Domino liver transplantation using a graft from a donor with familial hypercholesterolemia: seven‐yr follow‐up

Abstract

A 46-yr-old female with hepatocellular carcinoma and severe hepatitis B-related liver cirrhosis received a domino liver graft from a 25-yr-old female with homozygous familial hypercholesterolemia (HFHC) in September 2001. Hypercholesterolemia occurred in the graft recipient within one yr after transplantation and was partially controlled by atorvastatin. Three yr after transplantation, an autologous CD34(+) cell transplantation was performed in order to better control the hypercholesterolemia. Only preliminary results of this domino liver transplantation (DLT) were published in 2003, without a long-term analysis of the hypercholesterolemic effects in recipient. Subsequent to DLT, the average plasma cholesterol level in the domino donor rapidly normalized and seven yr after had a value of 182 mg/dL. After seven-yr follow-up, the domino recipient has no hepatocarcinoma recurrence. Moreover, no signs of cardiovascular or atherosclerotic lesions were noted despite an elevated plasma cholesterol level (339 mg/dL after seven yr of follow-up) resistant to drug therapy and stem cell autotransplantation. In conclusion, DLT using a liver graft from a patient with HFHC provides a viable option for marginal recipients.

Domino liver transplantation for select metabolic disorders: Expanding the living donor pool

“…None had evidence of autonomic dysfunction. Similar concerns exist with DLT with livers form patients with primary hyperoxaluria, acute intermittent porphyria, and familial homozygous hypercholesterolemia since the liver is the main source of the production of the abnormal metabolites in these metabolic disorders …”

“…These procedures are pursued with the prospect that DLT recipients will not develop clinical or subclinical manifestation of enzyme defect associated with the donor liver. However, there are several reports that suggest DLT allografts can transmit the primary disease in the post‐DLT period . Several DLT have been performed using livers from MSUD patients.…”

Fatal cardiac atherosclerosis in a child 10 years after liver transplantation: A case report and a review

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Hence, this is the first report of a child succumbing to severe cardiovascular atherosclerosis 10 years after successful LTx for homozygous hypercholesterolemia.…”

Genetic, hematological, and immunological disorders transmissible with liver transplantation

“…Conversely, liver allografts can transfer these defects . Given the need to use innovative approaches to increase the number of potential donor organs as well as the narrow window of time between the identification of a potential donor and viable organ procurement during which the donor's history can be investigated, marginal donors, living donor liver grafts, split‐liver grafts from deceased donors, and domino liver grafts from patients with metabolic liver disease are often used . This increases the risk of metabolic disease transmission from donor to recipient.…”