Systemic thrombotic microangiopathy associated with complement pathway mutations in living donor kidney transplant: case report

El síndrome urémico hemolítico (SUH) se caracteriza por la presencia de anemia hemolítica,plaquetopenia e insuficiencia renal aguda. Si bien se distingue clásicamente en típico o infeccioso y atípico, es menester reconocer situacionesclínicas en las que se pone de manifiesto, como por ejemplo, embarazo, puerperio inmediato,tumores, trasplante, drogas, etc., condiciones clínicas que han sido denominadas amplificadoras del complemento.La recurrencia postrasplante del síndrome urémico hemolítico atípico (SUHa) ha sido descrita en porcentajes variables en pacientes con mutaciones del factor H, factor B, factor I y C3, y gen de la trombomodulina, en reportes de casos aislados. Se presenta el caso de una paciente con enfermedad renal crónica (ERC) secundaria a agenesia renal, receptora preemptive de un riñón de donante vivo relacionado que presentó disfunción del injerto renal secundaria a microangiopatía trombótica, asociado acomplicación neurológica, hemorragias, disfunción orgánica múltiple y óbito. Se describen los hallazgos del estudio genético yanatomopatológico de necropsia.Hemolytic uremic syndrome (HUS) is characterized by the presence of hemolytic anemia, thrombocytopenia and acute kidney injury. Although it is usually distinguished as typical or infectious and atypical, it is necessary to recognize clinical situations in which it is revealed, such as pregnancy, immediate postpartum period, tumors, transplantation, drugs, etc., i.e. clinical conditions that have been called complement-amplifying conditions. Post-transplantation recurrence of atypical hemolytic uremic syndrome (aHUS) has been described in variable percentages in patients with mutations of factor H, factor B, factor I and C3, and thrombomodulin gene, in reports of isolated cases. We present the case of a patient with chronic kidney disease (CKD) secondary to renal agenesis, a preemptive recipient of a related living donor kidney, which presented renal graft dysfunction secondary to thrombotic microangiopathy, associated with neurological complications, hemorrhages, multiple organ dysfunction and death. The findings of the genetic and pathological autopsy study are described.Fil: Gutierrez, Roberto. Fundación Favaloro; ArgentinaFil: Fortunato, Rita Marcela. Fundación Favaloro; ArgentinaFil: Vigliano, Carlos. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; ArgentinaFil: Espinoza, Álvaro. Fundación Favaloro; ArgentinaFil: Nava, Alison. Fundación Favaloro; ArgentinaFil: De Francesco, Juan. Fundación Favaloro; ArgentinaFil: Raffaele, Pablo Miguel. Fundación Favaloro; Argentin

Calciphylaxis after renal transplant. Three clinical cases report

Introduction: Calciphylaxis (CFX) is a syndrome characterized by deposition of calcium in the intima and media of vessels, intimal proliferation, fibrosis, luminal thrombosis, tissue ischemia and necrosis. Its initial report and subsequent descriptions were associated with chronic renal failure. There is little information regarding the possible effect of the recovery of renal function secondary to kidney transplantation in the incidence of this disease. Methods: Center retrospective study. We analyze in this report the three cases of patients who developed CFX after a renal transplant within a cohort of 448 kidney and kidney-pancreas transplant patients from January 1th 2001 to January 1th 2014 in our Hospital. Results: Three patients were found to have CFX. All of them had hypercalcemia (serum calcium average 11.5 mg/dl) at first year post transplant and 2 patients at diagnosis of CFX. PTHi in the three CFX patients was 2 pg/ml, 62,3pg/ml and 3561pg/ml respectively. Hypoalbuminemia was found in all patients. Two patients were diabetic. Only one patient was obese and under anticoagulation treatment. In all cases a biopsy provided the diagnosis of certainty for calciphylaxis. Median serum creatinine at diagnosis was 1.5 mg/dl (1.2 mg/dl 1.2 mg/dl and 2 mg/dl, respectively) and the average time between transplantation and calciphylaxis diagnosis was 32 months. In all cases, strict control of phosphorus and hypercalcemia and sodium IV thiosulfate treatment was performed. The evolution was successful in two patients, controlling blood calcium and improving cutaneous manifestations with preservation of renal function. Conclusions: CFX prevalence in a cohort of 448 kidney and kidney-pancreas transplant patients from 2001 to 2014 was 0.66%, less than reported in dialysis patients. Factors associated with CFX in our patients were hypercalcemia in the first year after renal transplant and at the time of the event, hypoalbuminemia, diabetes and disorders of the parathyroid gland. The persistence of hypercalcemia in the first year after renal transplant should be an element of high clinical suspicion of this complication in the kidney transplant recipients

Microangiopatía trombótica sistémica asociado a mutaciones del complemento en trasplante renal donante vivo. Reporte de caso

Hemolytic uremic syndrome (HUS) is characterized by the presence of hemolytic anemia, thrombocytopenia and acute kidney injury. Although it is usually distinguished as typical or infectious and atypical, it is necessary to recognize clinical situations in which it is revealed, such as pregnancy, immediate postpartal period, tumors, transplantation, drugs, etc., i.e. clinical conditions that have been called complement-amplifying conditions.Post-transplantation recurrence of atypical hemolytic uremic syndrome (aHUS) has been described in variable percentages in patients with mutations of factor H, factor B, factor I and C3, and thrombomodulin gene, in reports of isolated cases. We present the case of a patient with chronic kidney disease (CKD) secondary to renal agenesis, a preemptive recipient of a related living donor kidney, which presented renal graft dysfunction secondary to thrombotic microangiopathy, associated with neurological complications, hemorrhages, multiple organ dysfunction and death.  The findings of the genetic and pathological autopsy study are described.El síndrome urémico hemolítico (SUH) se caracteriza por la presencia de anemia hemolítica, plaquetopenia e insuficiencia renal aguda. Si bien se distingue clásicamente en típico o infeccioso y atípico, es menester reconocer situaciones clínicas en las que se pone de manifiesto, como por ejemplo, embarazo, puerperio inmediato, tumores, trasplante, drogas, etc., condiciones clínicas que han sido denominadas amplificadoras del complemento.La recurrencia postrasplante del síndrome urémico hemolítico atípico (SUHa) ha sido descrita en porcentajes variables en pacientes con mutaciones del factor H, factor B, factor I y C3, y gen de la trombomodulina, en reportes de casos aislados. Se presenta el caso de una paciente con enfermedad renal crónica (ERC) secundaria a agenesia renal, receptora preemptive de un riñón de donante vivo relacionado que presentó disfunción del injerto renal secundaria a microangiopatía trombótica, asociado a complicación neurológica, hemorragias, disfunción orgánica múltiple y óbito. Se describen los hallazgos del estudio genético y anatomopatológico de necropsia

Combined cardiorenal transplant in heart and advanced renal disease

Introduction: Renal failure (RF) is a post cardiac transplantation predictor of morbimortality. The combined cardiorenal transplant (CCRTx) in cardiac transplantation (CTx) candidates with chronic renal disease is a therapeutic option. Our aim was to evaluate the CCRTx follow up outcomes in a single Centre. Methods: Between 2/1993 and 12/2014 we performed 442 CTx. Since 2006, 20 patients (p) underwent CCRTx using allografts from the same donor. The inclusion criteria were: RF with CrCl ≤ 40 mil/min or dialysis requirement in CTx candidates. All p received Thymoglobulin and immunosuppression with tacrolimus, mycophenolate mofetil and steroids.Median follow up: 46 months (7-96). Results: Mean age: 58±7 years, 85% were male. Mean Creatinine (Cr): 3,1±2,5 mg/dl and ClCr 27,5+10 mil/min. Three p required dialysis during the pre-transplantation phase and 4 p were under chronic dialysis. Etiologies: cardiomyopathies: coronary 10 p, noncoronary 9 p and re CTx, 1 p; nephropathies: nephroangiosclerosis 5 p, cardiorenal syndrome 10 p, diabetes 2 p, glomerulopathy 1 p, polycystosis 1 p and toxic nephritis 1 p. At 30 days and 1 year post CCRTx, Cr was 1,2±0,4 mg/dl and 1,1±0,2 mg/dl respectively. In-hospital mortality was 3/20 p (15%), 2 p due to sepsis and 1 p due to cardiac graft failure. Late mortality 5/17 p (29 %), 4p due to sepsis and 1 p due to liver sarcoma. Survival at 1 and 3 years was 76 and 72%, respectively. Conclusions: In our series CCRTx was a safe and effective treatment for CTx candidates with CrCl < 40 ml/min

Paired donation in Argentina. Bioethics reflections based on the study of living donors. Joint Document of the Bioethics Committees of the Sociedad Argentina de Nefrología and the Sociedad Argentina de Trasplante

Este documento está orientado a profundizar el análisis bioético para el establecimiento de normas claras y aplicables según criterios sustentables y adecuados en cuanto a los procedimientos relativos a la Donación Pareada en el trasplante renal y a su implementación como alternativa en la actividad trasplantológica, que al mismo tiempo consoliden la prohibición de la comercialización, obtención de privilegios y toda otra desviación de la buena práctica de los trasplantes de órganos. La Donación Pareada es el intercambio de órganos que se lleva a cabo cuando un donante vivo es incompatible con su par receptor, pero compatible con el receptor de otro par donante/receptor a su vez incompatibles entre sí, y/o redunde en beneficios inmunológicos, infectológicos y etarios equitativamente. Implicaría también un beneficio adicional a la procuración de órganos, en la medida que se erige en una alternativa a la dación de órganos provenientes de cadáveres o personas relacionadas con el receptor, toda vez que la Donación Pareada complementa a la normativa actualmente vigente en la República Argentina; por ello, cabe concluir sobre su carácter excepcional, desde que se instrumenta a partir del procedimiento judicial especial regulado en al artículo 56 de la Ley 24.193. Teniendo en cuenta numerosos estudios realizados y el estado del arte en países pioneros en esta práctica tales como Holanda, Turquía, Noruega, Corea del Sur, Estados Unidos de Norteamérica, España, Gran Bretaña, que hacen referencia a que los trasplantes con donante vivo mejoran la sobrevida y la calidad de vida de los pacientes trasplantados, al tiempo que bajo la figura de Donación Pareada se incrementaría el numero de trasplantes. De ser así no habría nada que objetar. Sin embargo es necesario destacar que estas apreciaciones generales han sido estudiadas fundamentalmente en otros países, contextos y culturas. Desde la perspectiva de la ética aplicada se hace necesario entonces observar ante cualquier innovación en el sistema que regule la trasplantología Argentina para adecuarla a este contexto y la problemática específica de nuestro país y pasar revista a la situación actual

Growing knowledge: an overview of Seed Plant diversity in Brazil

Abstract An updated inventory of Brazilian seed plants is presented and offers important insights into the country's biodiversity. This work started in 2010, with the publication of the Plants and Fungi Catalogue, and has been updated since by more than 430 specialists working online. Brazil is home to 32,086 native Angiosperms and 23 native Gymnosperms, showing an increase of 3% in its species richness in relation to 2010. The Amazon Rainforest is the richest Brazilian biome for Gymnosperms, while the Atlantic Rainforest is the richest one for Angiosperms. There was a considerable increment in the number of species and endemism rates for biomes, except for the Amazon that showed a decrease of 2.5% of recorded endemics. However, well over half of Brazillian seed plant species (57.4%) is endemic to this territory. The proportion of life-forms varies among different biomes: trees are more expressive in the Amazon and Atlantic Rainforest biomes while herbs predominate in the Pampa, and lianas are more expressive in the Amazon, Atlantic Rainforest, and Pantanal. This compilation serves not only to quantify Brazilian biodiversity, but also to highlight areas where there information is lacking and to provide a framework for the challenge faced in conserving Brazil's unique and diverse flora