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3 results on '"de la Cerda, Francisco"'

1. Is the atypical hemolytic uremic syndrome risk polymorphism in Membrane Cofactor Protein MCPggaac relevant in kidney transplantation? A case report

Author

Sánchez-Moreno, Ana [0000-0003-0684-7598], Rodríguez-Barba, Adela [0000-0003-4382-3085], Arjona, Emilia [0000-0002-0753-3657], Rodríguez de Córdoba, Santiago [0000-0001-6401-1874], Sánchez-Moreno, Ana, de la Cerda, Francisco, Rodríguez-Barba, Adela, Fijo, Julia, Bedoya, Rafael, Arjona, Emilia, Rodríguez de Córdoba, Santiago, Sánchez-Moreno, Ana [0000-0003-0684-7598], Rodríguez-Barba, Adela [0000-0003-4382-3085], Arjona, Emilia [0000-0002-0753-3657], Rodríguez de Córdoba, Santiago [0000-0001-6401-1874], Sánchez-Moreno, Ana, de la Cerda, Francisco, Rodríguez-Barba, Adela, Fijo, Julia, Bedoya, Rafael, Arjona, Emilia, and Rodríguez de Córdoba, Santiago

Abstract

aHUS is a rare disease characterized by episodes of TMA that frequently progresses to CKD and often recurs after KT. The most frequent cause of aHUS is defective regulation of complement activation because of genetic anomalies. Eculizumab interrupts the process of TMA and improves renal function. We describe one female patient with aHUS who debuted in 2005 at 3-mo-old with extrarenal manifestations and progressed to end-stage kidney disease (ESKD) within a year. Her family history included several affected members with similar bad outcomes. Our patient carries a strong aHUS genetic predisposition consisting in a pathogenic gain-of-function mutation in complement factor B concurrent with the MCP aHUS risk haplotype MCPggaac. She received a kidney transplant in 2011 without eculizumab prophylaxis.The graft, which was negative for the MCPggaac risk haplotype, had an unexpected excellent evolution without aHUS recurrence. Different retrospective studies have shown that the risk of aHUS recurrence after KT correlates well with the genetic load of aHUS risk factors. Knowing important contribution of the MCPggaac risk haplotype to the risk of developing aHUS in Factor B mutations carriers, we speculate whether the absence of this polymorphism in the graft that our patient received may have decreased the risk of aHUS recurrence after KT.

Published
2020

2. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome

Author

Instituto de Salud Carlos III, European Commission, Ministerio de Economía y Competitividad (España), Cavero, Teresa [0000-0001-5187-9906], Arjona, Emilia [0000-0002-0753-3657], Soto, Karina [0000-0002-6977-9361], Caravaca-Fontán, Fernando [0000-0002-5830-9663], Blasco, Miquel [0000-0003-0789-7992], Huerta, Ana [0000-0003-3342-7628], Fulladosa, Xavier [0000-0003-1974-9874], Ramos, Natalia [0000-0001-9832-326X], Morales-Ruiz, E. [0000-0002-5824-6973], Draibe, Juliana [0000-0002-2819-8560], Quintana, Luis F. [0000-0001-7582-8476], Barros, Xoana [0000-0001-9690-9769], Cao, Mercedes [0000-0002-8389-1800], Rodríguez de Córdoba, Santiago [0000-0001-6401-1874], Praga, Manuel [0000-0001-9270-1071], Cavero, Teresa, Arjona, Emilia, Soto, Karina, Caravaca-Fontán, Fernando, Rabasco, Cristina, Bravo, Luis, de la Cerda, Francisco, Martín, Nadia, Blasco, Miquel, Ávila, Ana, Huerta, Ana, Cabello-Chaves, Virginia, Jarque, Ana, Alcázar, Concepción, Fulladosa, Xavier, Carbayo, Javier, Anaya, Sara, Cobelo, Carmen, Ramos, Natalia, Iglesias, Elena, Baltar, José, Martínez-Gallardo, Rocío, Pérez, Lourdes, Morales-Ruiz, E., González, Roberto, Macía, Manuel, Draibe, Juliana, Pallardó, Luis, Quintana, Luis F., Espinosa, Mario, Barros, Xoana, Pereira, Fernando, Cao, Mercedes, Moreno, Juan Antonio, Rodríguez de Córdoba, Santiago, Praga, Manuel, Instituto de Salud Carlos III, European Commission, Ministerio de Economía y Competitividad (España), Cavero, Teresa [0000-0001-5187-9906], Arjona, Emilia [0000-0002-0753-3657], Soto, Karina [0000-0002-6977-9361], Caravaca-Fontán, Fernando [0000-0002-5830-9663], Blasco, Miquel [0000-0003-0789-7992], Huerta, Ana [0000-0003-3342-7628], Fulladosa, Xavier [0000-0003-1974-9874], Ramos, Natalia [0000-0001-9832-326X], Morales-Ruiz, E. [0000-0002-5824-6973], Draibe, Juliana [0000-0002-2819-8560], Quintana, Luis F. [0000-0001-7582-8476], Barros, Xoana [0000-0001-9690-9769], Cao, Mercedes [0000-0002-8389-1800], Rodríguez de Córdoba, Santiago [0000-0001-6401-1874], Praga, Manuel [0000-0001-9270-1071], Cavero, Teresa, Arjona, Emilia, Soto, Karina, Caravaca-Fontán, Fernando, Rabasco, Cristina, Bravo, Luis, de la Cerda, Francisco, Martín, Nadia, Blasco, Miquel, Ávila, Ana, Huerta, Ana, Cabello-Chaves, Virginia, Jarque, Ana, Alcázar, Concepción, Fulladosa, Xavier, Carbayo, Javier, Anaya, Sara, Cobelo, Carmen, Ramos, Natalia, Iglesias, Elena, Baltar, José, Martínez-Gallardo, Rocío, Pérez, Lourdes, Morales-Ruiz, E., González, Roberto, Macía, Manuel, Draibe, Juliana, Pallardó, Luis, Quintana, Luis F., Espinosa, Mario, Barros, Xoana, Pereira, Fernando, Cao, Mercedes, Moreno, Juan Antonio, Rodríguez de Córdoba, Santiago, and Praga, Manuel

Abstract

Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy (TMA), but pathogenic mutations in complement genes have been reported in patients with hypertension-induced TMA. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension. A cohort of 110 patients with malignant hypertension caused by diseases other than aHUS served as control. Thirty-six patients with aHUS presented Grade 2 or Grade 3 hypertension and funduscopic examination showed malignant hypertension in 19. Genetic abnormalities in complement were found in 19 patients (37% among patients with malignant hypertension). Plasmapheresis was performed in 46 patients and 26 received eculizumab. Renal and hematological responses were significantly lower after plasmapheresis (24%) than after eculizumab (81%). Renal survival was significantly higher in patients treated with eculizumab (85% at one, three and five years) compared to patients who did not receive this treatment (54%, 46% and 41%), respectively. Response to eculizumab was independent of hypertension severity and the presence of complement genetic abnormalities. Among patients with malignant hypertension caused by other diseases the prevalence of TMA was very low (5%). Thus, severe and malignant hypertension are common among patients with aHUS and eculizumab treatment leads to a higher renal survival when compared to plasmapheresis. However, TMA is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS.

Published
2019

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