75 results match your criteria: "Clinical University Hospital of Santiago de Compostela[Affiliation]"

Prognostic and survival factors in head and neck extra-nodal non-Hodgkin's lymphoma.

Oral Surg Oral Med Oral Pathol Oral Radiol

February 2025

Oral Sciences Research Group, Endodontics and Restorative Dentistry Unit, School of Medicine and Dentistry, University of Santiago de Compostela, Health Research Institute of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.

Article Synopsis
  • The objective of the study was to characterize primary extranodal non-Hodgkin lymphomas in the head and neck and assess patient survival and prognostic factors over a period from 2005 to 2016.
  • A total of 145 patients were analyzed, with 69.3% having localized disease and 91.8% being of B-cell phenotype, predominantly diffuse large B-cell lymphoma; most were treated with immunochemotherapy.
  • Survival rates at 3 and 5 years were 75% and 73%, with adverse prognostic indicators including low hemoglobin, high ß2-microglobulin, high LDH levels, advanced disease stages, and presence of B symptoms at diagnosis.*
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Article Synopsis
  • The study presents a rare case of two 9-year-old twins with galactose mutarotase (GALM) deficiency who have remained asymptomatic, even after gradually reintroducing galactose into their diet after a period of strict dietary restriction.
  • Initial detection was through newborn screening, leading to biochemical normalization after dietary adjustments; the genetic analysis revealed a complete deletion of a specific exon in the GALM gene.
  • The findings suggest that dietary liberalization may not negatively affect health in GALM deficiency, prompting further exploration into dietary management for this condition.
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Pain and heart rate variability in neonates receiving dexmedetomidine.

J Perinatol

November 2024

Neonatology, BCNatal, Barcelona Center for Maternal Fetal and Neonatal Medicine, Hospital Sant Joan de Déu and Hospital Clinic, University of Barcelona, Barcelona, Spain.

Introduction: Neonates in a NICU experience pain. Based on the cardiovascular and nociceptive systems link, a Newborn Infant Parasympathetic Evaluation (NIPE) monitor was designed to assess pain. The use of α-agonists as analgesic-sedative drugs has increased in neonates.

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Cribriform morular thyroid carcinoma (CMTC) has been included within the group of thyroid tumors of uncertain histogenesis in the recent World Health Organization classification of endocrine tumors. Most CMTCs occur in young euthyroid women with multiple (and bilateral) thyroid nodules in cases associated with familial adenomatous polyposis (FAP) or as single nodules in sporadic cases. CMTC generally behaves indolently, while aggressiveness and mortality are associated with high‑grade CMTC.

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Use of mycophenolate mofetil for the treatment of fibrotic hypersensitivity pneumonitis.

Am J Med Sci

January 2025

Pulmonology Department. Clinical-University Hospital of Santiago de Compostela. Santiago de Compostela, Spain; Health Research Institute of Santiago de Compostela (IDIS), Santiago de Compostela, Spain; Department of Medicine, Faculty of Medicine, University of Santiago de Compostela, Spain.

Introduction: The optimal treatment of fibrosing hypersensitivity pneumonitis (fHP) is not well understood. The aim of the study was to obtain information about the usefulness of mycophenolate mofetil (MMF) in its treatment.

Material And Methods: Quasi-experimental analysis of patients diagnosed with fHP and treated with MMF for one year, in a single centre.

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Bilateral Uveal Melanoma: An Insight into Genetic Predisposition in Four New Unrelated Patients and Review of Published Cases.

J Clin Med

May 2024

Grupo de Oftalmología Traslacional, Área Oncología, Instituto de Investigación Sanitaria de Santiago de Compostela (IDIS), 15706 Santiago de Compostela, Spain.

: Primary bilateral uveal melanoma (BUM) is an exceptionally rare form of uveal melanoma (UM). This study aimed to explore the potential existence of a genetic predisposition towards the development of BUM. : We employed an exome sequencing approach on germline DNA from four unrelated patients diagnosed with BUM, seeking pathogenic or likely pathogenic variants indicative of a genetic predisposition to UM.

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Article Synopsis
  • * It analyzed data from 49 patients, revealing a 63% penetrance and showing that men had earlier symptom onset compared to women, with only 20% presenting the classic AFD symptoms.
  • * Nearly 20% of patients experienced significant cardiovascular and renal events, and the study highlights the importance of understanding this variant's effects for better clinical management and awareness of AFD progression.
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Strict adherence to a diet is an essential pillar of long-term treatment for many inborn errors of metabolism (IEMs). Tools that educate patients about dietary management can positively condition adherence and prevent morbidity. We designed a free online dietary calculation program (Odimet, version 2.

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Background And Aims: The role of occupation is uncertain in the onset of inflammatory bowel diseases. The aim of this study is to identify if there is a role of occupation in these diseases.

Materials And Methods: A case-control study with incident cases with inflammatory bowel diseases was designed.

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Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria.

Nutrients

September 2023

Diagnosis and Treatment of Congenital Metabolic Diseases Unit (UDyTEMC), Department of Pediatrics, Faculty of Medicine, Clinical University Hospital of Santiago de Compostela, University of Santiago de Compostela, CIBERER, MetabERN, Institute of Clinical Research of Santiago de Compostela (IDIS), Rúa de San Francisco s/n, 15706 Santiago de Compostela, Spain.

Many adults with phenylketonuria (PKU) rely on medical nutrition therapy (MNT; low phenylalanine (Phe) diet with protein substitutes/medical foods) to maintain blood Phe concentrations within recommended ranges and prevent PKU-associated comorbidities. Despite disease detection through newborn screening and introduction of MNT as early as birth, adherence to MNT often deteriorates from childhood onwards, complicating the assessment of its effectiveness in the long term. Via a modified Delphi process, consensus (≥70% agreement) was sought on 19 statements among an international, multidisciplinary 13-member expert panel.

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Clinician-to-clinician electronic consultation in cardiology is also a digital health technology for cardiovascular care.

Eur Heart J Digit Health

March 2023

Instituto de Investigación Sanitaria de Santiago de Compostela. Choupana s/n, 15706, Santiago de Compostela, A Coruña, Spain.

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Quantitative PET tracking of intra-articularly administered Zr-peptide-decorated nanoemulsions.

J Control Release

April 2023

Nano-Oncology and Translational Therapeutics Group, Health Research Institute of Santiago de Compostela (IDIS), SERGAS, Santiago de Compostela 15706, Spain; University of Santiago de Compostela (USC), Santiago de Compostela 15706, Spain; Biomedical Research Networking Center on Oncology (CIBERONC), Madrid 28029, Spain; DIVERSA Technologies SL, Edificio Emprendia, Campus Vida Universidad de Santiago de Compostela 15782, Spain. Electronic address:

Intra-articular (IA) administration of drugs for the treatment of diseases such as rheumatoid arthritis, osteoarthritis and psoriatic arthritis is a common strategy; however, the rapid clearance from the synovial fluid restricts their effectivity due to the limited retention time. Drug Delivery Systems (DDS) are currently being developed to increase their joint retention time. This study compares the biodistribution and retention time of a senolytic peptide (PEP), with potential application in osteoarthritis disease, and this senolytic peptide encapsulated in a DDS based on a lipid nanoemulsion (PEPNE) by using positron emission tomography (PET) imaging.

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The lipidomic and inflammatory profiles of visceral and subcutaneous adipose tissues are distinctly regulated by the SGLT2 inhibitor empagliflozin in Zucker diabetic fatty rats.

Biomed Pharmacother

May 2023

Cellular and Molecular Cardiology Research Unit, Institute of Biomedical Research and Xerencia de Xestión Integrada de Santiago (XXIS/SERGAS), Santiago de Compostela, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Institute of Health Carlos III, Madrid, Spain.

The pharmacological inhibition of sodium-glucose cotransporter 2 (SGLT2) has emerged as a treatment for patients with type 2 diabetes mellitus (T2DM), cardiovascular disease and/or other metabolic disturbances, although some of the mechanisms implicated in their beneficial effects are unknown. The SGLT2 inhibitor (SGLT2i) empagliflozin has been suggested as a regulator of adiposity, energy metabolism, and systemic inflammation in adipose tissue. The aim of our study was to evaluate the impact of a 6-week-empagliflozin treatment on the lipidome of visceral (VAT) and subcutaneous adipose tissue (SAT) from diabetic obese Zucker Diabetic Fatty (ZDF) rats using an untargeted metabolomics approach.

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Cribriform morular thyroid carcinoma (CMTC) is a rare malignant thyroid tumor with a peculiar growth pattern secondary to permanent activation of the WNT/β-catenin pathway. CMTC may be associated with familial adenomatous polyposis or sporadic; it shares morphological features with papillary thyroid carcinoma (PTC) and was considered a variant of PTC in the 2017 WHO classification of tumors of endocrine organs. The new 5th edition of the WHO classification of endocrine and neuroendocrine tumors considered CMTC an independent thyroid neoplasm of uncertain histogenesis.

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Identification of Clinical Variants beyond the Exome in Inborn Errors of Metabolism.

Int J Mol Sci

October 2022

Centro de Diagnóstico de Enfermedades Moleculares, Centro de Biología Molecular, Departamento de Biología Molecular, Universidad Autónoma de Madrid, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), IdiPAZ, 28049 Madrid, Spain.

Article Synopsis
  • - Inborn errors of metabolism (IEM) are rare diseases present in 1 in every 1000 newborns, and next-generation sequencing is improving diagnosis rates, but it’s not always 100% accurate.
  • - This study utilized a personalized multi-omics approach (metabolomic, genomic, transcriptomic) and functional genomics to help diagnose six unresolved IEM cases, including disorders like galactosemia and maple syrup urine disease.
  • - Researchers identified eight novel genetic variants that affect gene splicing and expression, demonstrating that combining different omics technologies can effectively resolve complex clinical cases.
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Background: Early treated patients with phenylketonuria (PKU) often become lost to follow-up from adolescence onwards due to the historical focus of PKU care on the pediatric population and lack of programs facilitating the transition to adulthood. As a result, evidence on the management of adolescents and young adults with PKU is limited.

Methods: Two meetings were held with a multidisciplinary international panel of 25 experts in PKU and comorbidities frequently experienced by patients with PKU.

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Glucose transporter 1 deficiency syndrome (GLUT1DS) is a neurometabolic disorder caused by haploinsufficiency of the GLUT1 glucose transporter (encoded by SLC2A1) leading to defective glucose transport across the blood-brain barrier. This work describes the genetic analysis of 56 patients with clinical or biochemical GLUT1DS hallmarks. 55.

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Thyroid cancer is the malignant tumor that is increasing most rapidly in the world, mainly at the expense of sporadic papillary thyroid carcinoma. The somatic alterations involved in the pathogenesis of sporadic follicular cell derived tumors are well recognized, while the predisposing alterations implicated in hereditary follicular tumors are less well known. Since the genetic background of syndromic familial non-medullary carcinoma has been well established, here we review the pathogenesis of non-syndromic familial non-medullary carcinoma emphasizing those aspects that may be useful in clinical and pathological diagnosis.

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Diagnostic Interval in Extranodal Non-Hodgkin Head and Neck Lymphomas.

J Clin Med

February 2022

Department of Surgery and Medical-Surgical Specialties, School of Medicine and Dentistry, Universidade de Santiago de Compostela, Santiago de Compostela, 15705 La Coruña, Spain.

The aim of this study is to measure the diagnostic interval (DI) of primary extranodal non-Hodgkin lymphomas (PE-NHL) affecting the head and neck and to discover any associated factors. With this aim, we performed a retrospective observational study in northwestern Spain on patients diagnosed between 1 January 2005 and 1 January 2016. A search was made across the electronic health records of the public health system of this region (SERGAS).

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Purpose: Genetic variants in genes involved in the distribution, metabolism, accumulation or repair of lesions are likely to influence the response of drugs used in the treatment of Head and Neck Cancer (HNC). We examine the effect of 36 SNPs on clinical outcomes in patients with locally advanced HNC who were receiving platinum-based chemoradiotherapy (CRT).

Methods: These SNPs were genotyped in 110 patients using the iPLEX Gold assay on the MassARRAY method in blood DNA samples and used Kaplan-Meier and Cox regression analyses to compare genotype groups with the survival.

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The benefit of complete revascularization in elderly patients with non-ST elevation myocardial infarction (NSTEMI), and multivessel disease remains debated (MVD). The aim of our study was to determine the current long-term prognostic benefit of complete revascularization in this population. A retrospective cohort study of 1722 consecutive elderly NSTEMI patients was performed.

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Article Synopsis
  • The study examines the long-term effects of cardiac resynchronization therapy (CRT) on patients with heart failure and reduced left ventricular ejection fraction (LVEF), focusing on changes in LVEF and their impact on mortality.
  • It involved 328 heart failure patients over an average follow-up of 4.2 years, observing significant improvements in LVEF and left ventricular end-systolic volume (LVESV) during the first year post-CRT, with some gradual changes continuing over time.
  • The findings suggest that ongoing measurements of LVEF after CRT provide better insights into individual mortality risk than a single measurement, highlighting the importance of reverse remodeling, especially in the initial year.
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Anaplastic thyroid carcinoma (ATC) and poorly differentiated thyroid carcinoma (PDTC) have limited treatment options, and immune profiling may help select patients for immunotherapy. The prevalence and relevance of programmed death-1 ligand (PD-L1) expression and the presence of immune cells in ATC and PDTC has not yet been well established. The present study investigated PD-L1 expression (clone 22C3) and cells in the tumor microenvironment (TME), including tumor-infiltrating lymphocytes (TILs), tumor-associated macrophages (TAMs) and dendritic cells, in whole tissue sections of 15 cases of ATC and 13 cases of PDTC.

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Objectives: To evaluate the success rate of glucocorticoid discontinuation and to study which factors are associated with successful discontinuation.

Methods: Data from two treat-to-target studies, BeSt (target Disease Activity Score (DAS) ≤2.4) and IMPROVED (target DAS <1.

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Edelfosine nanoemulsions inhibit tumor growth of triple negative breast cancer in zebrafish xenograft model.

Sci Rep

May 2021

Nano-Oncology and Translational Therapeutics Unit, Health Research Institute of Santiago de Compostela (IDIS), Clinical University Hospital of Santiago de Compostela (CHUS), SERGAS, Santiago de Compostela, Spain.

Triple negative breast cancer (TNBC) is known for being very aggressive, heterogeneous and highly metastatic. The standard of care treatment is still chemotherapy, with adjacent toxicity and low efficacy, highlighting the need for alternative and more effective therapeutic strategies. Edelfosine, an alkyl-lysophospholipid, has proved to be a promising therapy for several cancer types, upon delivery in lipid nanoparticles.

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