McGowan Institute for Regenerative Medicine faculty member George Mazariegos, MD, is director of Pediatric Transplantation at Children’s Hospital of Pittsburgh of UPMC and the Thomas E. Starzl Transplantation Institute. Dr. Mazariegos is leading clinical efforts to evaluate lowering or withdrawal of immunosuppression in long-term liver transplant recipients in order to maximize quality of life and minimize post-transplant complications. In collaboration with colleagues at the Thomas E. Starzl Transplantation Institute, Dr. Mazariegos is conducting analysis of immunologic characteristics of patients successfully withdrawn from immunosuppression. His team is attempting to develop a “tolerance assay” that can be used to prospectively characterize patients who may be candidates for less immunosuppression.
As reported in the Pittsburgh Post-Gazette, Jared Burk is one such long-term liver transplant recipient. The May 8, 1998, surgery performed by Dr. Mazariegos corrected a rare genetic enzyme deficiency that affected Jared’s liver's ability to detoxify ammonia from the blood, which twice almost killed him. Today, Jared has no restrictions—he attends school, goes to Scouts, collects rocks, plays Nintendo, enjoys his pet Labrador retriever, and participates in the annual U.S. Transplant Games in Pittsburgh.
“I think in many ways Jared is typical of the far majority of kids [who are living good quality lives with liver transplants]," said Dr. Mazariegos. “He is a great example of how kids are doing so well and getting back to normal life, more routinely than ever before."
At Children's, Dr. Mazariegos said, "well over 90 percent" of liver transplant patients have reached 10-year survival and many of them have gone beyond. An early patient, he said, "is 27 years out after transplant and off drugs with normal liver function. We have kids who have become moms; several of our patients have healthy children."
The surgeon said "a combination" of improved surgical techniques and immunosuppresant, or anti-rejection, drugs, has contributed to the impressive survival rate. So does the fact, he said, that Children's patients are monitored yearly long after transplantation.
Still, Jared is "more emblematic of how good patients are doing throughout the program. In terms of [drugs] he's among the best," Dr. Mazariegos added.
Dr. Mazariegos is an active pediatric liver transplant and pediatric intestinal transplant surgeon. His main clinical interests are focused on the care of pediatric patients who have liver and/or intestinal disease requiring transplantation. He also specializes in treatment of adult and pediatric patients who develop acute liver failure and who may require specialized liver support therapies until they recover or until donor organs are available.
Once a child is diagnosed with liver disease it is important to make a thorough assessment to determine the severity of the liver disease and its projected outcome. Decisions for management of these patients are made by a multidisciplinary team to examine all options of therapy and intervention. Liver disease symptoms and findings that may indicate the need for a liver transplantation are:
- Failure of synthetic function (decrease albumin, elevated clotting times, etc.)
- Portal hypertension with bleeding (variceal bleeding)
- Severe hypersplenism along with liver dysfunction (decreased platelet count)
- Unacceptable quality of life (itching, poor growth, failure to thrive)
- Recurrent cholangitis
- Encephalopathy (elevated ammonia levels in the blood)
- Progressive jaundice
Through its success, liver transplantation is now an option not only for patients with life-threatening liver disease but for those with life-disabling complications of pediatric liver disease.
Illustration: McGowan Institute for Regenerative Medicine.
Pittsburgh Post-Gazette (06/18/08)
Dr. Mazariegos biography, Children’s Hospital of Pittsburgh of UPMC
Children’s Hospital of Pittsburgh of UPMC Liver Disease Symptoms in Children
UPMC Thomas E. Starzl Transplantation Institute
U.S. Transplant Games, Pittsburgh, PA, 07/11-16/08