Search

Your search keyword '"Cañedo Villarroya, Elvira"' showing total 26 results
Start Over Author "Cañedo Villarroya, Elvira"
26 results on '"Cañedo Villarroya, Elvira"'

1. Correction: Vitamin C and folate status in hereditary fructose intolerance

Author

Cano, Ainara, Alcalde, Carlos, Belanger-Quintana, Amaya, Cañedo-Villarroya, Elvira, Ceberio, Leticia, Chumillas-Calzada, Silvia, Correcher, Patricia, Couce, María Luz, García-Arenas, Dolores, Gómez, Igor, Hernández, Tomás, Izquierdo-García, Elsa, Chicano, Dámaris Martínez, Morales, Montserrat, Pedrón-Giner, Consuelo, Jáuregui, Estrella Petrina, Peña-Quintana, Luis, Sánchez-Pintos, Paula, Serrano-Nieto, Juliana, Suarez, María Unceta, Miñana, Isidro Vitoria, and de las Heras, Javier

Published
2023
Full Text
View/download PDF

2. Vitamin C and folate status in hereditary fructose intolerance

Author

Cano, Ainara, Alcalde, Carlos, Belanger-Quintana, Amaya, Cañedo-Villarroya, Elvira, Ceberio, Leticia, Chumillas-Calzada, Silvia, Correcher, Patricia, Couce, María Luz, García-Arenas, Dolores, Gómez, Igor, Hernández, Tomás, Izquierdo-García, Elsa, Chicano, Dámaris Martínez, Morales, Montserrat, Pedrón-Giner, Consuelo, Jáuregui, Estrella Petrina, Peña-Quintana, Luis, Sánchez-Pintos, Paula, Serrano-Nieto, Juliana, Suarez, María Unceta, Miñana, Isidro Vitoria, and de las Heras, Javier

Published
2022
Full Text
View/download PDF

3. FRI-153 High prevalence of steatotic liver disease and systemic inflammation in hereditary fructose intolerance (HFI) patients independent of age, BMI and the presence of metabolic syndrome

Author

Delgado, Teresa Cardoso, primary, Cano, Ainara, additional, Mercado-Gómez, Maria, additional, Buque, Xabier, additional, Alonso, Cristina, additional, Serrano-Macia, Marina, additional, Alcalde, Carlos, additional, Belanger-Quintana, Amaya, additional, Cañedo-Villarroya, Elvira, additional, Hualde, Leticia Ceberia, additional, Chumillas-Calzada, Silvia, additional, Correcher, Patricia, additional, García-Arenas, Dolores, additional, Gómez, Igor, additional, Hernández, Tomáz, additional, Izquierdo-García, Elsa, additional, Chicano, Dámaris Martínez, additional, Morales, Montserrat, additional, Pedrón-Giner, Consuelo, additional, Jáuregui, Estrella Petrina, additional, Peña, Luis, additional, Sánchez-Pintos, Paula, additional, Serrano-Nieto, Juliana, additional, Suárez, Maria Unceta, additional, Miñana, Isidro Vitoria, additional, Larena, Jose Alejandro, additional, Couce, María Luz, additional, Martínez-Chantar, María Luz, additional, Aspichueta, Patricia, additional, and de las Heras Montero, Javier, additional

Published
2024
Full Text
View/download PDF

8. Newborn screening for propionic, methylmalonic acidemia and vitamin B12 deficiency. Analysis of 588,793 newborns

Author

Martín-Rivada, Álvaro, primary, Cambra Conejero, Ana, additional, Martín-Hernández, Elena, additional, Moráis López, Ana, additional, Bélanger-Quintana, Amaya, additional, Cañedo Villarroya, Elvira, additional, Quijada-Fraile, Pilar, additional, Bellusci, Marcelo, additional, Chumillas Calzada, Silvia, additional, Bergua Martínez, Ana, additional, Stanescu, Sinziana, additional, Martínez-Pardo Casanova, Mercedes, additional, Ruíz-Sala, Pedro, additional, Ugarte, Magdalena, additional, Pérez González, Belén, additional, and Pedrón-Giner, Consuelo, additional

Published
2022
Full Text
View/download PDF

9. Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients

Author

Bélanger-Quintana, Amaya, primary, Arrieta Blanco, Francisco, additional, Barrio-Carreras, Delia, additional, Bergua Martínez, Ana, additional, Cañedo Villarroya, Elvira, additional, García-Silva, María Teresa, additional, Lama More, Rosa, additional, Martín-Hernández, Elena, additional, López, Ana Moráis, additional, Morales-Conejo, Montserrat, additional, Pedrón-Giner, Consuelo, additional, Quijada-Fraile, Pilar, additional, Stanescu, Sinziana, additional, and Casanova, Mercedes Martínez-Pardo, additional

Published
2022
Full Text
View/download PDF

10. Vitamin C and folate status in hereditary fructose intolerance

Author

Pediatría, Pediatria, Cano San José, Ainara, Alcalde, Carlos, Belanger Quintana, Amaya, Cañedo Villarroya, Elvira, Ceberio, Leticia, Chumillas Calzada, Silvia, Correcher, Patricia, Couce, María Luz, García Arenas, Dolores, Gómez, Igor, Hernández, Tomás, Izquierdo García, Elsa, Martínez Chicano, Dámaris, Morales, Montserrat, Pedrón Giner, Consuelo, Petrina Jáuregui, Estrella, Peña Quintana, Luis, Sánchez Pintos, Paula, Serrano Nieto, Juliana, Unceta Suárez, María, Vitoria Miñana, Isidro, De las Heras Montero, Javier Adolfo, Pediatría, Pediatria, Cano San José, Ainara, Alcalde, Carlos, Belanger Quintana, Amaya, Cañedo Villarroya, Elvira, Ceberio, Leticia, Chumillas Calzada, Silvia, Correcher, Patricia, Couce, María Luz, García Arenas, Dolores, Gómez, Igor, Hernández, Tomás, Izquierdo García, Elsa, Martínez Chicano, Dámaris, Morales, Montserrat, Pedrón Giner, Consuelo, Petrina Jáuregui, Estrella, Peña Quintana, Luis, Sánchez Pintos, Paula, Serrano Nieto, Juliana, Unceta Suárez, María, Vitoria Miñana, Isidro, and De las Heras Montero, Javier Adolfo

Abstract

Background Hereditary fructose intolerance (HFI) is a rare inborn error of fructose metabolism caused by the deficiency of aldolase B. Since treatment consists of a fructose-, sucrose- and sorbitol-restrictive diet for life, patients are at risk of presenting vitamin deficiencies. Although there is no published data on the status of these vitamins in HFI patients, supplementation with vitamin C and folic acid is common. Therefore, the aim of this study was to assess vitamin C and folate status and supplementation practices in a nationwide cohort of HFI patients. Methods Vitamin C and folic acid dietary intake, supplementation and circulating levels were assessed in 32 HFI patients and 32 age- and sex-matched healthy controls. Results Most of the HFI participants presented vitamin C (96.7%) and folate (90%) dietary intake below the recommended population reference intake. Up to 69% received vitamin C and 50% folic acid supplementation. Among HFI patients, 15.6% presented vitamin C and 3.1% folate deficiency. The amount of vitamin C supplementation and plasma levels correlated positively (R = 0.443; p = 0.011). Interestingly, a higher percentage of non-supplemented HFI patients were vitamin C deficient when compared to supplemented HFI patients (30% vs. 9.1%; p = 0.01) and to healthy controls (30% vs. 3.1%; p < 0.001). Conclusions Our results provide evidence for the first time supporting vitamin C supplementation in HFI. There is great heterogeneity in vitamin supplementation practices and, despite follow-up at specialised centres, vitamin C deficiency is common. Further research is warranted to establish optimal doses of vitamin C and the need for folic acid supplementation in HFI.

Published
2022

13. Transferrin Isoforms, Old but New Biomarkers in Hereditary Fructose Intolerance

Author

Cano, Ainara, primary, Alcalde, Carlos, additional, Belanger-Quintana, Amaya, additional, Cañedo-Villarroya, Elvira, additional, Ceberio, Leticia, additional, Chumillas-Calzada, Silvia, additional, Correcher, Patricia, additional, Couce, María Luz, additional, García-Arenas, Dolores, additional, Gómez, Igor, additional, Hernández, Tomás, additional, Izquierdo-García, Elsa, additional, Martínez Chicano, Dámaris, additional, Morales, Montserrat, additional, Pedrón-Giner, Consuelo, additional, Petrina Jáuregui, Estrella, additional, Peña-Quintana, Luis, additional, Sánchez-Pintos, Paula, additional, Serrano-Nieto, Juliana, additional, Unceta Suarez, María, additional, Vitoria Miñana, Isidro, additional, and de las Heras, Javier, additional

Published
2021
Full Text
View/download PDF

15. Transferrin Isoforms, Old but New Biomarkers in Hereditary Fructose Intolerance

Author

Pediatría, Pediatria, Cano, Ainara, Alcalde, Carlos, Belanger Quintana, Amaya, Cañedo Villarroya, Elvira, Ceberio, Leticia, Chumillas Calzada, Silvia, Correcher, Patricia, Couce, María Luz, García Arenas, Dolores, Gómez, Igor, Hernández, Tomás, Izquierdo García, Elsa, Martínez Chicano, Dámaris, Morales, Montserrat, Pedrón Giner, Consuelo, Petrina Jáuregui, Estrella, Peña Quintana, Luis, Sánchez Pintos, Paula, Serrano Nieto, Juliana, Unceta Suárez, María, Vitoria Miñana, Isidro, De las Heras Montero, Javier Adolfo, Pediatría, Pediatria, Cano, Ainara, Alcalde, Carlos, Belanger Quintana, Amaya, Cañedo Villarroya, Elvira, Ceberio, Leticia, Chumillas Calzada, Silvia, Correcher, Patricia, Couce, María Luz, García Arenas, Dolores, Gómez, Igor, Hernández, Tomás, Izquierdo García, Elsa, Martínez Chicano, Dámaris, Morales, Montserrat, Pedrón Giner, Consuelo, Petrina Jáuregui, Estrella, Peña Quintana, Luis, Sánchez Pintos, Paula, Serrano Nieto, Juliana, Unceta Suárez, María, Vitoria Miñana, Isidro, and De las Heras Montero, Javier Adolfo

Abstract

Hereditary Fructose Intolerance (HFI) is an autosomal recessive inborn error of metabolism characterised by the deficiency of the hepatic enzyme aldolase B. Its treatment consists in adopting a fructose-, sucrose-, and sorbitol (FSS)-restrictive diet for life. Untreated HFI patients present an abnormal transferrin (Tf) glycosylation pattern due to the inhibition of mannose-6-phosphate isomerase by fructose-1-phosphate. Hence, elevated serum carbohydrate-deficient Tf (CDT) may allow the prompt detection of HFI. The CDT values improve when an FSS-restrictive diet is followed; however, previous data on CDT and fructose intake correlation are inconsistent. Therefore, we examined the complete serum sialoTf profile and correlated it with FSS dietary intake and with hepatic parameters in a cohort of paediatric and adult fructosemic patients. To do so, the profiles of serum sialoTf from genetically diagnosed HFI patients on an FSS-restricted diet (n = 37) and their age-, sex- and body mass index-paired controls (n = 32) were analysed by capillary zone electrophoresis. We found that in HFI patients, asialoTf correlated with dietary intake of sucrose (R = 0.575, p < 0.001) and FSS (R = 0.475, p = 0.008), and that pentasialoTf+hexasialoTf negatively correlated with dietary intake of fructose (R = −0.386, p = 0.024) and FSS (R = −0.400, p = 0.019). In addition, the tetrasialoTf/disialoTf ratio truthfully differentiated treated HFI patients from healthy controls, with an area under the ROC curve (AUROC) of 0.97, 92% sensitivity, 94% specificity and 93% accuracy.

Published
2021

16. Classic Ketogenic Diet and Modified Atkins Diet in SLC2A1 Positive and Negative Patients with Suspected GLUT1 Deficiency Syndrome: A Single Center Analysis of 18 Cases

Author

Ruiz Herrero, Jana, primary, Cañedo Villarroya, Elvira, additional, González Gutiérrez-Solana, Luis, additional, García Alcolea, Beatriz, additional, Gómez Fernández, Begoña, additional, Puerta Macfarland, Laura Andrea, additional, and Pedrón-Giner, Consuelo, additional

Published
2021
Full Text
View/download PDF

20. Pediatric parenteral nutrition: Clinical practice guidelines from the Spanish Society of Parenteral and Enteral Nutrition (SENPE), the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH)

Author

Pedrón, Consuelo, Cuervas-Mons Vendrell, Margarita, Galera Martínez, Rafael, Gómez López, Lilianne, Gomis Muñoz, Pilar, Irastorza Terradillos, Iñaki, Martínez Costa, Cecilia, Moreno Villares, José Manuel, Pérez-Portabella Maristany, Cleofé, Pozas del Río, M. Teresa, Redecillas Ferreiro, Susana E., Prieto Bozano, Gerardo, Balmaseda Serrano, Elena, Cañedo Villarroya, Elvira, Gutiérrez Junquera, Carolina, Morais López, Ana, Meavilla Olivas, Silvia, Navas López, Víctor Manuel, Rubio Murillo, María, Vives Piñera, Inmaculada, Vidal Casariego, Alfonso, Cuerda Compés, Cristina de la, Matía Martín, Pilar, Frías Soriano, Laura, Ruiz López, M. Dolores, Vaquerizo Alonso, Clara, and UAM. Departamento de Pediatría

Subjects
Medicina, Child, Newborn, Parenteral nutrition, Standardization
Abstract

Introduction: Parenteral nutrition (PN) in childhood is a treatment whose characteristics are highly variable depending on the age and pathology of the patient. Material and methods: The Standardization and Protocols Group of the Spanish Society for Parenteral and Enteral Nutrition (SENPE) is an interdisciplinary group formed by members of the SENPE, the Spanish Society of Gastroenterology, Hepatology and Pediatric Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH) that intends to update this issue. For this, a detailed review of the literature has been carried out, looking for the evidences that allow us to elaborate a Clinical Practice Guide following the criteria of the Oxford Center for Evidence-Based Medicine. Results: This manuscript summarizes the recommendations regarding indications, access routes, requirements, modifications in special situations, components of the mixtures, prescription and standardization, preparation, administration, monitoring, complications and home NP. The complete document is published as a monographic number. Conclusions: This guide is intended to support the prescription of pediatric PN. It provides the basis for rational decisions in the context of the existing evidence. No guidelines can take into account all of the often compelling individual clinical circumstances., Introducción: la nutrición parenteral (NP) en la infancia es un tratamiento cuyas características son muy variables en función de la edad y la patología que presente el paciente. Material y métodos: el grupo de Estandarización y Protocolos de la Sociedad Española de Nutrición Parenteral y Enteral (SENPE) es un grupo interdisciplinar formado por miembros de la SENPE, Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP) y Sociedad Española de Farmacia Hospitalaria (SEFH) que pretende poner al día este tema. Para ello, se ha realizado una revisión pormenorizada de la literatura buscando las evidencias que nos permiten elaborar una Guía de Práctica Clínica siguiendo los criterios del Oxford Centre for Evidence-Based Medicine. Resultados: este manuscrito expone de forma resumida las recomendaciones en cuanto a indicaciones, vías de acceso, requerimientos, modificaciones en situaciones especiales, componentes de las mezclas, prescripción y estandarización, preparación, administración, monitorización, complicaciones y NP domiciliaria. El documento completo se publica como número monográfico. Conclusiones: esta guía pretende servir de apoyo para la prescripción de la NP pediátrica. Constituye la base para tomar decisiones en el contexto de la evidencia existente. Ninguna guía puede tener en cuenta todas las circunstancias clínicas individuales

Published
2017

24. Control de carbohidratos en los medicamentos antiepilépticos para niños con dieta cetogénica.

Author

Prado, Silvia Martín, del Río, Maite Pozas, Villarroya, Elvira Cañedo, Giner, Consuelo Pedrón, Martín Prado, Silvia, Pozas Del Río, Maite, Cañedo Villarroya, Elvira, and Pedrón Giner, Consuelo

Abstract

Introduction: Introduction: patients who follow a ketogenic diet for the control of epileptic seizures must carry out a strict control of carbohydrates from the foods they eat and the medicines they are prescribed. In the initiation of a ketogenic diet and when a doctor prescribes a new medication, it is necessary to select the most appropriate pharmaceutical form so that the supply of excipients in the form of carbohydrates from the drugs is minimized. Objectives: the goal of the present paper was to compile a list of carbohydrate and caloric contents in antiepileptic drugs commonly used in pediatric neurology. Methods: in each medication included in the list, the content of excipients considered carbohydrates and derivatives that could influence the patient's ketosis was reviewed. The caloric content from carbohydrates and polyols in each medication was calculated. Results: the table provides the total carbohydrate and caloric content for antiepileptic medications in pediatric patients consuming the ketogenic diet. Conclusions: this table is intended to be a useful tool to help clinicians select a pharmaceutical form that is less likely to affect the ketogenic diet. Additionally, knowing the carbohydrate content of a new medication will allow adjustment of the diet to maintain ketosis. [ABSTRACT FROM AUTHOR]

Published
2022
Full Text
View/download PDF

25. [Ketogenic dietary therapies for epilepsy: Experience in 160 patients over 18 years].

Author

Ruiz Herrero J, Cañedo Villarroya E, García Peñas JJ, García Alcolea B, Gómez Fernández B, Puerta Macfarland LA, and Pedrón-Giner C

Abstract

Aim: Ketogenic dietary therapies (KDT) produce anticonvulsant and neuroprotective effects, reduce seizures and improve the cognitive state in patients with epilepsy. Our purpose was to evaluate the effects of KDT in children with refractory epilepsy (effectiveness, side effects, impact on nutritional status and growth)., Methods: A retrospective and prospective observational descriptive study was conducted in a Spanish tertiary hospital (January 2000 to December 2018). One hundred sixty pediatric patients with epilepsy were treated with KDT (82 males; mean age 5 years 9 months). Seizures, anti-epileptic drugs, anthropometric measures, side effects, and laboratory assessment were monitored baseline and at 3, 6, 12 and 24 months after the onset of KDT., Results: In these time intervals, the seizure-free patients were: 13.7, 12.5, 14.4 and 10.6%, respectively, and a reduction of seizures≥50% was achieved in 41.9, 37.5, 28.7 and 16.2%. Side effects were frequent, especially digestive disorders, hypercalciuria, hypoglycemia, hepatic dysfunction and dyslipidemia. Prealbumin, retinol binding protein, vitamin A and magnesium decreased significantly. Height was affected, especially in children below 2 years., Conclusions: KDT are effective for refractory epilepsy in children. However, adverse effects are frequent, and it may affect nutritional status and growth., (Copyright © 2021 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)

Published
2021
Full Text
View/download PDF

26. [Newborn Screening Program in the Community of Madrid: evaluation of positive cases.]

Author

Cambra Conejero A, Martínez Figueras L, Ortiz Temprado A, Blanco Soto P, Martín Rivada Á, Palomino Pérez L, Cañedo Villarroya E, Pedrón Giner C, Quijada Fraile P, Martín-Hernández E, García Silva MT, Chumillas Calzada S, Bellusci M, Belanger-Quintana A, Stanescu S, Martínez-Pardo Casanova M, Moráis López A, Bergua Martínez A, Ruiz-Salas P, Pérez González B, Ugarte M, and Ruano MLF

Subjects
Amino Acid Metabolism, Inborn Errors epidemiology, Carnitine analogs & derivatives, Carnitine blood, Cities, Female, Humans, Infant, Newborn, Lipid Metabolism, Inborn Errors epidemiology, Male, Predictive Value of Tests, Prevalence, Spain, Acyl-CoA Dehydrogenase deficiency, Amino Acid Metabolism, Inborn Errors diagnosis, Lipid Metabolism, Inborn Errors diagnosis, Neonatal Screening methods, Tandem Mass Spectrometry methods
Abstract

Objective: Tandem mass spectrometry (MS/MS) is being used for newborn screening since this laboratory testing technology increases the number of metabolic disorders that can be detected from dried blood-spot specimens. In the Community of Madrid, it was implemented in March 2011 and it includes 13 aminoacidopathies, fatty acid oxidation disorders and organic acidemias. The aim of this study was to describe our experience and evaluate the screening positive cases in a period of 9 years (2011-2019)., Methods: During the period of the study, a total of 592.822 neonates were screened with this expanded program by MS/MS in the Community of Madrid. Amino acids, acylcarnitines, and succinylacetone were quantified in all samples that met the quality criteria. Means, medians, percentiles and standard deviation of the analytes and ratios of interest were calculated., Results: 901 patients (0,15 %) with a positive screening test were referred to clinical evaluation. 230 patients were diagnosed of 30 different inborn errors of metabolism (prevalence 1:2577), 11 of which were not included as a target in the Community of Madrid newborn screening program. The global positive predictive value was 25,6 %. During this period of time, two false negative cases were detected. The most prevalent disorders were phenylketonuria/hyperphenylalaninemia and medium chain acyl-CoA dehydrogenase deficiency (1:6444 and 1:13174 respectively). 93 % of the patients were detected in the presymptomatic stage., Conclusions: During the last 9 years a large number of cases of IEM have been detected with an acceptable global positive predictive value. These results confirm the utility of inborn errors of metabolism newborn screening as a public health program., Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Published
2020

Catalog

Books, media, physical & digital resources
See catalog results