Successful liver transplant for girl with rare disease
Renji Hospital announced a successful liver transplant for a 2-year-old girl with a rare disease, whose family has a history of severe reactions to normal anesthetics.
The girl was diagnosed with ornithine transcarbamylase deficiency, a genetic disorder resulting in urea cycle disorders that can cause brain and liver damage. Because medications failed to control her condition, she began vomiting frequently and had poor consciousness. Her family took her to Renji Hospital in October, where doctors determined a liver transplant could cure her disease.
Doctors had planned to harvest her father's liver for the transplant. However, during a pre-surgery check, they discovered the family has a history of malignant hyperthermia, a rare and inherited muscle abnormality that can be fatal if normal anesthetics are used.
Only a drug called dantrolene sodium can control the condition, which is so rare there are less than 10 cases reported in the entire country every year with a death rate of 71.4 percent.
Due to the risk of using the father's liver for the transplant, doctors decided to use a donated organ instead.
The girl was matched with an organ in January but was unable to receive the surgery because Renji did not have dantrolene sodium in stock — and neither did any other hospital in Shanghai.
However, after contacting hospitals in other provinces, doctors were able to secure 12 doses of the medication.
They tracked down another matching organ three months later and performed the successful transplant on Tuesday.
Doctors said the girl is recovering well.
Doctors from Renji Hospital in surgery.
