15 results on '"López-Jiménez V"'
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2. Likely Recurrence of C3 Glomerulonephritis in Kidney Transplantation: An Entity to Bear in Mind: Case Report
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Perea-Ortega, L., primary, León-Fradejas, M., additional, Alonso-Titos, J., additional, Cabello-Díaz, M., additional, Toledo-Rojas, R., additional, Sola-Moyano, E., additional, Martín-Reyes, G., additional, López-Jiménez, V., additional, Burgos-Rodríguez, D., additional, and Hernández-Marrero, D., additional
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- 2015
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3. Transplant Glomerulopathy: Clinical Course and Factors Relating to Graft Survival
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López Jiménez, V., primary, Fuentes, L., additional, Jiménez, T., additional, León, M., additional, Garcia, I., additional, Sola, E., additional, Cabello, M., additional, Gutierrez, C., additional, Burgos, D., additional, Ruiz, P., additional, and Hernandez, D., additional
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- 2012
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4. Creatinine Clearance and Proteinuria as Early Markers of Kidney Graft Survival
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Martínez Esteban, D., primary, Jironda Gallegos, C., additional, Cabello Diaz, M., additional, Frias, P., additional, López Jiménez, V., additional, Gutierrez de la Fuente, C., additional, Sola Moyano, E., additional, Burgos Rodríguez, D., additional, González Molina Alcaide, M., additional, and Hernández Marrero, D., additional
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- 2010
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5. Dieta hipoalergénica materna para mejorar el cólico del lactante: revisión sistemática.
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Escobedo Mesas, E., López Jiménez, V., Casado Gómez, O., and Grande Trillo, A.
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HYPOALLERGENIC infant formulas , *COLIC , *BREASTFEEDING - Abstract
Introducción: Los cólicos del lactante, llanto de más de 3 horas al día, más de 3 días a la semana con duración de más de 3 semanas, son un motivo de gran preocupación parental que genera numerosas consultas sanitarias. Objetivos: Valorar en base a la evidencia disponible sobre el cólico infantil, los beneficios que aporta una dieta hipoalergénica (DH) materna. Metodología: Revisión sistemática con palabras claves "Infan-tile colic" e "hypoallergenic" en bases de datos (Pubmed, Coch-rane,...) filtrando sólo estudios de alta evidencia. Resultados: Dentro de los estudios analizados, destacamos un estudio con 115 niños en el que se redujo la sintomatología en un 61% de los niños cuyas madres realizaban DH frente a un 41% del grupo control (p = 0,047) y otro estudio con 107 niños en el que se redujo la clínica en un 74% de los niños cuyas madres realizaban DH frente a un 37% del grupo control (p < 0,001). Discusión: Analizando los resultados de la búsqueda se observa que la evidencia científica sobre esta medida es escasa. Sin embargo, los ensayos clínicos realizados con tal fin parecen mostrar beneficio en la aplicación de esta intervención. Consideramos por tanto que sería útil profundizar en la investigación de este campo e intentar su aplicación dado lo inocuo de la misma. [ABSTRACT FROM AUTHOR]
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- 2012
6. Evolving spectrum but persistent high mortality of COVID-19 among patients on kidney replacement therapy in the vaccine era: the Spanish COVID-19 KRT Registry
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Quiroga, Borja, Ortiz, Alberto, Cabezas-Reina, Carlos Jesús, Ruiz Fuentes, María Carmen, López Jiménez, Verónica, Zárraga Larrondo, Sofía, Toapanta, Néstor, Molina Gómez, María, de Sequera, Patricia, Sánchez-Álvarez, Emilio, Spanish COVID-19 KRT Registry collaborative group, Institut Català de la Salut, [Quiroga B] IIS-La Princesa, Nephrology Department, Hospital de la Princesa, Madrid, Spain. [Ortiz A] IIS-Fundación Jimenez Diaz, School of Medicine, Universidad Autónoma de Madrid, Fundación Renal Iñigo Alvarez de Toledo-IRSIN, REDinREN, Instituto de Investigación Carlos III, Madrid, Spain. [Cabezas-Reina CJ] Nephrology Department, Hospital Universitario de Toledo, Toledo, Spain. [Ruiz Fuentes MC] Nephrology Department, Hospital Virgen de las Nieves, Granada, Spain. [López Jiménez V] Nephrology Department, Hospital Regional Universitario de Málaga, Universidad de Málaga, Instituto de Investigación Biomédica de Málaga, Málaga, Spain. [Zárraga Larrondo S] Nephrology Department, Hospital Universitario de Cruces, Bizkaia, Spain. [Toapanta N] Servei de Nefrologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain, and Vall d'Hebron Barcelona Hospital Campus
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kidney transplant ,terapéutica::tratamiento de reemplazo renal [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS] ,Transplantation ,Otros calificadores::Otros calificadores::Otros calificadores::/mortalidad [Otros calificadores] ,SARS-CoV-2 ,COVID-19 ,Ronyons - Trasplantació - Mortalitat ,COVID-19 (Malaltia) - Mortalitat ,Virus Diseases::RNA Virus Infections::Nidovirales Infections::Coronaviridae Infections::Coronavirus Infections [DISEASES] ,Therapeutics::Renal Replacement Therapy [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT] ,mortality ,Investigative Techniques::Epidemiologic Methods::Data Collection::Registries [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT] ,técnicas de investigación::métodos epidemiológicos::recopilación de datos::registros [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS] ,Registres mèdics ,Nephrology ,Other subheadings::Other subheadings::Other subheadings::/mortality [Other subheadings] ,virosis::infecciones por virus ARN::infecciones por Nidovirales::infecciones por Coronaviridae::infecciones por Coronavirus [ENFERMEDADES] ,dialysis - Abstract
COVID-19; Kidney transplant; Dialysis COVID-19; Trasplantament de ronyó; Diàlisi COVID-19; Trasplante de riñón; Diálisis Background Kidney replacement therapy (KRT) conferred a high risk for coronavirus disease 2019 (COVID-19) related mortality early in the pandemic. We evaluate the presentation, treatment and outcomes of COVID-19 in patients on KRT over time during the pandemic. Methods This registry-based study involved 6080 dialysis and kidney transplant (KT) patients with COVID-19, representing roughly 10% of total Spanish KRT patients. Epidemiology, comorbidity, infection, vaccine status and treatment data were recorded, and predictors of hospital admission, intensive care unit (ICU) admission and mortality were evaluated. Results Vaccine introduction decreased the number of COVID-19 cases from 1747 to 280 per wave. Of 3856 (64%) COVID-19 KRT patients admitted to the hospital, 1481/3856 (38%) were admitted during the first of six waves. Independent predictors for admission included KT and the first wave. During follow-up, 1207 patients (21%) died, 500/1207 (41%) during the first wave. Among vaccinated patients, mortality was 19%, mostly affecting KT recipients. Overall, independent predictors for mortality were older age, disease severity (lymphopaenia, pneumonia) and ICU rejection. Among patient factors, older age, male sex, diabetes, KT and no angiotensin receptor blockers (ARB) were independent predictors of death. In KT recipients, individual immunosuppressants were independent predictors of death. Over time, patient characteristics evolved and in later pandemic waves, COVID-19 was mainly diagnosed in vaccinated KT recipients; in the few unvaccinated dialysis patients, ICU admissions increased and mortality decreased (28% for the first wave and 16–22% thereafter). Conclusions The clinical presentation and outcomes of COVID-19 during the first wave no longer represent COVID-19 in KRT patients, as the pandemic has become centred around vaccinated KT recipients. Vaccines lowered the incidence of diagnosed COVID-19 and mortality. However, mortality remains high despite increased access to ICU care. We want to thank all the implicated Spanish centres for their altruist collaboration. A.O. research is supported by FIS/Fondos FEDER [PI18/01366, PI19/00588, PI19/00815, DTS18/00032, ERA-PerMed-JTC2018 (KIDNEY ATTACK AC18/00064 and PERSTIGAN AC18/00071, ISCIII-RETIC REDinREN RD016/0009)], Sociedad Española de Nefrología, FRIAT, Comunidad de Madrid en Biomedicina B2017/BMD-3686 CIFRA2-CM. Instituto de Salud Carlos III (ISCIII) RICORS program to RICORS2040 (RD21/0005/0001), FEDER funds.
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- 2022
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7. Management of heart disease in renal transplant recipients: a national Delphi survey-based SET/SEC/SEN consensus document.
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García-Cosío MD, Cruzado JM, Farrero M, Blasco Peiró MT, Crespo M, Delgado Jiménez JF, Díaz Molina B, Fernández Rivera C, Garrido Bravo IP, López Jiménez V, Melilli E, Mirabet Pérez S, Pérez Tamajón ML, Rangel Sousa D, Rodrigo Calabia E, and Hernández Marrero D
- Abstract
Renal transplantation improves the survival and quality of life of patients with end-stage renal disease. Cardiovascular disease is the leading cause of morbidity and mortality in renal transplant recipients. The bidirectional relationship between renal and heart disease creates a unique clinical scenario that demands a comprehensive and personalized approach. This expert consensus, drafted by the Spanish Society of Transplantation, the Spanish Society of Cardiology, and the Spanish Society of Nephrology, aims to assess current practices and propose strategies for the management of heart disease in renal transplant recipients. A panel of Spanish nephrologists and cardiologists with expertise in renal and heart transplantation reviewed the scientific evidence concerning the current management of heart disease in renal transplant recipients. Subsequently, consensus statements were created through a 2-round Delphi methodology, resulting in 30 statements covering key topics such as the identification of renal transplant candidates, the management of heart disease in renal transplant recipients, and eligibility for combined heart-kidney transplantation in patients with both end-stage renal disease and cardiac disease. These consensus statements provide expert guidance for the management of heart disease in renal transplant recipients, an area where published clinical evidence remains limited., (Copyright © 2024 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.)
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- 2024
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8. Predicting Kidney Transplantation Outcomes from Donor and Recipient Characteristics at Time Zero: Development of a Mobile Application for Nephrologists.
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Pérez Valdivia MÁ, Calvillo Arbizu J, Portero Barreña D, Castro de la Nuez P, López Jiménez V, Rodríguez Benot A, Mazuecos Blanca A, de Gracia Guindo MC, Bernal Blanco G, Gentil Govantes MÁ, Bedoya Pérez R, and Rocha Castilla JL
- Abstract
(1) Background: We report on the development of a predictive tool that can estimate kidney transplant survival at time zero. (2) Methods: This was an observational, retrospective study including 5078 transplants. Death-censored graft and patient survivals were calculated. (3) Results: Graft loss was associated with donor age (hazard ratio [HR], 1.021, 95% confidence interval [CI] 1.018-1.024, p < 0.001), uncontrolled donation after circulatory death (DCD) (HR 1.576, 95% CI 1.241-2.047, p < 0.001) and controlled DCD (HR 1.567, 95% CI 1.372-1.812, p < 0.001), panel reactive antibody percentage (HR 1.009, 95% CI 1.007-1.011, p < 0.001), and previous transplants (HR 1.494, 95% CI 1.367-1.634, p < 0.001). Patient survival was associated with recipient age (> 60 years, HR 5.507, 95% CI 4.524-6.704, p < 0.001 vs. < 40 years), donor age (HR 1.019, 95% CI 1.016-1.023, p < 0.001), dialysis vintage (HR 1.0000263, 95% CI 1.000225-1.000301, p < 0.01), and male sex (HR 1.229, 95% CI 1.135-1.332, p < 0.001). The C-statistics for graft and patient survival were 0.666 (95% CI: 0.646, 0.686) and 0.726 (95% CI: 0.710-0.742), respectively. (4) Conclusions: We developed a mobile app to estimate survival at time zero, which can guide decisions for organ allocation.
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- 2024
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9. Best practices during COVID-19 pandemic in solid organ transplant programs in Spain.
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Pascual J, Mazuecos A, Sánchez-Antolín G, Solé A, Ventura-Aguiar P, Crespo M, Farrero M, Fernández-Rivera C, Garrido IP, Gea F, González-Monte E, González-Rodríguez A, Hernández-Gallego R, Jiménez C, López-Jiménez V, Otero A, Pascual S, Rodríguez-Laiz GP, Ruiz JC, Sancho A, Santos F, Serrano T, Tabernero G, Zarraga S, and Delgado JF
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- Humans, Pandemics prevention & control, Spain epidemiology, Communicable Disease Control, COVID-19, Organ Transplantation methods
- Abstract
Clinical management of transplant patients abruptly changed during the first months of COVID-19 pandemic (March to May 2020). The new situation led to very significant challenges, such as new forms of relationship between healthcare providers and patients and other professionals, design of protocols to prevent disease transmission and treatment of infected patients, management of waiting lists and of transplant programs during state/city lockdown, relevant reduction of medical training and educational activities, halt or delays of ongoing research, etc. The two main objectives of the current report are: 1) to promote a project of best practices in transplantation taking advantage of the knowledge and experience acquired by professionals during the evolving situation of the COVID-19 pandemic, both in performing their usual care activity, as well as in the adjustments taken to adapt to the clinical context, and 2) to create a document that collects these best practices, thus allowing the creation of a useful compendium for the exchange of knowledge between different Transplant Units. The scientific committee and expert panel finally standardized 30 best practices, including for the pretransplant period (n = 9), peritransplant period (n = 7), postransplant period (n = 8) and training and communication (n = 6). Many aspects of hospitals and units networking, telematic approaches, patient care, value-based medicine, hospitalization, and outpatient visit strategies, training for novelties and communication skills were covered. Massive vaccination has greatly improved the outcomes of the pandemic, with a decrease in severe cases requiring intensive care and a reduction in mortality. However, suboptimal responses to vaccines have been observed in transplant recipients, and health care strategic plans are necessary in these vulnerable populations. The best practices contained in this expert panel report may aid to their broader implementation., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier Inc.)
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- 2023
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10. Efficacy and Safety of Eculizumab in Kidney Transplant Patients With Primary Atypical Hemolytic-Uremic Syndrome.
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González CC, López-Jiménez V, Vázquez-Sánchez T, Vázquez-Sánchez E, Cabello M, and Hernández-Marrero D
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- Antibodies, Monoclonal, Humanized therapeutic use, Complement Inactivating Agents therapeutic use, Humans, Atypical Hemolytic Uremic Syndrome drug therapy, Kidney Transplantation adverse effects
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The atypical hemolytic-uremic syndrome (aHUS) is characterized by the triad of non-immune hemolytic anemia, thrombocytopenia, and acute renal failure. The aHUS is related to complement dysregulation; since the approval of eculizumab for this entity (a monoclonal antibody that inhibits C5 activation and blocks the formation of the membrane attack complex) the prognosis has improved. The recurrence of aHUS after kidney transplantation is frequent and implies loss of the graft in a high percentage of cases. Eculizumab prophylaxis to prevent recurrence has allowed successful kidney transplantation in this group of patients. We present a series of kidney transplant patients with chronic kidney disease secondary to aHUS and the use of eculizumab for prevention of recurrence., (Copyright © 2021 Elsevier Inc. All rights reserved.)
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- 2022
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11. COVID-19-related Mortality During the First 60 Days After Kidney Transplantation.
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Pascual J, Melilli E, Jiménez-Martín C, González-Monte E, Zárraga S, Gutiérrez-Dalmau A, López-Jiménez V, Juega J, Muñoz-Cepeda M, Lorenzo I, Facundo C, Ruiz-Fuentes MDC, Mazuecos A, Sánchez-Álvarez E, and Crespo M
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- Adult, Aged, COVID-19, Coronavirus Infections diagnosis, Coronavirus Infections immunology, Coronavirus Infections virology, Female, Host-Pathogen Interactions, Humans, Immunocompromised Host, Immunosuppressive Agents adverse effects, Kidney Transplantation adverse effects, Male, Middle Aged, Opportunistic Infections diagnosis, Opportunistic Infections immunology, Opportunistic Infections virology, Pandemics, Pneumonia, Viral diagnosis, Pneumonia, Viral immunology, Pneumonia, Viral virology, Registries, Risk Assessment, Risk Factors, SARS-CoV-2, Spain, Time Factors, Treatment Outcome, Betacoronavirus pathogenicity, Coronavirus Infections mortality, Kidney Transplantation mortality, Opportunistic Infections mortality, Pneumonia, Viral mortality
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- 2020
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12. Necrotizing herpetic retinopathy in kidney-transplanted patients on mycophenolate mofetil.
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Morión Grande M, Martín-Gómez MA, Quereda Castañeda A, López Jiménez V, and Cabezas Fernández T
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- Aged, Humans, Immunosuppressive Agents therapeutic use, Male, Mycophenolic Acid therapeutic use, Varicella Zoster Virus Infection pathology, Immunosuppressive Agents adverse effects, Kidney Transplantation, Mycophenolic Acid adverse effects, Retinal Diseases virology
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- 2016
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13. Effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism.
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Borrego Utiel FJ, Bravo Soto JA, Merino Pérez MJ, González Carmelo I, López Jiménez V, García Álvarez T, Acosta Martínez Y, and Mazuecos Blanca MA
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- Adult, Aged, Alkaline Phosphatase blood, Bone Density Conservation Agents administration & dosage, Bone Density Conservation Agents therapeutic use, Bone Diseases, Metabolic etiology, Bone Diseases, Metabolic prevention & control, Bone and Bones metabolism, Calcitriol therapeutic use, Cinacalcet therapeutic use, Drug Substitution, Drug Therapy, Combination, Ergocalciferols administration & dosage, Ergocalciferols therapeutic use, Female, Follow-Up Studies, Humans, Hyperparathyroidism, Secondary etiology, Immunosuppressive Agents therapeutic use, Male, Middle Aged, Parathyroid Hormone blood, Postoperative Complications etiology, Retrospective Studies, Bone Density Conservation Agents pharmacology, Bone and Bones drug effects, Calcium metabolism, Ergocalciferols pharmacology, Hyperparathyroidism, Secondary drug therapy, Kidney Transplantation, Phosphorus metabolism, Postoperative Complications drug therapy
- Abstract
Introduction: Secondary hyperparathyroidism is highly prevalent in kidney transplant recipients, and commonly results in hypercalcaemia; an association to osteopenia and bone fractures has also been observed. Paricalcitol has proved effective to control secondary hyperparathyroidism in chronic kidney disease in both dialysed and non-dialysed patients, with a low hypercalcaemia incidence. Currently available experience on paricalcitol use in kidney transplant recipients is scarce. Our main aim was to show the effect of paricalcitol on mineral bone metabolism in kidney transplant recipients with secondary hyperparathyroidism., Material and Methods: A retrospective multicentre study in kidney transplant recipients aged>18 years with a 12-month or longer post-transplantation course, stable renal function, having received paricalcitol for more than 12 months, with available clinical follow-up for a 24-month period., Results: A total of 69 patients with a 120 ± 92-month post-transplantation course were included. Baseline creatinine was 2.2 ± 0.9 mg/dl y GFR-MDRD was 36 ± 20 ml/min/1.73 m(2). Paricalcitol doses were gradually increased during the study: baseline 3.8 ± 1.9 μg/week, 12 months 5.2 ± 2.4 μg/week; 24 months 6.0 ± 2.9 μg/week (P<.001). Serum PTH levels showed a significant fast decline: baseline 288 ± 152 pg/ml; 6 months 226 ± 184 pg/ml; 12 months 207 ± 120; 24 months 193 ± 119 pg/ml (P<.001). Reduction from baseline PTH was ≥30% in 42.4% of patients at 12 months y in 65.2% of patients at 24 months. Alkaline phosphatase showed a significant decrease in first 6 months followed by a plateau: baseline 92 ± 50 IU/l; 6 months 85 ± 36 IU/l, 12 months 81 ± 39 IU/l (P<.001). Overall, no changes were observed in serum calcium and phosphorus, and in urine calcium excretion. PTH decline was larger in patients with higher baseline levels. Patients with lower baseline calcium levels showed significantly increased levels (mean increase was 0.5-0.6 mg/dl) but still within normal range, whereas patients with baseline calcium>10mg/dl showed gradually decreasing levels. Fifteen (21.7%) patients had received prior calcitriol therapy. When shifted to paricalcitol, such patients required paricalcitol doses significantly larger than those not having received calcitriol. Paricalcitol was used concomitantly to cinacalcet in 11 patients with significant PTH reductions being achieved; clinical course was similar to other patients and paricalcitol doses were also similar., Conclusions: Paricalcitol is an effective therapy for secondary hyperparathyroidism in kidney transplant recipients. Overall, no significant changes were observed in calcium and phosphorus levels or urinary excretion. Patients having previously received calcitriol required higher paricalcitol doses. When used in patients receiving cinacalcet, paricalcitol results in a significant PTH fall, with paricalcitol doses being similar to those used in patients not receiving cinacalcet., (Copyright © 2015 The Authors. Published by Elsevier España, S.L.U. All rights reserved.)
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- 2015
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14. [Cinacalcet in the management of normocalcaemic secondary hyperparathyroidism after kidney transplantation: one-year follow-up multicentre study].
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Torregrosa JV, Morales E, Díaz JM, Crespo J, Bravo J, Gómez G, Gentil MA, Rodríguez-Benot A, Rodríguez-García M, López-Jiménez V, Gutiérrez-Dalmau Á, Jimeno L, Pérez-Sáez MJ, Romero R, and Gómez-Alamillo C
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- Cinacalcet, Female, Follow-Up Studies, Humans, Male, Middle Aged, Retrospective Studies, Time Factors, Hyperparathyroidism, Secondary drug therapy, Kidney Transplantation, Naphthalenes therapeutic use, Postoperative Complications drug therapy
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Background: The effect of cinacalcet in patients with persistent secondary hyperparathyroidism (SHPT) after kidney transplantation (RT) has mainly been reported in patients with secondary hypercalcaemia., Objectives: Our objective was to assess the long-term effect of cinacalcet on patients with a RT and normocalcaemic SHPT., Methods: A one-year multicentre, observational, retrospective study that included kidney recipients with SHPT (intact parathyroid hormone [iPTH] >120 pg/ml) and calcium levels within the normal range (8.4-10.2 mg/dl). Patients began treatment with cinacalcet in clinical practice., Results: 32 patients with a mean age (standard deviation [SD]) of 54 (11) years, 56% male, were included in the study. Treatment with cinacalcet began a median of 16 months after RT (median dose of 30 mg/day). Levels of iPTH decreased from a median (P25, P75) of 364 (220, 531) pg/ml at the start of the study to 187 (98, 320) after 6 months (48.6% reduction, P=.001) and to 145 (91, 195) after 12 months (60.2% reduction, P=.001), without there being changes in calcium and phosphorus levels (P=.214 and P=.216, respectively). No changes were observed in kidney function or anti-calcineuric drug levels. 3.1% of patients discontinued cinacalcet due to intolerance and 6.2% due to a lack of efficacy., Conclusions: In patients with normocalcaemic SHPT after RT, cinacalcet improves the control of serum PTH values without causing changes to calcaemia, phosphataemia or kidney function. Cinacalcet showed good tolerability.
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- 2014
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15. [Rhabdomyolysis due to muscle enzyme deficiencies].
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Toledo Rojas R, López Jiménez V, Martín Reyes G, Torres Rueda A, and Frutos Sanz MA
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- Acute Kidney Injury etiology, Adult, Humans, Male, Rhabdomyolysis complications, Carnitine O-Palmitoyltransferase deficiency, Glycogen Phosphorylase, Muscle Form deficiency, Rhabdomyolysis etiology
- Abstract
Rhabdomyolysis is a syndrome characterized by injure of skeletal muscle with the release of intracellular constituents into the circulation. Acute renal failure is a common complication and is the leading cause of morbidity and mortality in these patients. The most common aetiology is traumatisms, muscle compressions and extreme exertions. Most commonly, the cause of rhabdomyolysis is evident from the careful clinical history. Nevertheless, when the precipitant is not obvious the diagnosis is difficult and a raised clinical suspicion is required. We should investigate used medication or drugs, infections, electrolyte abnormalities and a number of inherited enzyme deficiencies, in which cases the muscle is unable to use available energy. We report two clinical cases of acute renal failure due to rhabdomyolysis by metabolic myopathies due to a carnitine palmitoyltransferase deficiency on the one hand and by myophosphorylase deficiency on the other. We describe their clinical features and progress.
- Published
- 2009
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