BACKGROUND: Crigler-Najjar syndrome type I (CNS I) is a very rare autosomal recessive inherited disease that liver transplantation can properly deal with. METHODS: We present one case of an 18-month-old child with CNS I diagnosed by clinical findings and genetic detecting LTx was performed 5 days after kernicterus broke out and neurological symptoms were successfully reversed. RESULT: Magnetic resonance imaging and magnetic resonance spectroscopy showed encouraging results that brain pathology had a trend to return to normal in 1-year follow-up, combined with electroencephalogram and motor development estimate studies. CONCLUSIONS: Liver transplantation can cure CNS I with reversible neurological symptoms to some extent in time Magnetic resonance spectroscopy may be a future option of predicting brain conditions and selecting suitable patients with CNS I for transplantation.
Zhen-Hua Tu, De-Sheng Shang, Jin-Cai Jiang, Wu Zhang, Min Zhang, Wei-Lin Wang, Hai-Yan Lou,Shu-Sen Zheng Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Key Laboratory of Combined Multi-Organ Transplantation, Ministry of Public Health & Key Laboratory of Organ Transplantation of Zhejiang Province
BACKGROUND: Liver transplantation is so far the most effective therapeutic modality for end-stage liver diseases, but ischemia/reperfusion (I/R) injury represents a critical barrier to liver transplantation. Primary graft dysfunction and small-for-size syndrome are closely associated with I/R injury. Ischemic preconditioning (IPC) is defined as a brief period of liver ischemia followed by reperfusion, and has demonstrated protections against a prolonged I/R injury and improved the capacity of regeneration. The article aimed to review IPC literatures for the understanding of the effects of IPC on I/R injury involving in the procurement of donor liver and protective mechanisms. DATA SOURCES: A literature search of MEDLINE and Web of Science databases using 'liver transplantation', 'liver regeneration', 'hepatectomy', 'ischemia/reperfusion' and 'ischemic preconditioning' was performed, and then a large amount of related data was collected. RESULTS: The literature search provided a huge amount of evidence for the protective effects of IPC on I/R injury in liver transplantation, including reduction of blood loss in hepatectomy, intraoperative hemodynamic stability and its significant role in liver regeneration. The mechanism involves in balancing inflammatory cytokines, enhancing energy status and mitigating microcirculatory disturbance. CONCLUSION: IPC plays an essential role in hepatectomy before and after harvest of living donor liver and implantation of liver graft.
BACKGROUND: The last decade has witnessed great progress in living donor liver transplantation worldwide. However, biliary complications are more common in partial liver transplantation than in whole liver transplantation. This is due to an impaired blood supply of the hilar bile duct during organ procurement and recipient surgery, commonly encountered anatomical variations, a relatively small graft duct, and complicated surgical techniques used in biliary reconstruction. DATA SOURCES: MEDLINE and PubMed were searched for articles on 'living donor liver transplantation', 'biliary complication', 'anatomical variation', 'biliary reconstruction', 'stenting' and related topics. RESULT: In this review, biliary complications were analyzed with respect to anatomical variation, surgical techniques in biliary reconstruction, and protection of the arterial plexus of the hilar bile duct. CONCLUSION: Transecting the donor bile duct at the right place to secure a larger bile duct stump, anastomosing techniques, and stenting methods as well as preserving the blood supply to the bile duct are all important in reducing biliary complications.
Xiao-Ning Feng, Chao-Feng Ding, Mei-Yuan Xing, Min-Xia Cai and Shu-Sen ZhengDivision of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China
