Abstract:BackgroundAtypical hemolytic uremic syndrome (aHUS), a rare thrombotic microangiopathy, is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. Caused by genetic mutations in the alternative complement cascade, aHUS often will culminate in end-stage renal disease and occasionally death. Renal transplantation in aHUS patients has been contraindicated in the past due to the recurrence risk, with certain immunosuppressive regimens being commonly attributed. In this study, we analyzed the … Show more
“…Discontinuation of CNI was common in managing these patients in the present study which is consistent with other studies [9,15,19]. The discontinuation of CNI may be attributed to potential endothelial injury and/or vasoconstriction linked to CNI, which…”
BackgroundAlternate complement dysregulation postrenal transplantation can result in thrombotic microangiopathy (TMA). There is a scarcity of data regarding outcomes based on the timing of TMA post‐transplant, coupled with a lack of follow‐up biopsy findings post TMA diagnosis. This study aims to assess allograft and patient outcomes in individuals developing early TMA, defined within 4 months post‐transplantation, and explore any differences in follow‐up surveillance biopsies compared to a non‐TMA group.DesignThis is a single center retrospective study between January 1, 2002 and October 10, 2019. Patients who developed TMA within 4 months post‐transplantation were compared to a propensity matched non‐TMA group.ResultsThirty‐one patients developed TMA within 4 months of renal transplantation. Index TMA biopsy featured noticeable glomerular, and vascular lesions along with acute tubular injury. Four‐month surveillance biopsy showed significant glomerulitis, transplant glomerulopathy and chronic interstitial fibrosis as compared to non‐TMA group. However, at 1 year, these differences were no longer significant. There was no significant difference in patient survival (TMA vs. non‐TMA, p = 0.083); however, death censored graft survival was significantly lower in the TMA group (p < 0.001). TMA patients had a significantly lower estimated glomerular filtration rate at 4 months and at 1 year as compared to the non‐TMA group.ConclusionEarly onset TMA post renal transplant leads to decreased renal function and lower graft survival. Early recognition and prompt treatment may help in reducing the adverse outcomes.
“…Discontinuation of CNI was common in managing these patients in the present study which is consistent with other studies [9,15,19]. The discontinuation of CNI may be attributed to potential endothelial injury and/or vasoconstriction linked to CNI, which…”
BackgroundAlternate complement dysregulation postrenal transplantation can result in thrombotic microangiopathy (TMA). There is a scarcity of data regarding outcomes based on the timing of TMA post‐transplant, coupled with a lack of follow‐up biopsy findings post TMA diagnosis. This study aims to assess allograft and patient outcomes in individuals developing early TMA, defined within 4 months post‐transplantation, and explore any differences in follow‐up surveillance biopsies compared to a non‐TMA group.DesignThis is a single center retrospective study between January 1, 2002 and October 10, 2019. Patients who developed TMA within 4 months post‐transplantation were compared to a propensity matched non‐TMA group.ResultsThirty‐one patients developed TMA within 4 months of renal transplantation. Index TMA biopsy featured noticeable glomerular, and vascular lesions along with acute tubular injury. Four‐month surveillance biopsy showed significant glomerulitis, transplant glomerulopathy and chronic interstitial fibrosis as compared to non‐TMA group. However, at 1 year, these differences were no longer significant. There was no significant difference in patient survival (TMA vs. non‐TMA, p = 0.083); however, death censored graft survival was significantly lower in the TMA group (p < 0.001). TMA patients had a significantly lower estimated glomerular filtration rate at 4 months and at 1 year as compared to the non‐TMA group.ConclusionEarly onset TMA post renal transplant leads to decreased renal function and lower graft survival. Early recognition and prompt treatment may help in reducing the adverse outcomes.
“…These drugs are commonly employed for chemotherapy and post-transplant immunosuppression. 70 Supportive needs should also be monitored including: Red blood cell transfusion for hemoglobin <7 g/dL in adults and <6 g/dL in children Platelet transfusion for platelet count <50k/μL Fluids for hydration and electrolyte imbalances Figure 6 A multidisciplinary approach involving nephrologists, hematologists, community advocates, and potentially other sub-specialists (eg, gynecologists, dermatologists) is required for comprehensive management of hemolytic-uremic syndrome. …”
Section: Comprehensive Carementioning
confidence: 99%
“…These drugs are commonly employed for chemotherapy and post-transplant immunosuppression. 70 Supportive needs should also be monitored including:…”
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) defined by the triad of hemolytic anemia, thrombocytopenia, and acute kidney injury. Microthrombi develop in the glomerular capillaries secondary to endothelial damage and exert shear stress on red blood cells, consume platelets, and contribute to renal dysfunction and failure. Per current understanding of pathophysiology, HUS is classified into infectious, secondary, and atypical disease. The most common etiology is infectious sequelae of Shiga toxin-producing
Escherichia coli
(STEC); other causative organisms include shigella and salmonella. Secondary HUS arises from cancer, chemotherapy, solid organ and hematopoietic stem cell transplant, pregnancy, or autoimmune disorders. Primary atypical hemolytic-uremic syndrome (aHUS) is associated with genetic mutations in complement and complement regulatory proteins. Under physiologic conditions, complement regulators keep the alternative complement system continuously active at low levels. In times of inflammation, mutations in complement-related proteins lead to uncontrolled complement activity. The hyperactive inflammatory state leads to glomerular endothelial damage, activation of the coagulation cascade, and TMA findings. Atypical hemolytic-uremic syndrome is a rare disorder with a prevalence of 2.21 to 9.4 per million people aged 20 years or younger; children between the ages of 0 and 4 are most affected. Multidisciplinary health care is necessary for timely management of its extra-renal manifestations. These include vascular disease of the heart, brain, and skin, pulmonary hypertension and hemorrhage, and pregnancy complications. Adequate screening is required to monitor for sequelae. First-line treatment is the monoclonal antibody eculizumab, but several organ systems may require specialized interventions and coordination of care with sub-specialists.
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