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Article: Descriptive Analysis of Carrier and Affected Hereditary Fructose Intolerance in Women during Pregnancy.

Zuriaga, Estefanía / Santander, Sonia / Lomba, Laura / Izquierdo-García, Elsa / Luesma, María José

2024 Volume 12, Issue 5

Abstract: 1) Background: Hereditary fructose intolerance (HFI) is a rare autosomal recessive metabolic disorder resulting from aldolase B deficiency, requiring a fructose, sorbitol and sucrose (FSS)-free diet. Limited information exists on the relationship ... ...

Abstract	(1) Background: Hereditary fructose intolerance (HFI) is a rare autosomal recessive metabolic disorder resulting from aldolase B deficiency, requiring a fructose, sorbitol and sucrose (FSS)-free diet. Limited information exists on the relationship between pregnancy outcomes and HFI. This study aims to analyze pregnancy-related factors in a cohort of thirty Spanish women, with twenty-three being carriers and seven being HFI-affected (45 pregnancies). (2) Methods: A descriptive, cross-sectional and retrospective study utilized an anonymous questionnaire. (3) Results: Findings encompassed physical and emotional states, nutritional habits, pathology development and baby information. Notable results include improved physical and emotional states compared to the general population, with conventional analyses mostly within normal ranges. Persistent issues after pregnancy included hepatic steatosis, liver adenomas and hemangiomas. Carrier mothers' babies exhibited higher weight than those of patient mothers, while the weights of carrier children born with HFI were similar to disease-affected children. (4) Conclusions: Pregnant women with HFI did not significantly differ in physical and emotional states, except for nausea, vomiting, and cravings. Post-pregnancy, HFI patients and carriers exhibited persistent hepatic issues. Significantly, babies born to HFI-affected mothers had lower weights. This study sheds light on pregnancy outcomes in HFI, emphasizing potential complications and the need for ongoing monitoring and care.
Language	English
Publishing date	2024-02-29
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2721009-1
ISSN	2227-9032
ISSN	2227-9032
DOI	10.3390/healthcare12050573
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Article ; Online: Design of mobile and website health application devices for drug tolerability in hereditary fructose intolerance.

Izquierdo-García, Elsa / Lázaro-Cebas, Andrea / Montero Pastor, Berta / Such Díaz, Ana / Álvaro-Alonso, Elena Alba / López Guerra, Laura / Escobar-Rodríguez, Ismael

Orphanet journal of rare diseases

2024 Volume 19, Issue 1, Page(s) 12

Abstract: Background: Hereditary fructose intolerance (HFI) is a rare metabolic disease caused by aldolase B deficiency. The aim of our study was to analyse excipient tolerability in patients with HFI and other related diseases and to design mobile and website ... ...

Abstract	Background: Hereditary fructose intolerance (HFI) is a rare metabolic disease caused by aldolase B deficiency. The aim of our study was to analyse excipient tolerability in patients with HFI and other related diseases and to design mobile and website health applications to facilitate the search for drugs according to their tolerance. Results: A total of 555 excipients listed in the Spanish Medicines Agency database (July 2023) were classified as suitable for HFI patients, suitable with considerations ((glucose and glucose syrup, intravenous sucrose, oral mannitol, polydextrose, gums and carrageenans, ethanol, sulfite caramel and vanilla), not recommended (intravenous mannitol) and contraindicated (fructose, oral sucrose, invert sugar, sorbitol, maltitol, lactitol, isomaltitol, fruit syrups, honey, sucrose esters and sorbitol esters). Glucose and glucose syrup were classified as suitable with considerations due to its possible fructose content and their potential endogenous fructose production. For other related intolerances, wheat starch was contraindicated and oatmeal was not recommended in celiac disease; oral lactose and lactose-based coprocessed excipient (Cellactose®) were not recommended in lactose intolerance; and glucose, invert sugar and oral sucrose were not recommended in diabetes mellitus. The applications were named IntoMed®. Results are listed in order of tolerability (suitable drugs appear first and contraindicated drugs at the end), and they are accompanied by a note detailing their classified excipients. If a drug contains excipients within different categories, the overall classification will be the most restrictive. The apps are also able to classify substances with the same criteria if they act as active ingredients. The tools exhibited good usability (82.07 ± 13.46 points on the System Usability Scale [range: 0-100]) on a sample of HFI patients, their families and health care professionals. Conclusions: IntoMed® is a tool for finding information about the tolerability of drugs according to excipients for patients with HFI and other related intolerances, with good usability. It is a fast and reliable system that covers the current excipient legislation and expands on it with other specific information: HFI patients should be alert for excipients such as mannitol (especially in intravenous drugs), fruit syrups, honey, sulfite caramel or vanilla. Glucose might contain or produce fructose, and special precaution is needed because of potential errors in their composition.
MeSH term(s)	Humans ; Fructose Intolerance ; Excipients ; Lactose ; Fructose ; Mannitol ; Sorbitol ; Glucose ; Sucrose ; Sulfites
Chemical Substances	Excipients ; Lactose (J2B2A4N98G) ; Fructose (30237-26-4) ; Mannitol (3OWL53L36A) ; Sorbitol (506T60A25R) ; Glucose (IY9XDZ35W2) ; Sucrose (57-50-1) ; Sulfites
Language	English
Publishing date	2024-01-05
Publishing country	England
Document type	Journal Article
ZDB-ID	2225857-7
ISSN	1750-1172 ; 1750-1172
ISSN (online)	1750-1172
ISSN	1750-1172
DOI	10.1186/s13023-023-03011-x
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Article ; Online: Reply letter to the editor concerning the article 'Safety of Sars-Cov-2 vaccines administration for adult patients with hereditary fructose intolerance'.

Izquierdo-Garcia, Elsa / Alvaro Alonso, Elena Alba / Montero Pastor, Berta / Such Díaz, Ana / Escobar Rodríguez, Ismael

Human vaccines & immunotherapeutics

2022 Volume 18, Issue 5, Page(s) 2051414

Abstract: According to the current European medicines legislation, on the labeling is mandatory a warning contraindicating for hereditary fructose intolerance (HFI) patients medicines with oral or parenteral fructose and sorbitol, and oral sucrose, invert sugar, ... ...

Abstract	According to the current European medicines legislation, on the labeling is mandatory a warning contraindicating for hereditary fructose intolerance (HFI) patients medicines with oral or parenteral fructose and sorbitol, and oral sucrose, invert sugar, isomaltitol, lactitol and maltitol, but parenteral sucrose is not mentioned. Intravenous administration of sucrose does not increase blood glucose concentrations, because sucrose is poorly oxidized to CO
MeSH term(s)	Adult ; COVID-19/prevention & control ; COVID-19 Vaccines/adverse effects ; Fructose Intolerance/complications ; Humans ; SARS-CoV-2 ; Sucrose
Chemical Substances	COVID-19 Vaccines ; Sucrose (57-50-1)
Language	English
Publishing date	2022-03-31
Publishing country	United States
Document type	Journal Article ; Comment
ZDB-ID	2664176-8
ISSN	2164-554X ; 2164-5515
ISSN (online)	2164-554X
ISSN	2164-5515
DOI	10.1080/21645515.2022.2051414
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Article ; Online: Physicochemical and Microbiological Stability of Two Oral Solutions of Methadone Hydrochloride 10 mg/mL.

Álvaro-Alonso, Elena Alba / Lorenzo, Ma Paz / Gonzalez-Prieto, Andrea / Izquierdo-García, Elsa / Escobar-Rodríguez, Ismael / Aguilar-Ros, Antonio

Molecules (Basel, Switzerland)

2022 Volume 27, Issue 9

Abstract: In this article, we studied physicochemical and microbiological stability and determined the beyond-use date of two oral solutions of methadone in three storage conditions. For this, two oral solutions of methadone (10 mg/mL) were prepared, with and ... ...

Abstract	In this article, we studied physicochemical and microbiological stability and determined the beyond-use date of two oral solutions of methadone in three storage conditions. For this, two oral solutions of methadone (10 mg/mL) were prepared, with and without parabens, as preservatives. They were packed in amber glass vials kept unopened until the day of the test, and in a multi-dose umber glass bottle opened daily. They were stored at 5 ± 3 °C, 25 ± 2 °C and 40 ± 2 °C. pH, clarity, and organoleptic characteristics were obtained. A stability-indicating high-performance liquid chromatography method was used to determine methadone. Microbiological quality was studied and antimicrobial effectiveness testing was also determined following European Pharmacopoeia guidelines. Samples were analyzed at days 0, 7, 14, 21, 28, 42, 56, 70, and 91 in triplicate. After 91 days of storage, pH remained stable at about 6.5-7 in the two solutions, ensuring no risk of methadone precipitation. The organoleptic characteristics remained stable (colorless, odorless, and bitter taste). The absence of particles was confirmed. No differences were found with the use of preservatives. Methadone concentration remained within 95-105% in all samples. No microbial growth was observed. Hence, the two oral methadone solutions were physically and microbiologically stable at 5 ± 3 °C, 25 ± 2 °C, and 40 ± 2 °C for 91 days in closed and opened amber glass bottles.
MeSH term(s)	Amber ; Chromatography, High Pressure Liquid ; Drug Compounding ; Drug Stability ; Drug Storage ; Methadone ; Solutions
Chemical Substances	Amber ; Solutions ; Methadone (UC6VBE7V1Z)
Language	English
Publishing date	2022-04-28
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	1413402-0
ISSN	1420-3049 ; 1431-5165 ; 1420-3049
ISSN (online)	1420-3049
ISSN	1431-5165 ; 1420-3049
DOI	10.3390/molecules27092812
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Article ; Online: Social and health care needs in patients with hereditary fructose intolerance in Spain.

Izquierdo-García, Elsa / Escobar-Rodríguez, Ismael / Moreno-Villares, José Manuel / Iglesias-Peinado, Irene

Endocrinologia, diabetes y nutricion

2019 Volume 67, Issue 4, Page(s) 253–262

Abstract: Introduction: Hereditary fructose intolerance is a metabolic disease due to an aldolase B deficiency. Our objective was to ascertain the social and health care needs of those with this deficiency.: Material and methods: A prospective, observational ... ...

Title translation	Necesidades sociosanitarias en pacientes con intolerancia hereditaria a la fructosa en España.
Abstract	Introduction: Hereditary fructose intolerance is a metabolic disease due to an aldolase B deficiency. Our objective was to ascertain the social and health care needs of those with this deficiency. Material and methods: A prospective, observational study was performed. A survey of social and health care needs was conducted to hereditary fructose intolerance patients living in Spain. Results: Most patients had been diagnosed, mainly by genetic analysis in children and based on fructose overload in adults. Population surveyed had no sequelae (72.34%) or disability (64%), and 83.33% of children and 52.38% of adults were taking drugs (p <.05) (2.06 drugs on average). Most patients had attended medical visits in the past two years, mainly in metabolic disease units (42.5%) and/or nutrition units (42.5%), but less than a half attended reference centers (mostly children [p <0.05]). Although 48% were satisfied with health care, they felt discriminated in recreational activities, school, health and/or daily activities. The most reliable sources of information were the specialized care physician (69.39%) and patients' association (59.18%). Fifty-five percent reported no problem in any quality of life dimension, although some had problems in daily activities, pain, and anxiety. Conclusions: Although hereditary fructose intolerance is less disabling than other rare diseases, it is important to know the needs of those who suffer from it. Although time to diagnosis has shortened, the poorer health care and satisfaction with it perceived in adults makes it necessary to emphasize the needs of this population, and the critical need of training and information of health care professionals.
MeSH term(s)	Adolescent ; Adult ; Child ; Female ; Fructose Intolerance/genetics ; Fructose Intolerance/therapy ; Health Services Needs and Demand ; Humans ; Male ; Prospective Studies ; Self Report ; Social Factors ; Spain ; Young Adult
Language	Spanish
Publishing date	2019-08-07
Publishing country	Spain
Document type	Journal Article ; Observational Study
ISSN	2530-0180
ISSN (online)	2530-0180
DOI	10.1016/j.endinu.2019.06.005
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Article ; Online: Jarabes de medicamentos: errores en ficha técnica con posibles consecuencias en pacientes con intolerancia hereditaria a la fructosa.

Izquierdo-García, Elsa / Escobar Rodríguez, Ismael / Moreno-Villares, José Manuel / Iglesias Peinado, Irene

Anales de pediatria (Barcelona, Spain : 2003)

2017 Volume 87, Issue 6, Page(s) 351–353

Title translation	Drug syrups: Errors in drug labels with possible consequences in patients with heritary fructose intolerance.
MeSH term(s)	Child ; Dosage Forms ; Drug Labeling ; Fructose Intolerance/complications ; Glucose/adverse effects ; Glucose/analysis ; Humans ; Medication Errors
Chemical Substances	Dosage Forms ; Glucose (IY9XDZ35W2)
Language	Spanish
Publishing date	2017-03-21
Publishing country	Spain
Document type	Letter
ISSN	1695-9531
ISSN (online)	1695-9531
DOI	10.1016/j.anpedi.2017.02.004
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Article ; Online: Correction: Vitamin C and folate status in hereditary fructose intolerance.

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 / de Las Heras, Javier

European journal of clinical nutrition

2023 Volume 77, Issue 11, Page(s) 1102–1103

Language	English
Publishing date	2023-08-30
Publishing country	England
Document type	Published Erratum
ZDB-ID	639358-5
ISSN	1476-5640 ; 0954-3007
ISSN (online)	1476-5640
ISSN	0954-3007
DOI	10.1038/s41430-023-01334-3
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Article: The PANDEMYC Score. An Easily Applicable and Interpretable Model for Predicting Mortality Associated With COVID-19.

Torres-Macho, Juan / Ryan, Pablo / Valencia, Jorge / Pérez-Butragueño, Mario / Jiménez, Eva / Fontán-Vela, Mario / Izquierdo-García, Elsa / Fernandez-Jimenez, Inés / Álvaro-Alonso, Elena / Lazaro, Andrea / Alvarado, Marta / Notario, Helena / Resino, Salvador / Velez-Serrano, Daniel / Meca, Alejandro

Journal of clinical medicine

2020 Volume 9, Issue 10

Abstract: This study aimed to build an easily applicable prognostic model based on routine clinical, radiological, and laboratory data available at admission, to predict mortality in coronavirus 19 disease (COVID-19) hospitalized patients.: Methods: We ... ...

Abstract	This study aimed to build an easily applicable prognostic model based on routine clinical, radiological, and laboratory data available at admission, to predict mortality in coronavirus 19 disease (COVID-19) hospitalized patients. Methods: We retrospectively collected clinical information from 1968 patients admitted to a hospital. We built a predictive score based on a logistic regression model in which explicative variables were discretized using classification trees that facilitated the identification of the optimal sections in order to predict inpatient mortality in patients admitted with COVID-19. These sections were translated into a score indicating the probability of a patient's death, thus making the results easy to interpret. Results: Median age was 67 years, 1104 patients (56.4%) were male, and 325 (16.5%) died during hospitalization. Our final model identified nine key features: age, oxygen saturation, smoking, serum creatinine, lymphocytes, hemoglobin, platelets, C-reactive protein, and sodium at admission. The discrimination of the model was excellent in the training, validation, and test samples (AUC: 0.865, 0.808, and 0.883, respectively). We constructed a prognostic scale to determine the probability of death associated with each score. Conclusions: We designed an easily applicable predictive model for early identification of patients at high risk of death due to COVID-19 during hospitalization.
Keywords	covid19
Language	English
Publishing date	2020-09-23
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662592-1
ISSN	2077-0383
ISSN	2077-0383
DOI	10.3390/jcm9103066
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Article ; Online: Experiencia autorreferida en pacientes tratados con antivirales directos frente al virus de la hepatitis C.

Cañamares Orbis, Irene / Saez de la Fuente, Javier / Izquierdo García, Elsa / Esteban Alba, Concepción / Such Díaz, Ana / Escobar Rodríguez, Ismael

Farmacia hospitalaria : organo oficial de expresion cientifica de la Sociedad Espanola de Farmacia Hospitalaria

2016 Volume 40, Issue n06, Page(s) 569–578

Abstract: Background and objective: To learn about and analyze the self-reported treatment experience of HCV patients who started treatment with direct acting antivirals agents (DAA), at a real-time, proactive and integrated into the pharmaceutical care ... ...

Title translation	Self-reported experience in patients treated with Hepatitis C direct acting antivirals.
Abstract	Background and objective: To learn about and analyze the self-reported treatment experience of HCV patients who started treatment with direct acting antivirals agents (DAA), at a real-time, proactive and integrated into the pharmaceutical care healthcare process, using a prospective questionnaire completed by patients as clinical tool. Material and methods: Observational and cross-sectional study conducted between April (start of the National Strategic Plan) and December 2015 in the Outpatient Pharmacy Service. The questionnaire includes variables related to health related quality of life (HRQOL), adherence, adverse effects (AEs), satisfaction, and usefulness of the Pharmacy Service implemented training program. A descriptive analysis of all variables included in the study was conducted and the influence of different variables analyzed in the degree of adherence and HRQOL. The analysis of the differences was performed using chi-square test and simple logistic regression model for calculation of OR. We use SPSS version 20 program and statistical significance for values of p < 0.05 was considered. Results: 155 of the 226 surveys returned, with a response rate of 68.6%. Referring to the HRQOL (evaluation of physical and emotional state), 38.7% of patients reports that their physical and emotional state is much better from the start of treatment. The presence of EA and worse global information of their disease was associated with worse physical and emotional state (p < 0.05). Reported adherence was 84.5% and the treatment was evaluated as very good or good by 87% of patients. 52.9% had no adverse effects related to the medication and the training process performed by the specialist pharmacist at the first visit 96.7% of patients assessed as very good or good. Conclusions: Self-reported experience acquired through direct and constant contact with patients provides information on important aspects of treatment. We believe that these tools should be incorporated into pharmaceutical care procedures as a way to maintain continuity in patients direct contact. They also induce patients to a self-assessment of various aspects of their own treatment, which can help achieve greater involvement in it and can contribute to achieve the maximum health outcome in pharmacotherapy.
MeSH term(s)	Adolescent ; Adult ; Aged ; Ambulatory Care ; Antiviral Agents/therapeutic use ; Cross-Sectional Studies ; Female ; Hepatitis C/drug therapy ; Hepatitis C/psychology ; Humans ; Male ; Middle Aged ; Patient Compliance ; Patients ; Prospective Studies ; Quality of Life ; Self Report ; Young Adult
Chemical Substances	Antiviral Agents
Language	Spanish
Publishing date	2016-11-01
Publishing country	Spain
Document type	Journal Article ; Observational Study
ZDB-ID	1122680-8
ISSN	2171-8695 ; 1130-6343
ISSN (online)	2171-8695
ISSN	1130-6343
DOI	10.7399/fh.2016.40.6.10503
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Article ; Online: Detection of galactomannan content in generic piperacillin-tazobactam products.

Saez de la Fuente, Javier / Campelo-Gutierrez, Carolina / Izquierdo-Garcia, Elsa / Aznar, Esteban / Caro, Jose Manuel / Gimenez-Manzorro, Alvaro / Diaz, Benito Garcia / Becares, Javier

Infectious diseases (London, England)

2018 Volume 51, Issue 2, Page(s) 157–159

MeSH term(s)	Antimicrobial Stewardship ; Mannans ; Piperacillin ; Tazobactam
Chemical Substances	Mannans ; galactomannan (11078-30-1) ; Tazobactam (SE10G96M8W) ; Piperacillin (X00B0D5O0E)
Language	English
Publishing date	2018-10-29
Publishing country	England
Document type	Letter ; Comment
ZDB-ID	2839775-7
ISSN	2374-4243 ; 2374-4235
ISSN (online)	2374-4243
ISSN	2374-4235
DOI	10.1080/23744235.2018.1530451
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