Organ Transplantation Research Today is a free monthly online journal that collates and summarizes the latest research about Organ Transplantation, including details on risks, prognosis, procedure, surgery.

Cryopreserved liver cell transplantation controls ornithine transcarbamylase deficient patient while awaiting liver transplantation.

Stéphenne X, Najimi M, Smets F, Reding R, de Ville de Goyet J, Sokal EM

Pediatric Research Laboratory and Pediatric Liver Unit, Université Catholique de Louvain, Cliniques St. Luc, Brussels, Belgium.

Liver cell transplantation was performed in a child with urea cycle disorder poorly equilibrated by conventional therapy as a bridge to transplantation. A 14-month-old boy with ornithine transcarbamylase (OTC) deficiency received 0.24 billion viable cryopreserved cells/kg over 16 weeks. Tacrolimus and steroids were given as immunosuppressive treatment while the patient was kept on the pre-cell transplant therapy. Mean blood ammonia level decreased significantly following the seven first infusions, while urea levels started to increase from undetectable values. After those seven infusions, an ammonium peak up to 263 microg/dL, clinically well tolerated, was observed. Interestingly, blood urea levels increased continuously to reach 25 mg/dL, after the last three infusions. Eventually, he benefited from elective orthotopic liver transplantation (OLT) and the post-surgical course was uneventful. We conclude that use of cryopreserved cells allowed short- to medium-term metabolic control and urea synthesis in this male OTC-deficient patient while waiting for OLT.

Published 5 July 2005 in Am J Transplant, 5(8): 2058-61.
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