A5023553220- Cystic Fibrosis Research Advances
- Child Nutrition and Feeding Issues
- Neonatal Respiratory Health Research
- Vitamin D Research Studies
- Pregnancy and Medication Impact
- Eosinophilic Esophagitis
- Gut microbiota and health
- Infant Nutrition and Health
- Microscopic Colitis
- Child Nutrition and Water Access
- Inflammatory Bowel Disease
- Pediatric Hepatobiliary Diseases and Treatments
- Body Composition Measurement Techniques
- Digestive system and related health
- Intracranial Aneurysms: Treatment and Complications
- Immunodeficiency and Autoimmune Disorders
- Mobile Health and mHealth Applications
- Family and Disability Support Research
- Acute Ischemic Stroke Management
- Cerebrovascular and Carotid Artery Diseases
- Adolescent and Pediatric Healthcare
- Pharmaceutical studies and practices
- Tracheal and airway disorders
- Lysosomal Storage Disorders Research
Hospital Universitario Ramón y Cajal
2019-2025
Instituto Cajal
2025
Universidad de Alcalá
2024-2025
Instituto Ramón y Cajal de Investigación Sanitaria
2024
Cystic Fibrosis Trust
2020
Background Cystic fibrosis-related liver disease (CFRLD) is a health problem that can affect as many 30–40% of cystic fibrosis patients by the age 12 years. We studied epidemiology CFRLD thanks to first exclusively pediatric patient registry date. Methods Descriptive cross-sectional study. Information from medical records January 2018 December 2020 collected. was classified according European Society Paediatric Gastroenterology, Hepatology and Nutrition 2017 criteria. Results Data were...
Undernutrition is defined as “a condition resulting from imbalanced nutrition or abnormal utilization of nutrients.” In this paper, the term malnutrition used to refer undernutrition. Malnutrition may be driven by poor socioeconomic conditions disease, and it estimated that disease-related (DRM) impacts up 28% hospitalized children in Europe. results alterations gastrointestinal function lead malabsorption macro- micro-nutrients. It can altered gut motility a deficiency stomach acid, which...
Abstract This is a prospective, multicenter study of cohort 224 cystic fibrosis (CF) patients treated with CF transmembrane conductance regulator (CFTR) modulators (CFTRm). Our aim was to prospectively analyze the effect CFTRm treatment on fat‐soluble vitamin serum levels. Demographic and clinical data were recorded, levels analyzed at baseline, 6 12 months after starting treatment. Two groups separately: receiving dual therapy lumacaftor/ivacaftor or tezacaftor/ivacaftor (Lum/Tez+Iva),...
Background Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency, leading to fat malabsorption, malnutrition and abdominal discomfort. Until recently, no specific tool was available for assessing gastro-intestinal related quality of life (GI QOL) in CF. As the Horizon2020 project MyCyFAPP aims improve GI QOL by using a newly designed mobile application, sensitive reliable outcome measure needed. We aimed study applicability existing child-specific Pediatric Quality...
Abstract Objectives To study the health‐related quality of life (HRQoL) and its possible determinant factors in Spanish children with eosinophilic esophagitis (EoE) their parents. Methods Multicenter observational cross‐sectional study. The version Pediatric Quality Life Eosinophilic Esophagitis Module was filled out by EoE patients aged 8–18 Demographic, psychosocial, clinical variables were studied. Multiple linear regression performed to identify related HRQoL. Results A total 279 parents...
Introduction: In recent years, guidelines for vitamin D supplementation have been updated and prophylactic recommended doses increased in patients with cystic fibrosis (CF). Objective: To evaluate safety efficacy of these new recommendations. Results: Two cohorts pancreatic insufficient CF were compared before (cohort 1: 179 patients) after 2: 71 American Foundation European Society recommendations published. Cohort 2 received higher Vitamin doses: 1509 (1306–1711 95% CI) vs 1084 (983–1184...
Objetives/Background: Vitamin D Binding Protein (VDBP) and free vitamin are new markers that being studied as a possible of status. The main aim our study was to analyse the VDBP genotype quantify levels in sample cystic fibrosis (CF) patients. Methods: Multicentre, cross-sectional prospective including patients with CF, exocrine pancreatic insufficiency who were clinically stable. We investigated (total free) different haplotypes. Free measured using an electro-chemiluminescence assay....
Vitamin D-binding protein (VDBP) and free vitamin D are new markers that being studied as a possible of status. The main aim our study was to analyze the VDBP genotype quantify levels in sample cystic fibrosis (CF) patients.
Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and healthy diet with specific nutrient distribution. Nutritional status an indicator of disease prognosis survival. This study aimed at assessing the effectiveness self-management mobile app in supporting patients CF to achieve dietary goals set by nutrition guidelines. A clinical trial was conducted pancreatic insufficient children CF, followed six European centres, where developed within MyCyFAPP project...
The Paediatric Eosinophilic Oesophagitis Module (PedsQL-EoE) was developed in English as a valid and reliable questionnaire to assess health-related quality of life (HRQoL) children with EoE. aim this study evaluate the validity reliability PedsQL-EoE that previously adapted Spanish by our group.This cross-sectional multicentre conducted 36 paediatric gastroenterology units. Groups without dietary restrictions were studied separately. consists 33 items divided into seven factors....
Sanfilippo syndrome, or mucopolysaccharidosis type III (MPS III), is a rare lysosomal disease caused by congenital enzymatic deficiencies in heparan sulfate (HS) degradation, leading to organ dysfunction. The most severe hallmark of MPS comprises neurological alterations, although gastrointestinal symptoms (GISs) have also been shown be relevant many patients. Here, we explored the contribution gut microbiota GISs. We analyzed composition and functionality two siblings with same mutation...
Introduction: Limited studies have explored the impact of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators on glucose tolerance and insulin secretion in patients with CF, yielding varied results. This study aims to assess alterations metabolism over 24 52 weeks following CFTR modulator initiation a cohort pediatric adult CF. Materials Methods: A prospective longitudinal conducting oral test (OGTT) C-peptide levels. The rate at 60 min (ISR60) insulinogenic index...
When people with cystic fibrosis (PwCFs) are treated transmembrane conductance regulator protein modulator (CFTRm), it leads to changes in body composition. An easy, non-invasive, and reproducible method assess this is by means of electrical bioimpedance measurement, which complements the information provided already-known anthropometric parameters.
Background/Objectives: Early life gut microbiota plays a pivotal role in shaping immunity, metabolism, and overall health outcomes. This is relevant healthy infants but may be even more crucial with chronic devastating diseases, such as cystic fibrosis (CF). While the introduction of solid foods modifies composition colonic microbiota, less knowledge available on those CF. The aim this descriptive observational study was to assess fecal six exclusively breast-fed CF, then explore changes...
Based on the European and American Cystic Fibrosis (CF) consensus recommendations, an increase in vitamin D (VD) supplementation patients with CF insufficient or defficient levels was proposed. The objective of our study to determine safety efficacy this new protocol. Multicentre nonrandomized uncontrolled experimental study. Patients (<30 ng/mL) received increasing doses VD (between 800 10 000 IU/day). were followed up for 12 months, during which their nutritional status, pulmonary function...