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Better preservation and evaluation of kidneys from donors after circulatory death serve to increase the number of kidneys available for transplantation and hypothermic machine perfusion has been shown to decrease ischemia reperfusion injury and delayed graft function. Data on relation between hemodynamic parameters during hypothermic machine perfusion and delayed graft function in kidneys from donors after circulatory death are so far scarce and not univocal. We aimed at assessing whether hemodynamic parameters measured during hypothermic machine perfusion (flow, mean perfusion pressure, and renal resistance) are associated with delayed graft function in 26 kidneys retrieved from uncontrolled donors after circulatory death. In our series, the incidence of delayed graft function was 57.7% (15/26). Recipients who developed delayed graft function had a longer warm ischemic time (p = 0.04). All hemodynamic parameters measured during hypothermic machine perfusion were comparable between recipients with delayed graft function and those without. According to our data, in kidneys from uncontrolled donors after circulatory death, a longer warm ischemic time (that is the overall time of no flow, as the sum of the no-flow and the no-touch period) is associated with delayed graft function. This finding underscores the pivotal role of ischemic injury in terms of absence of flow in affecting graft function. No association was detectable between hemodynamic parameters during hypothermic machine perfusion and the development of delayed graft function in our series. [ABSTRACT FROM AUTHOR]

Purpose: Optical coherence tomography angiography (OCTA) is a non-invasive and objective tool for the evaluation of the retinal microvascular changes in Fabry disease (FD). We investigated changes in retinal vasculature in FD patients, and the possible correlation with systemic parameters, by using OCTA, and reviewed the current status of literature. Methods: Thirteen FD patients (eight females, five males, mean age 49.85 ± 14.7 years) were compared with 13 age- and sex-matched healthy controls. OCTA 3 × 3 mm macular scans were performed in all subjects. We evaluated the vessel density and vessel perfusion in distinct macular areas (whole, inner, and outer) of both the superficial capillary plexus (SCP VD and SCP VP) and of the deep capillary plexus (DCP VD and DCP VP). We also evaluated the foveal avascular zone (FAZ) metrics (area, perimeter, and circularity), and correlation between systemic and OCTA parameters. A literature review on the current understanding of OCTA in FD is then presented. Results: FD patients showed significantly lower SCP VD values in the whole area (17.37 ± 2.08 mm−1 vs. 18.54 ± 1.21 mm−1; p -value 0.022), as well as in the outer area (17.46 ± 2.10 mm−1 vs. 19.08 ± 1.14 mm−1; p -value 0.002), but not in the inner. Even the DCP VD was significantly lower in all the imaged areas: whole (17.75 ± 3.93 mm−1 vs. 19.71 ± 1.20 mm−1; p -value 0.024), outer (18.25 ± 4.17 mm−1 vs. 20.33 ± 1.20 mm−1; p -value 0.023), and inner (19.54 ± 4.17 mm−1 vs. 21.96 ± 1.55 mm−1; p -value 0.011). There were no significant differences in vessel perfusion parameters (both SCP VP and DCP VP ones) and FAZ. No significant correlations were found between the OCTA parameters and systemic parameters (maximal left ventricular wall thickness and glomerular filtration rate) in FD patients. Conclusions: OCTA can be considered as a promising non-invasive tool, which enables a quantitative evaluation of retinal vascular involvement in FD, despite the varying data reported in literature. Our results support the use of OCTA as an objective tool to evaluate retinal vascular abnormalities in FD. The utility of OCTA in FD needs to be validated by longitudinal studies taking into account the overall progression of the disease. [ABSTRACT FROM AUTHOR]

Fabry's disease (FD) is an X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A, resulting in the accumulation of the glycosphingolipid globotriaosylceramide (Gb3). FD is characterized by the involvement of several systems including the renal, neurological, cardiovascular, cochleovestibular, ocular, gastrointestinal and cutaneous systems. Hemizygous male individuals are primarily affected. Heterozygous females may display moderate to severe disease, which is likely related to the pattern of X-chromosome inactivation. There is a wide spectrum of FD severity. In the classic form of the disease, the clinical manifestations start in childhood and worsen over time according to the glycosphingolipid deposition. In a small percentage of patients with residual enzyme activity the onset of symptoms is late and affects mainly the heart (late-onset variants). Substrate storage affects the kidney, leading to nephropathy as one of the major complications of FD; this may occur in both sexes. These patients develop proteinuria and progress rapidly to chronic renal failure leading to end-stage renal disease. Enzyme replacement therapy and chaperone therapy have been demonstrated to improve the course of the disease, especially when started early. Since the diagnosis of FD is difficult to make, these elements put the nephrologist in a crucial position to diagnose the disease and identify patients who need specific therapy. A multidisciplinary approach is vital in the management of the disease. We describe the role of a multidisciplinary team in the diagnosis, treatment and follow-up of Fabry patients. [ABSTRACT FROM AUTHOR]

Thrombotic microangiopathies (TMA) are a group of clinical conditions mainly characterized by low platelet count, hemolytic anemia and organ damage. TMA can be acquired or hereditary and classified according to their etiology. Both males and females can be affected although different prevalence in age is observed and related to the subgroup. Hyperactivation of the alternative pathway of complement cascade, independently from its etiology, is accountable for microvascular thrombosis and its related clinical manifestations. The onset of TMA is often aggressive and it can be difficult to make an early correct differential diagnosis. It is crucial to perform specific tests at clinical presentation to help the clinician in making the diagnosis and to begin a better and more specific treatment. Research of genetic mutations is helpful to identify genetic TMA, sporadic or familiar, but not for the diagnosis at clinical onset. Because of TMA rarity, patients should be promptly sent to the attention of hematologists. New approaches on diagnosis and therapies have improved the prognosis of TMA especially in adults but further studies are needed. [ABSTRACT FROM AUTHOR]