Abstract:
Objective:
To sum the experience of liver transplantation from pediatric cardiac death
donated (DCD) in the treatment of children with glycogen storage disease.
Methods: The donor was a 3 years and 8 months old boy, who was brain death of
severe viral encephalitis and secondary to cardiac death, implementation of
organ donation; the recipient was a 4 years and 2 months old boy with a body
weight of 15 kg and growth retardation, who suffered from glycogen storage disease,
secondary to liver dysfunction, severe hypoglycemia. The surgical was
classically orthotopically liver transplantation, the warm ischemia time was 6-min,
the cold ischemia time was 180-min. T-tube and gastrojejunostomy fistula was
placed during operation. The vital signs and transplanted liver function of the
recipient were monitored after operation, and the recipient was given
treatments of anti-infection, improving microcirculation and nutritional
support. The recipient was treated with immunosuppression protocol of
tacrolimus and prednisone to prevent rejection. Results: The operation time of
the recipient was 420-min, the anhepatic phase was 55-min, and the blood loss
was 400 ml. The recipient’s blood sugar became normal at 2-h, the endotracheal
intubation was removed at 6-h, Enteral nutrition was given through
gastrojejunostomy fistula at 24-h, the liver function returned to normal and
the recipient began to get out of bed at 7 days, the recipient was discharged
on the 18th day. The child grew fast postoperation after 1 month, and went to
kindergarten after 6-month. The recipient has healthy survival of 18 months to
present. Conclusion: The pediatric DCD liver is a better graft for pediatric
liver transplantation, which could gain a good result of transplantation. Liver
transplantation is a good way to treat a child with glycogen storage disease.