Трансплантационные технологии и исследования

Our case report is the only reported case in literature of transplanting a black coloured kidney successfully from a albino donor. Oculocutaneous albinism is similar to hermansky–pudlak syndrome and chediak higashi syndrome and could lead to more widespread lysosome excretory defects. These defects could lead to accumulation of some intra cellular material, leading to the gross discolouration of the kidney. Hermansky pudlak syndrome is a triad of albinism, platelets lacking dense bodies and storage of ceroid like material in tissues. The manifestations of storage disease are variable and include granulomatous colitis, restrictive lung disease, kidney failure and cardiomyopathy. The autofluorescent material stored in the hermansky pudlak syndrome contains dolichols. Our patient was a living related donor who was having oculocutaneous albinism. Her kidneys were normal functioning but on exposure was black coloured. Post transplant the graft showed ATN and parenchymal dysfunction which later recovered.

Background: Orthotopic liver transplantation (OLT) is a highly complex procedure and can offer difficult intraoperative control in patients with coagulopathy. The present study aimed to evaluate the profile of coagulation by Rotation thromboelastometry (ROTEM®) in the intraoperative of patients with prothrombotic risk factors submitted to liver transplantation. Methods: A prospective, observational pilot study, in which 24 patients submitted to OLT, of both sexes and age from 18 years, were included in the period from October 2014 to June 2017. Three samples were taken intraoperatively to analyze the profile of coagulation through the thromboelastometry assays (EXTEM, FIBTEM, INTEM and HEPTEM). Results: In the analysis of the tests in the EXTEM (clotting time [CT] and maximum clot firmness [MCF]) and INTEM (MCF), there was hypocoagulation along the OLT, with no statistical difference between the values obtained. In the FIBTEM tests (amplitude in 10 minutes [A10] and MCF),There was reduction in the neohepatic phase (stage III) in relation to the beginning of surgery (stage I), with statistical significance (P=0.0424 and 0.0227, respectively). In the analysis of CT in the INTEM, there was an increase in the stage III in relation to stage I and anhepatic phase (stage II), with statistical significance (P=0.0004 and 0.0012, respectively). The fibrinolytic activity by maximum lysis (ML) was higher in the stage I and stage II in relation to stage III, when analyzed by the EXTEM, presenting statistical significance (P=0.0016 and 0.0035, respectively). Conclusion: In patients with prothrombotic risk factors, data from the ROTEM® analysis showed some statistically significant changes, but we cannot say that it showed tendency to hypocoagulation, when there was not significance in most other tests. Therefore, in the FIBTEM tests the consumption of fibrinogen was more accentuated in stage III in relation to stage I and in relation to stage III, when analyzed by INTEM CT and EXTEM ML, the presence of heparin was higher and fibrinolysis was less pronounced, respectively.