Guang-Peng Zhou, PhD
Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University; Clinical Center for Pediatric Liver Transplantation, Capital Medical University, Beijing, China. Email: guangpeng_zhou@ccmu.edu.cn
Dr. Guang-Peng Zhou is now a clinical postdoctoral fellow and a resident at the Liver Transplantation Center of Beijing Friendship Hospital, Capital Medical University. Since 2015, he has been under the tutelage of Professor Zhi-Jun Zhu, a famous liver transplant surgeon in mainland China, and has participated in over 100 cases of liver transplantation, including living donor liver transplantation and auxiliary liver transplantation. Under the leadership of Professor Zhi-Jun Zhu, their team completed the first case of cross-auxiliary double domino donor liver transplantation in 2013 and the first case of cross-auxiliary domino liver transplantation by exchange of partial liver between two patients with hypermethioninemia and OTCD in 2018. "Liver transplantation without donation" can be achieved by this way. He is currently engaged in clinical and basic research related to liver transplantation and hepatocyte transplantation for the treatment of inherited metabolic liver diseases. He has published several scientific research papers in international journals as the first or co-first author and participated in international conferences including IPTA and ILTS as oral or poster abstract presenter for several times. He obtained “Young Investigator Awards” in 2022 Joint International Congress of ILTS, ELITA, & LICAGE.
Successful reversal of propionic acidemia associated cardiomyopathy after pediatric living donor liver transplantation: a case-based review
Guang-Peng Zhou1,3, Zhi-Gui Zeng1,3, Lin Wei1,3, Wei Qu1,3, Ying Liu1,2,3, Yu-Le Tan1,3, Jun Wang1,3, Li-Ying Sun1,2,3, Zhi-Jun Zhu1,3.
1Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 2Department of Critical Liver Diseases, Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China; 3Clinical Center for Pediatric Liver Transplantation, Capital Medical University, Beijing, People's Republic of China
Introduction: Propionic acidemia (PA) is a rare mitochondrial metabolic disorder involving multiple organs throughout the body. Since adherence to recommended conservative metabolic management cannot reliably prevent disease progression, liver transplantation (LT) is emerging as a viable alternative therapeutic option in selected PA patients. However, therapeutic outcomes of LT for PA associated cardiomyopathy are rarely reported.
Method: A thorough review of the current published literature was performed to evaluate outcomes of LT for patients with PA-related cardiomyopathy. Clinical, biochemical, and neurophysiological outcomes were compared with the 2-year-old female child managed at our institution.
Results: The pretransplant echocardiogram showed left ventricular dilatation and systolic dysfunction, and thus dilated cardiomyopathy was considered. A living donor liver transplant was performed using the mother's left lateral lobe. On the 35.8 months postoperatively, the child was on a liberated protein diet, but still required levocarnitine supplementation. The hepatic and cardiac function were both normal, but growth retardation was still present. During the follow-up period, there were no further propionic acidemia-related complications, such as metabolic decompensation, or any transplant-related complications. Published literature revealed that the cardioprotective potential of LT for individuals with PA has been proved by the fact that reversal of cardiomyopathy was achieved in all previously reported 11 pediatric patients with pre-existing PA-associated cardiomyopathy. In line with previous results, our patient with mild dilated cardiomyopathy also displayed a complete recovery of cardiac function after LT.
Conclusion: This case report and systematic review demonstrates that LT can successfully treat patients with PA-related cardiomyopathy, which relieves strict protein restriction, provides systemic metabolic stability, improves quality of life, and reverses cardiomyopathy. Nevertheless, recipients with PA remain at risk of developing PA-related complications including cardiomyopathy.
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