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Síndrome de Behçet em idade pediátrica
Get PDFIntroduction: Behçet´s syndrome is a systemic vasculitis characterized by recurrent oral and/or genital ulcers, and several systemic manifestations. The authors describe the case of a pediatric-onset Behçet´s syndrome.
Case report: An 11-year-old boy was referred to the Pediatric consultation after two episodes of great saphenous vein thrombophlebitis. He had experienced daily oral aphthae for the past three years, and various episodes of folliculitis with pustule formation. Laboratory study was normal. The boy showed no signs of uveitis. The diagnosis of Behçet´s syndrome diagnosis was established according to the international criteria, with positive HLA- B51 testing. Colchicine was initiated, with favourable response.
Conclusions: Due to clinical feature overlap with other conditions, Behçet’s syndrome diagnosis remains challenging. Consensus pediatric classification criteria developed in 2016 enabled greater sensitivity and earlier diagnosis.info:eu-repo/semantics/publishedVersio
Comparison of Kristjansson Respiratory Score and Wang Respiratory Score in infants with bronchiolitis in a hospital emergency department
Get PDFObjective: Several respiratory scores have been created to evaluate bronchiolitis' severity level, but it is still not clear which is the best score. The aim of this study is to compare the Wang Respiratory Score (WRS) and the Kristjansson Respiratory Score (KRS) in the setting of an emergency room.
Methods: We performed a prospective observational study with 60 infants with bronchiolitis admitted to a paediatric emergency department. For both scores, we assessed inter-rater reliability between two different health professionals (physician and physiotherapist), internal consistency, and correlation with SpO2 testing the intraclass-correlation coefficient (ICC), weighted kappa, Cronbach
α
coefficient and Spearman tests, respectively.
Results: The inter-rater reliability was higher in KRS (ICC 0.79) and the Cronbach
α
and weighted kappa had similar values in KRS versus WRS. The correlation between the KRS/WRS and SpO2 was poor/moderate upon admission and discharge for the first observer and the second observer.
Conclusions: While the internal consistency was similar in both scores, inter-rater reliability of KRS was higher than WRS, which allows us to conclude that it would have more consistent results when used to assess bronchiolitis' level of severity by health personnel in a busy hospital emergency room.info:eu-repo/semantics/publishedVersio
Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study
Get PDFBackground: Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities. In this study, we aimed to assess the effect of the disease across health-related quality of life dimensions, in both carriers of the mutation and patients, to compare health-related quality of life with general population, as well as to explore health-related quality of life prognostic factors among patients, including disease progression and treatment.
Methods: This study was a multi-institutional, longitudinal, prospective, observational study of hereditary Transthyretin Amyloidosis Polyneuropathy Portuguese adult subjects (621 asymptomatic carriers and 733 symptomatic patients) enrolled in the Transthyretin Amyloidosis Outcomes Survey. Health-related quality of life was captured with the preference-based instrument EQ-5D-3 L. For general population the dataset included all subjects enrolled in a representative national study (n = 1500). Different econometric models were specified; multivariate probit, generalized linear model and generalized estimating equations model; including demographic and clinical covariates.
Results: Hereditary Transthyretin Amyloidosis Polyneuropathy patients have their health status severely impaired in all quality of life dimensions and more anxiety/depression problems were found among asymptomatic carriers. No differences on utility were found between carriers and general population (p = 0.209). Among patients, the utility value is estimated to be 0.51 (0.021), a decrement of 0.27 as compared with general population utility. Higher disease duration, advanced disease stage and not receiving treatment are associated with impaired health-related quality of life. No differences were found between genders (p = 0.910) or between late (≥50 years) and early-onset patients (p = 0.254). The utility estimate ranged from 0.63 (0.009) in stage I to 0.01 (0.005) in stage IV.
Conclusions: Hereditary Transthyretin Amyloidosis Polyneuropathy symptoms and progressive associated disabilities substantially decrease patient's health-related quality of life. Clinical strategies focused on health-related quality of life preservation such as close follow-up of asymptomatic carriers, prompt diagnosis and adequate, early treatment would benefit patient's long-term outcomes, slowing the progressive decline in health-related quality of life.info:eu-repo/semantics/publishedVersio
Exercise preconditioning prevents left ventricular dysfunction and remodeling in monocrotaline-induced pulmonary hypertension
Get PDFBackground: Despite pulmonary arterial hypertension (PAH) directly affects the right ventricle (RV), important structural, functional, and molecular changes also occur in left ventricle (LV). The objective of our study was to analyze the hypothetical cardioprotective effects of exercise preconditioning on LV in rats with monocrotaline (MCT)-induced PAH.
Methods: Forty male Wistar rats were randomly separated in sedentary (SED) and trained group (EX; running sessions of 60 min/day, 5 days/wk, at 25 m/min, for 4 weeks). After 4 weeks, animals were injected with MCT (60 mg/kg; SED + MCT; EX + MCT) or vehicle (SED + V). Following an additional period of 4 weeks where all animals remained sedentary, we completed LV hemodynamic evaluation in baseline and isovolumic conditions and collected LV samples for histological and molecular analysis.
Results: Preconditioning with exercise was capable to restore LV systolic and diastolic dysfunction in both baseline and isovolumic conditions (P < .05). This improved was paralleled with prevention of LV cardiomyocytes atrophy, fibrosis, and endothelin 1 mRNA levels (P < .05).
Conclusions: Our findings suggest that exercise preconditioning can prevent LV dysfunction secondary to MCT-induced PAH, which is of particular interest for the familial form of the disease that is manifested by greater severity or earlier onset.info:eu-repo/semantics/publishedVersio
Diagnostic challenges of hypersensitivity pneumonitis with autoimmune features: Dealing with more than a coincidence?
No full textinfo:eu-repo/semantics/publishedVersio
Wearable Health Technology to Quantify the Functional Impact of Peripheral Neuropathy on Mobility in Parkinson’s Disease: A Systematic Review
Get PDFThe occurrence of peripheral neuropathy (PNP) is often observed in Parkinson's disease (PD) patients with a prevalence up to 55%, leading to more prominent functional deficits. Motor assessment with mobile health technologies allows high sensitivity and accuracy and is widely adopted in PD, but scarcely used for PNP assessments. This review provides a comprehensive overview of the methodologies and the most relevant features to investigate PNP and PD motor deficits with wearables. Because of the lack of studies investigating motor impairments in this specific subset of PNP-PD patients, Pubmed, Scopus, and Web of Science electronic databases were used to summarize the state of the art on PNP motor assessment with wearable technology and compare it with the existing evidence on PD. A total of 24 papers on PNP and 13 on PD were selected for data extraction: The main characteristics were described, highlighting major findings, clinical applications, and the most relevant features. The information from both groups (PNP and PD) was merged for defining future directions for the assessment of PNP-PD patients with wearable technology. We established suggestions on the assessment protocol aiming at accurate patient monitoring, targeting personalized treatments and strategies to prevent falls and to investigate PD and PNP motor characteristics.info:eu-repo/semantics/publishedVersio
SnapKi—An Inertial Easy-to-Adapt Wearable Textile Device for Movement Quantification of Neurological Patients
Get PDFThe development of wearable health systems has been the focus of many researchers who aim to find solutions in healthcare. Additionally, the large potential of textiles to integrate electronics, together with the comfort and usability they provide, has contributed to the development of smart garments in this area. In the field of neurological disorders with motor impairment, clinicians look for wearable devices that may provide quantification of movement symptoms. Neurological disorders affect different motion abilities thus requiring different needs in movement quantification. With this background we designed and developed an inertial textile-embedded wearable device that is adaptable to different movement-disorders quantification requirements. This adaptative device is composed of a low-power 9-axis inertial unit, a customised textile band and a web and Android cross application used for data collection, debug and calibration. The textile band comprises a snap buttons system that allows the attachment of the inertial unit, as well as its connection with the analog sensors through conductive textile. The resulting system is easily adaptable for quantification of multiple motor symptoms in different parts of the body, such as rigidity, tremor and bradykinesia assessments, gait analysis, among others. In our project, the system was applied for a specific use-case of wrist rigidity quantification during Deep Brain Stimulation surgeries, showing its high versatility and receiving very positive feedback from patients and doctors.info:eu-repo/semantics/publishedVersio
SINAS: Impacto na Melhoria da Qualidade em Cirurgia de Ambulatório num Centro Integrado de Cirurgia Ambulatório
Get PDFIntroduction: The National Health Assessment System is a system designed by the Portuguese Health Regulatory Entity in order to evaluate the overall quality of the health care institutions. One of the key areas evaluated by the National Health Assessment System is ambulatory surgery. The aim of this study is to demonstrate that the introduction of the National Health Assessment System evaluation at our ambulatory centre not only improved the overall quality of ambulatory surgery but also the quality of the clinical record entries.
Material and methods: A retrospective analysis was performed from the hospital's clinical database. The study was carried out at the ambulatory centre of the Hospital and University Centre of Porto, and included 100 consecutive surgical procedures, across all surgical specialties, previously selected by the National Health Assessment System audit performed in 2015 in our ambulatory surgery centre and other 46 surgical procedures performed in 2008 at our hospital, before the National Health Assessment System was implemented. The main outcome measure was the validation and record of the seven indicators of National Health Assessment System for ambulatory surgery.
Results: We have seen an improvement in all indicators after the National Health Assessment System implementation, except for criterion 4.
Discussion: Our study demonstrates that the introduction of the National Health Assessment System in our ambulatory centre resulted in the improvement in the quality of both of clinical practice, and clinical record keepingConclusion: We can conclude that the application of evaluation of quality indicators and benchmarking practices can be used to enhance healthcare outcomes.info:eu-repo/semantics/publishedVersio
Usher syndrome and Nebulin‐associated myopathy in a single patient due to variants in MYO7A and NEB
Get PDFIn a patient with Usher syndrome and atypical muscle complaints, we have identified two separate variants in MYO7A andNEB genes by exome sequencing. The homozygous variants in these two recessive genes could explain the full phenotype of our patient.The authors express their sincere gratitude to this family. UMIB is supported by National Funds through the FCT—Fundação para a Ciência e a Tecnologia (Portuguese national funding agency for science, research and technology) in the framework of the UID/Multi/00215/2019 project—Unit for Multidisciplinary Research in Biomedicine—UMIB/ICBAS/UP. Nuno Maia and Paula Jorge were awarded with Centro Hospitalar Universitário do Porto, 2017 PhD DEFI-CHUP and DEFI—2015 (145/12) grants, respectivelyinfo:eu-repo/semantics/publishedVersio