayed Graft Function; Frostbite; Glutathione; Humans; Ice; Infant; Insulin; International Normalized Ratio; Ischemia; Liver Transplantation; Male; Organ Preservation Solutions; Postoperative Period; Raffinose; Tissue and Organ Procurement; Treatment Outcome