ABSTRACT
Objective:To explore the clinical efficacy of liver transplantation for Wilson's disease(WD).Methods:From January 1999 to November 2021, clinical data were retrospectively reviewed for 16 recipients with WD undergoing liver transplantation.There were 9 males and 7 females with an age range of 29.5(14~54)years.They were followed up by telephone, outpatient services and hospitalization.The starting point of follow-up was operation date.And recipient death was an endpoint.Postoperative survival, improvement of neuropsychiatric symptom, changes of corneal K-F ring, altered levels of liver function and serum copper-protein at Month 1 post-operation were observed.The follow-up deadline was November 24, 2021.Results:15 recipients underwent classical orthotopic liver transplantation and the other one recipient underwent living-related liver transplantation.No perioperative deaths occurred.All 16 recipients were followed up for 122(6~260)months.The 1-, 5-, and 10-year survival rates were 93.8%、85.2%and 75.8%, respectively.Among 10 recipients with corneal K-F ring positive with varying degrees after operation and was disappeared in 2 recipients at 7 and 11 months.Among 5 recipients with neuropsychiatric manifestation, 4 recipients showed ameliorative neuropsychic symptoms with varying degrees after operation and 1 recipient died.All the levels of liver function and serum copper-protien of all recipients recovered obviously in 1 month and the 1-, 5-, and 10-year post-operation.Conclusions:Classical orthotopic liver transplantation and living-related liver transplantation not only effectively improves copper metabolism of patient with WD and relieves their severe neurological manifestation, but also improves their life and prolongs survival, which is worthy of clinical promotion.
ABSTRACT
There are still differences in the surgical treatment of children with pulmonary atresia(PA-VSD) with ventricular septal defect. The high rate of postoperative complications and reoperation intervention is still a problem that plagues surgeons. There are few reports on the understanding of the anatomy and physiological functions of MAPCAs. This article systematically reviews and analyzes domestic and foreign literature, and reviews the anatomical classification, surgical management strategies and related complications of PA-VSD patients.
ABSTRACT
Objective To study the effects of ischemia-reperfusion in liver transplantation on the pathophysiological changes of the lung and mechanisms of lung injury. Methods We studied 23 patients who received liver transplantation at Fuzhou General Hospital of PLA. We cut a small piece of the right lung for pathological study and for L-1β and TNF-a immunohistochemistry studies at 5 minutes after the beginning of operation (Ta), 5 minutes before the portal vein was opened (Tb) and three hours after the new liver was transplanted (Tc). We also collected peripheral blood to study the concentration of IL-1β and TNF-a in the plasma at the beginning of operation (T1), the portal vein 5 minutes before opening, the portal vein (T2) ten minutes after the opening (T3) , and one hour after the new liver was transplanted (T4), three hours after the new liver was transplanted (T5), and 12 hours after operation (T6). Results The cytokines TNF-a and IL-1β in peripheral blood were not obviously increased in the portal vein before it was opened, but were significantly increased after the portal vein was opened. Comparison of T1 and T2 separately with T3, T4 and T5 showed significant differences (P<0. 01). In light and electron microscopy, the structures of the lung tissues were normal at Ta and did not change significantly at Tb. There were significant abnormalities at Tc. The average positive points of TNF-a and IL-1β expressions in the lung tissues at Tc were significantly higher than Ta and Tb(P<0. 01). Conclusion Ischemia-reperfusion in liver transplantation led to a serious systemic inflammatory syndrome,and acute lung injury. TNF-a and IL-1β were involved in acute lung injury.