Repositório da Unidade Local de Saúde São José
Institution's Scientific Repository
Recent Submissions
Cold Agglutinin Syndrome and Hemophagocytic Lymphohistiocytosis: An Unusual Combination Caused by Epstein-Barr Virus Infection.
Publication . Sousa Nunes, Beatriz; Gouveia, Catarina; Kjollerstrom, Paula; Farela Neves, João
Autoimmune hemolytic anemia (AIHA) and hemophagocytic lymphohistiocytosis (HLH) are rare complications of infectious mononucleosis. The authors describe a 12-year-old male with acute infectious mononucleosis, hepatitis, cholestasis, and an autoimmune hemolytic disorder caused by cold agglutinins IgM (anti-i specificity). Clinical deterioration with persistent fever, anemia, and hepatosplenomegaly was consistent with cold AIHA plus concomitant HLH. The patient was treated with corticosteroids and acyclovir, with an uneventful recovery. Although rare, cold agglutinin syndrome and HLH can complicate infectious mononucleosis and should be considered in a patient with clinical deterioration. Corticosteroids are the mainstay treatment of HLH and may be beneficial in infection-associated cold agglutinin syndrome.
Reconciliação Medicamentosa num Serviço de Urgência: um Processo de Melhoria Contínua
Publication . de Almeida Marinho Diniz, Ana Cristina; Pereira Borges Sousa, Anabela; Tartaglia Reis, Cláudia; Sardinha Vieira Ramos, Susana Maria; Máximo Barreiros, Paulo Mnuel; dos Santos Sousa, Paulo Jorge
Objetivos: estruturar o plano de melhoria para a reconciliação da medicação num serviço de urgência português.
Método: elaborou-se o projeto de melhoria com a metodologia Plan, Do, Act, Plan (PDSA), em um estudo observacional, descritivo, quantitativo. Na etapa Plan, participaram 282 pacientes admitidos em serviço de urgência, cujo familiar teve teleconsulta de Enfermagem; e 447 na etapa Study. Realizada a estruturação da teleconsulta com a técnica Identify, Situation, Background, Assessment e Recommendation (ISBAR); formação da equipe.
Resultados: na prática habitual dos enfermeiros, identificaram-se 4,6% (n=13) discrepâncias não intencionais entre a prescrição e a medicação em uso no domicílio. Observou-se o aumento para 9,4% (n=42) de discrepâncias após a estruturação da teleconsulta, que passou a incluir as questões sobre a medicação habitual. A interrupção, sobretudo de medicamentos que atuam no sistema nervoso central, dose/frequência incorreta, foi a discrepância mais frequente, posteriormente corrigida.
Conclusão: o estudo contribuiu para identificar/corrigir as falhas na reconciliação de medicação, a fim de evidenciar a importância da participação da Enfermagem e da família
Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases: Five-year Experience of a Pediatric Tertiary Hospital in Portugal.
Publication . Rebelo, Mafalda; Francisco, Telma; Perry da Câmara, Rosário; Pereira, Andreia; Iraneta, Amets; Amorim, Marta; Paiva Lopes, Maria João; Lopes da Silva, Rita; Cordeiro, Ana Isabel
Introduction: Neurocutaneous syndromes (NCS) are a heterogeneous group of conditions with multiorgan involvement and diverse manifestations, evolving throughout life with significant morbidity. A multidisciplinary approach to NCS patients has been advocated, although a specific model is not yet established. The aim of this study was 1) to describe the organization of the recently created Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases (MOCND) at a Portuguese pediatric tertiary hospital; 2) to share our institutional experience focusing on the most common conditions, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC); 3) to analyze the advantages of a multidisciplinary center and approach in NCS.
Methods: Retrospective analysis of 281 patients enrolled in the MOCND over the first five years of activity (October 2016 to December 2021), reviewing genetics, family history, clinical features, complications, and therapeutic strategies for NF1 and TSC.
Results: The clinic works weekly with a core team of pediatricians and pediatric neurologists supported by other specialties as needed. Of the 281 patients enrolled, 224 (79.7%) had identifiable syndromes such as NF1 (n = 105), TSC (n = 35), hypomelanosis of Ito (n = 11), Sturge-Weber syndrome (n = 5), and others. In NF1 patients, 41.0% had a positive family history, all manifested café-au-lait macules, 38.1% neurofibromas with 45.0% being large plexiform neurofibromas. Sixteen were under treatment with selumetinib. Genetic testing was performed in 82.9% of TSC patients with pathogenic variants found in TSC2 gene in 72.4% patients (82.7% if considered contiguous gene syndrome). Family history was positive in 31.4%. All TSC patients presented hypomelanotic macules and fulfilled diagnostic criteria. Fourteen patients were being treated with mTOR inhibitors.
Conclusion: Offering a systematic and multidisciplinary approach to NCS patients enables timely diagnosis, promotes a structured follow-up, and encourages discussion to outline management plans for optimal care to every patient, with significant impact on the quality of life of patients and families.
Expanding Left Bundle Branch Area Pacing - A New Step in Daily Practice.
Publication . Oliveira, Mário
Ortho-Monitorizer: A Portable Device for Quantitative Monitoring of Temperature and Pressure in a 3D-Printed Upper Limb Orthosis.
Publication . Antão, Matilde; Rodrigues, Inês; Quintão, Carla; Quaresma, Cláudia
Background: Adherence to wrist-hand orthoses in patients with musculoskeletal conditions, such as Carpal Tunnel Syndrome, is crucial for effective rehabilitation. However, objective methods for monitoring wear time and pressure distribution remain limited.
Purpose: This study presents the Ortho-Monitorizer, a portable 3D-printed sensor-integrated device designed to provide real-time, quantitative monitoring of temperature and pressure in upper limb orthoses. The objective is to evaluate the system's feasibility in detecting patient adherence and identifying critical pressure points.
Study design: A descriptive and cross-sectional study was conducted to develop and validate the device, including sensor integration and data acquisition.
Methods: Using a 3D scanner and Fusion 360 software, the orthoses were customized and printed in thermoplastic polyurethane. Data from healthy participants (n = 55) and patients with Carpal Tunnel Syndrome (n = 2) were collected through the Ortho-Monitorizer's application, using six sensors (three temperature and three pressure sensors) placed at clinically relevant anatomical points. Data were acquired over five hand positions, and normal reference values were established.
Results: Mean temperature values ranged between 29.5∘C and 32.5∘C, while pressure values varied from 0.00 MPa to 0.08 MPa across different hand positions. One CTS patient exhibited pressure values above normal thresholds in specific positions, correlating with discomfort and numbness reports. The device achieved a System Usability Scale (SUS) score of 86.8% (healthy participants) and 92.5% (CTS patients), indicating high usability and acceptance.
Conclusions: The Ortho-Monitorizer provides a non-invasive, objective method for monitoring patient adherence to orthotic treatments. By offering real-time tracking of critical parameters, it enhances clinical decision-making and patient outcomes. Future research should explore wireless integration and long-term clinical validation to further optimize its applicability.