ABSTRACT
BACKGROUND: Urogenital schistosomiasis caused by Schistosoma haematobium is highly endemic in the municipality of Cubal in Angola. Currently, diagnosis is based on the observation of S. haematobium eggs in urine samples by microscopy but this method has low sensitivity. Few studies have been performed using molecular techniques in high-prevalence areas for the detection of S. haematobium. The objective of this study is to evaluate the usefulness of real-time PCR as a diagnostic technique for urogenital schistosomiasis among preschool-age children and its correlation with morbidity data. METHODS: A cross-sectional study was conducted in Cubal, Angola, involving 97 urine samples from preschool-age children analyzed by the dipstick test, microscopic examination of filtered urine, and real-time PCR. The diagnosis of urogenital schistosomiasis was based on microscopy and/or real-time PCR results. Clinical and ultrasonography evaluation was performed to rule out complications of schistosomiasis. RESULTS: We detected a total of 64.95% of samples positive by real-time PCR and 37.11% by microscopy. The sensitivity of parasitological diagnosis of urogenital schistosomiasis by real-time PCR and microscopy was 95.45% and 54.55%, respectively, and the sensitivity of real-time PCR compared with microscopy was 91.67%. A positive real-time PCR result was significantly related to older age (mean = 3.22 years), detection of eggs by microscopy, and abnormal urine dipstick results (18.56% with proteinuria, 31.96% with leukocyturia, and 31.96% with microhematuria) (p-value<0.05). Ultrasound analysis showed that 23.94% of children had urinary tract abnormalities, and it was significantly related to the real-time PCR diagnosis (p-value<0.05). CONCLUSIONS: Real-time PCR is a more sensitive technique than microscopy for urinary schistosomiasis diagnosis in preschool-age children in Cubal. This increase in sensitivity would allow earlier diagnosis and treatment, thus reducing the morbidity associated with schistosomiasis in its early stages.
Subject(s)
Real-Time Polymerase Chain Reaction , Schistosoma haematobium , Schistosomiasis haematobia , Sensitivity and Specificity , Humans , Angola/epidemiology , Schistosomiasis haematobia/diagnosis , Schistosomiasis haematobia/epidemiology , Schistosomiasis haematobia/urine , Child, Preschool , Cross-Sectional Studies , Real-Time Polymerase Chain Reaction/methods , Male , Female , Schistosoma haematobium/genetics , Schistosoma haematobium/isolation & purification , Animals , Prevalence , Microscopy/methods , Molecular Diagnostic Techniques/methodsABSTRACT
Accurate prediction and grading of gliomas play a crucial role in evaluating brain tumor progression, assessing overall prognosis, and treatment planning. In addition to neuroimaging techniques, identifying molecular biomarkers that can guide the diagnosis, prognosis and prediction of the response to therapy has aroused the interest of researchers in their use together with machine learning and deep learning models. Most of the research in this field has been model-centric, meaning it has been based on finding better performing algorithms. However, in practice, improving data quality can result in a better model. This study investigates a data-centric machine learning approach to determine their potential benefits in predicting glioma grades. We report six performance metrics to provide a complete picture of model performance. Experimental results indicate that standardization and oversizing the minority class increase the prediction performance of four popular machine learning models and two classifier ensembles applied on a low-imbalanced data set consisting of clinical factors and molecular biomarkers. The experiments also show that the two classifier ensembles significantly outperform three of the four standard prediction models. Furthermore, we conduct a comprehensive descriptive analysis of the glioma data set to identify relevant statistical characteristics and discover the most informative attributes using four feature ranking algorithms.
Subject(s)
Brain Neoplasms , Glioma , Machine Learning , Neoplasm Grading , Glioma/genetics , Glioma/pathology , Humans , Brain Neoplasms/genetics , Brain Neoplasms/pathology , Biomarkers, Tumor/genetics , Prognosis , AlgorithmsABSTRACT
Idiopathic Inflammatory Myopathies are rare conditions with several heterogeneous disease subtypes. They can range from limited muscle or skin involvement to severe, systemic, life-threatening disease. Although the etiology is unknown, some evidence suggests a role for external agents, particularly drugs. Herein, we present a case of a 71-year-old woman with chronic myeloid leukemia who developed imatinib-induced dermatomyositis sine dermatitis. The presentation was predominantly muscular, characterized by proximal muscle weakness and myalgia of the lower limbs, with positive anti-Mi2a antibodies. Spontaneous recovery was observed after drug discontinuation, without the need for immunosuppressive therapy. This is the first confirmed description of an imatinib-induced dermatomyositis sine dermatitis. It reflects the importance of a high awareness from rheumatologists and hematologists to accurately anticipate and identify similar situations.
Subject(s)
Dermatomyositis , Imatinib Mesylate , Humans , Female , Aged , Dermatomyositis/chemically induced , Dermatomyositis/diagnosis , Dermatomyositis/immunology , Imatinib Mesylate/adverse effects , Imatinib Mesylate/therapeutic use , Antineoplastic Agents/adverse effects , Antineoplastic Agents/therapeutic use , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Dermatitis/etiology , Dermatitis/diagnosis , Dermatitis/drug therapyABSTRACT
OBJECTIVES: During the COVID-19 pandemic, there was a significant impact on the management of non-COVID-19 related diseases, potentially increasing the incidence of paraneoplastic syndromes such as cancer-associated myositis (CAM).The aim of this study is to determine the incidence of CAM in our cohort before and after the COVID-19 pandemic onset. METHODS: We included patients with idiopathic inflammatory myopathy (IIM), diagnosed between June 2016 and June 2023. The patients were divided into two groups according to the date of IIM diagnosis. RESULTS: We included 132 patients; 65.1% (n=86) were diagnosed prior to and 34.9% (n=46) after the COVID-19 pandemic. The most common IIM was dermatomyositis (DM) before and after the COVID-19 pandemic onset (p=0.750). The most frequent myositis-specific antibody (MSA) before the COVID-19 pandemic was anti-Mi2 (15.1%). After the COVID-19 pandemic onset, anti-TIF1γ was the most common MSA (21.7%), with a significantly higher relative prevalence (p=0.006). The incidence of CAM was significantly higher after the COVID-19 pandemic onset (11 vs. 3 new cases, p<0.002). Patients with CAM more frequently had anti-TIF1γ-positivity (p<0.001) and a diagnosis after the pandemic (p=0.001) than non-CAM-IIM patients. No significant differences were found regarding vaccination status or previous COVID-19 infection in CAM and non-CAM-IIM patients. Diagnosis after the COVID-19 pandemic was an independent predictor of CAM among IIM patients (OR 0.012, 95% CI 0.000-0.400, p=0.013), regardless of age, sex or previous COVID-19 infection. CONCLUSIONS: There was a significant increase in the incidence of CAM after the COVID-19 pandemic. IIM diagnosis after the COVID-19 pandemic was an independent predictor of CAM.
Subject(s)
COVID-19 , Myositis , Neoplasms , Humans , Pandemics , Autoantibodies , COVID-19/epidemiology , Myositis/diagnosis , Neoplasms/epidemiologyABSTRACT
Complex, chronic or hard-to-heal wounds are a prevalent health problem worldwide, with significant physical, psychological and social consequences. This study aims to identify factors associated with the healing process of these wounds and develop a mobile application for wound care that incorporates these factors. A prospective multicentre cohort study was conducted in nine health units in Portugal, involving data collection through a mobile application by nurses from April to October 2022. The study followed 46 patients with 57 wounds for up to 5 weeks, conducting six evaluations. Healing time was the main outcome measure, analysed using the Mann-Whitney test and three Cox regression models to calculate risk ratios. The study sample comprised various wound types, with pressure ulcers being the most common (61.4%), followed by venous leg ulcers (17.5%) and diabetic foot ulcers (8.8%). Factors that were found to impair the wound healing process included chronic kidney disease (U = 13.50; p = 0.046), obesity (U = 18.0; p = 0.021), non-adherence to treatment (U = 1.0; p = 0.029) and interference of the wound with daily routines (U = 11.0; p = 0.028). Risk factors for delayed healing over time were identified as bone involvement (RR 3.91; p < 0.001), presence of odour (RR 3.36; p = 0.007), presence of neuropathy (RR 2.49; p = 0.002), use of anti-inflammatory drugs (RR 2.45; p = 0.011), stalled wound (RR 2.26; p = 0.022), greater width (RR 2.03; p = 0.002), greater depth (RR 1.72; p = 0.036) and a high score on the healing scale (RR 1.21; p = 0.001). Integrating the identified risk factors for delayed healing into the assessment of patients and incorporating them into a mobile application can enhance decision-making in wound care.
Subject(s)
Diabetic Foot , Varicose Ulcer , Humans , Cohort Studies , Prospective Studies , Wound Healing , Varicose Ulcer/therapy , Diabetic Foot/drug therapyABSTRACT
There is an ongoing effort to increase rare disease awareness amongst healthcare providers. This front is important and can help to address several challenges faced by rare disease patients, such as lengthy diagnosis times, difficulty in finding adequate providers of medical services and experts, and adequate treatment if one exists. On another front, there is the need for awareness among citizens and their support in the advocacy for public policies towards rare disease patients and families. Awareness campaigns are prevalent in social networks and fundraising events. In this poster, we present a complementary approach to engage society and promote rare disease awareness through children's literature and music. A Portuguese teenager wrote a book ('My Life with my sister'), describing simple and daily moments spent with her teenage sister affected by Sanfilippo syndrome. A professional illustrator designed and illustrated the book. The book is bilingual in Portuguese and English. The author, with the assistance of her music teacher, also composed a song which was recorded with the participation of professional musicians and made into a video clip telling their story and the books. The book and song promote the inclusion and love for people affected by rare diseases and their families. To increase outreach, sister organizations translated the book, adapted the song, and published/ recorded the material in Brazilian Portuguese and French. The proceeds from the sales go towards the Sanfilippo foundations in their respective countries to fund common research projects. The material is being advertised on social media, television, interviews, newspapers, podcasts, libraries, schools, bookshops, book fairs, and others. To date, more than eight hundred books have been sold to individuals and companies. The interviews and video clips add to more than twelve hundred views. The target audience is children, parents, teachers but also companies, and their employees.
ABSTRACT
BACKGROUND: Schistosomiasis is one of the most important neglected tropical diseases, with a great impact on public health and more than 200,000 deaths annually. Schistosoma haematobium causes urinary tract (UT) morbidity. Since schistosomiasis morbidity control programs focus on children older than 5 years, pre-school age children (PSAC) morbidity is not well known. METHODS: We conducted a cross-sectional study in Cubal (Angola) among 245 PSAC with the objective of evaluating the prevalence of S. haematobium infection, the intensity of infection, and associated morbidity. For this purpose, urine filtration test followed by microscopic visualization and ultrasound examinations were performed. RESULTS: The estimated overall prevalence of urogenital schistosomiasis was 30.2% (CI 95%; 24.5-35.9), with 20.3% (CI 95%; 15.3-25.3) of the samples analysed showing a high intensity of infection. A total of 54.5% (CI 95%; 47.6-61.8) of infected children presented UT lesions, showing a significant association between schistosomiasis infection and UT morbidity (p-value < 0.001). Bladder wall thickening was the most common lesion, being present in 100% of abnormal ultrasounds. We found that anaemia and severe malnutrition were not significantly associated with the development of UT lesions. CONCLUSIONS: S. haematobium infection in PSAC causes great UT detectable morbidities. Therefore, there is an evident need of including them in mass drug administration (MDA) campaigns and consequently the development of an adapted praziquantel treatment dosage for children under 2 years of age.
Subject(s)
Schistosomiasis haematobia , Animals , Humans , Child , Child, Preschool , Infant , Schistosomiasis haematobia/drug therapy , Schistosomiasis haematobia/epidemiology , Schistosomiasis haematobia/urine , Prevalence , Angola/epidemiology , Cross-Sectional Studies , Morbidity , Schistosoma haematobiumABSTRACT
Long-term monitoring of zooplankton assemblages provides essential knowledge to assess key factors impacting marine ecosystems. Despite the importance of this type of data, monitoring stations worldwide are spatially and temporally limited due to the difficulty of maintaining them. In the northeastern Atlantic area, Cascais-Watch is one monitoring site operating since 2005, despite some constraints throughout the years, and has allowed the collection of important data on the zooplankton communities of the area. The present work summarizes the knowledge collected until 2015 on the biodiversity and dynamics of zooplankton in the site. The results showed a year-round high productivity of the zooplankton abundance, biomass and diversity for the area, with no significant general trends or periodicity, despite the relatively lower winter and higher spring values. The results revealed two main transition periods with marked changes in species composition and dominance of the most abundant taxa. This shift was tentatively attributed to the extended annual dry season verified in Portugal after 2011, the low values of upwelling and precipitation, and the warmer waters. The zooplankton abundance presented an interannual increase for spring periods, and the proportion of Copepoda, the dominant taxa, was lower during summer months, corresponding to increased abundances of Mollusca, Diplostraca (Cladocera) and Cnidaria. In particular, the study shows an increasing abundance of the gelatinous species (particularly Cnidaria) for spring/summer months in recent years, suggesting changes in primary production and prey dynamics. Other relevant tendencies were the higher abundance of meroplankton, such as Bivalvia and fish larvae/eggs, and the decreasing trend in the abundance of the meroplanktonic coastal crustaceans, Decapoda and Cirripedia taxa, highlighting possible changes in the benthic coastal populations in the study region. The present study highlights probable changes and trends in the zooplankton community that should be monitored in the following years.
Subject(s)
Cnidaria , Ecosystem , Animals , Zooplankton , Portugal , Biodiversity , Atlantic OceanABSTRACT
Studies have reported that children with Congenital Zika Syndrome (CZS) experience changes in their sleep patterns, which can result in mood disturbances, behavioral issues and delays in growth and development. This systematic review synthesized the available evidence on the prevalence of sleep disorders in children with CZS. Eligible studies were those with an observational design that reported sleep disorders in children with CZS using validated questionnaires, polysomnography/electroencephalographic recording or parent/caregiver reports. Searches were conducted in PubMed, Web of Science, SCOPUS and Embase, as well as a gray literature search using Google Scholar. The Freeman-Tukey double-arcsine transformation with a random-effects model was used to estimate the pooled prevalence of sleep disorders with a 95% confidence interval (CI). Five studies were included and data from 340 Brazilian children with CZS were analyzed. The overall prevalence of sleep disorders was 27.4% (95% CI 16.7-39.4), without differences among studies using validated questionnaires (29.4%, 95% CI 21.4-37.8) or report from parents and caregivers (27.4%, 95% CI 11.5-47.0). Sleep disorders are prevalent in children with CZS, impacting their development and quality of life. It is critical to examine the quality of sleep in these children to develop appropriate interventions that can mitigate these issues.
The article discusses a systematic review of studies that have explored the prevalence of sleep disorders in children with Congenital Zika Syndrome (CZS), a condition caused by the Zika virus. The study found that children with CZS often experience changes in their sleep patterns, which can lead to mood disturbances, behavioral issues and delays in growth and development. The review included five studies with a total of 340 Brazilian children with CZS, and the overall prevalence of sleep disorders was found to be 27.4%. This indicates that sleep disorders are prevalent in children with CZS and can significantly impact their development and quality of life. The authors suggest that further research is needed to develop appropriate interventions to mitigate these issues.
Subject(s)
Pregnancy Complications, Infectious , Sleep Wake Disorders , Zika Virus Infection , Zika Virus , Child , Humans , Pregnancy , Female , Zika Virus Infection/complications , Zika Virus Infection/epidemiology , Zika Virus Infection/congenital , Prevalence , Quality of Life , Brazil/epidemiology , Sleep Wake Disorders/epidemiology , Sleep Wake Disorders/etiology , Pregnancy Complications, Infectious/epidemiologyABSTRACT
BACKGROUND: Urogenital schistosomiasis is one of the most prevalent parasitic diseases in sub-Saharan Africa. It is a poverty-related disease conditioned by behavioural practices. METHODS: Our objective is to evaluate the awareness, mindset and habits about urogenital schistosomiasis in the community of Cubal (Angola), as well as its association with infection and urinary tract morbidity in pre-school age children. A cross-sectional study of knowledge, attitudes and practices at home was conducted between February and May 2022 with 250 participants. RESULTS: Overall, 93.6% of those surveyed had some prior knowledge about schistosomiasis and, among all the symptoms associated with this disease, blood in the urine was the best known (54.4%). Nevertheless, 57.6% obtained a medium knowledge score. Regarding attitude, the majority of respondents had a high attitude score (79.2%) with 96.0% willing to participate in mass drug administration campaigns. Laundry in the river was the most common risk practice (61.2%) and 55.2% out of the total were classified with a low practice score. CONCLUSION: Low knowledge about symptoms and transmission by caregivers was the outstanding risk factor for infection in pre-school age children (OR = 16.93, 95%CI: 3.93-72.82), and lack of knowledge that avoiding entering the river prevents schistosomiasis was the main risk factor for morbidity in PSAC (OR = 8.14, 95%CI: 1.14-58.25).
Subject(s)
Schistosomiasis haematobia , Animals , Humans , Child , Child, Preschool , Schistosomiasis haematobia/diagnosis , Schistosoma haematobium , Angola/epidemiology , Cross-Sectional Studies , Health Knowledge, Attitudes, Practice , Morbidity , Risk Factors , Surveys and Questionnaires , PrevalenceABSTRACT
AIMS: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort. METHODS: Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022. RESULTS: Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron's sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125). CONCLUSIONS: Reuma.pt/myositis adequately captures the main features of inflammatory myopathies' patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.
ABSTRACT
OBJECTIVES: The main goal of this study was to characterise the frequency and phenotype of B, T follicular helper (Tfh) and T follicular regulatory (Tfr) cells in peripheral blood and the cytokine environment present in circulation in children with extended oligoarticular juvenile idiopathic arthritis (extended oligo JIA) and polyarticular JIA (poly JIA) when compared with healthy controls, children with persistent oligoarticular JIA (persistent oligo JIA) and adult JIA patients. METHODS: Blood samples were collected from 105 JIA patients (children and adults) and 50 age-matched healthy individuals. The frequency and phenotype of B, Tfh and Tfr cells were evaluated by flow cytometry. Serum levels of APRIL, BAFF, IL-1ß, IL-2, IL-4, IL-6, IL-10, IL-17A, IL-21, IL-22, IFN-γ, PD-1, PD-L1, sCD40L, CXCL13 and TNF were measured by multiplex bead-based immunoassay and/or ELISA in all groups included. RESULTS: The frequency of B, Tfh and Tfr cells was similar between JIA patients and controls. Children with extended oligo JIA and poly JIA, but not persistent oligo JIA, had significantly lower frequencies of plasmablasts, regulatory T cells and higher levels of Th17-like Tfh cells in circulation when compared with controls. Furthermore, APRIL, BAFF, IL-6 and IL-17A serum levels were significantly higher in paediatric extended oligo JIA and poly JIA patients when compared with controls. These immunological alterations were not found in adult JIA patients in comparison to controls. CONCLUSIONS: Our results suggest a potential role and/or activation profile of B and Th17-like Tfh cells in the pathogenesis of extended oligo JIA and poly JIA, but not persistent oligo JIA.
Subject(s)
Arthritis, Juvenile , Interleukin-17 , Humans , Child , Interleukin-6 , T-Lymphocyte Subsets , CytokinesABSTRACT
This two-arm, parallel-group, double-blind, randomized clinical trial design evaluated the risk of postoperative bleeding in anticoagulated patients undergoing dental extraction treated with topical TXA in comparison to collagen-gelatin sponge. Forty patients were randomly included in one of the study groups: (1) topical use of 4.8% TXA solution; and (2) resorbable hydrolyzed collagen-gelatin sponge applied to the surgical alveolus. Primary outcomes were postoperative bleeding episodes and secondary outcomes were thromboembolic events and postoperative INR values. The relative risk (RR), the absolute risk reduction (RAR) and the number needed to treat (NNT) were used as effect estimates and calculated from the counting of bleeding episodes observed during the first postoperative week. The bleeding rate under the TXA treatment was 22.2%, while in the collagen-gelatin sponge group it was 45.7%, resulting in a RR of 0.49 (95% CI 0.24-099; p = 0.046), RAR 23.5% and NNT 4.3. TXA was more effective in reducing bleeding in surgical sites located in the mandible (RR = 0.10; 95% CI 0.01-0.71; p = 0.021) and the posterior region (RR = 0.39; 95% CI 0.18-0.84; p = 0.016). Within the limitations of the study it seems that topical TXA is more effective in controlling bleeding after tooth extractions in anticoagulated patients than collagen-gelatin sponge. CLINICAL TRIAL REGISTRATION: RBR-83qw93.
Subject(s)
Antifibrinolytic Agents , Tranexamic Acid , Humans , Tranexamic Acid/therapeutic use , Gelatin , Antifibrinolytic Agents/therapeutic use , Postoperative Hemorrhage/etiology , Postoperative Hemorrhage/prevention & control , Collagen/therapeutic use , Administration, Topical , Tooth Extraction/adverse effects , Blood Loss, SurgicalSubject(s)
Lung Diseases, Interstitial , Myositis , Humans , Myositis/complications , Antibodies , Autoantibodies , Antibodies, AntinuclearABSTRACT
Health professionals are able to improve the care quality of chronic wounds by monitoring and reporting the wound status. Resorting to visual representations of wound status enhances comprehension by facilitating knowledge transfer to all stakeholders. However, selecting appropriate healthcare data visualisations is a critical challenge and healthcare platforms must be designed to meet their users' needs and constraints. This article describes the methods used to identify the design requirements and inform the development of a wound monitoring platform through a user-centred approach.
Subject(s)
Health Personnel , Quality of Health Care , HumansABSTRACT
Background: Idiopathic inflammatory myopathies (IIM) are a rare heterogeneous group of diseases characterised by chronic skeletal muscle inflammation, but other organs are also frequently involved. IMM represent a diagnostic challenge and a multidisciplinary approach is important to ensure successful diagnosis and adequate follow-up of these patients. Objective: To describe the general functioning of our multidisciplinary myositis clinic, highlighting the benefits of multidisciplinary team management in patients with confirmed or suspected IIM and to characterise our clinical experience. Methods: Description of the organization of a dedicated multidisciplinary myositis outpatient clinic, supported by IMM specific electronic assessment tools and protocols based on our Portuguese Register - Reuma.pt. In addition, an overview of our activity between 2017 and 2022 is provided. Results: An IIM multidisciplinary care clinic, based on a close collaboration between Rheumatologists, Dermatologists and Physiatrist is detailed in this paper. One hundred and eighty-five patients were assessed in our myositis clinic; 138 (75%) of those were female, with a median age of 58 [45-70] years. At the last appointment, 130 patients had a confirmed IIM diagnosis, and the mean disease duration was 4 [2-6] years. The most frequent diagnosis was dermatomyositis (n = 34, 26.2%), followed by antisynthetase syndrome (n = 27, 20.8%) and clinically amyopathic/paucimyopathic dermatomyositis (n = 18, 13.8%). Twenty-four patients (18.5%) were on monotherapy and 94 (72.3%) were on combination therapy. Conclusion: A multidisciplinary approach is important to ensure the correct diagnosis and follow-up of these patients. A myositis clinic, with a standardised practice at a tertiary hospital level, contributes to a standardization of care and opens research opportunities.
ABSTRACT
PURPOSE: Osteogenesis imperfecta (OI) is a rare inherited disease affecting collagen-rich tissues. Ocular complications have been reported such as thin corneas, low ocular rigidity, keratoconus, among others. The purpose of this study is to characterize corneal tomographic features in OI patients compared to unaffected patients, with particular focus on commonly studied keratoconus indices. METHODS: Cross-sectional case-control study including 37 OI patients and 37 age-matched controls. Patients and controls underwent comprehensive ophthalmological examination including corneal Scheimpflug tomography with a Pentacam HR device (Oculus Optikgeräte GmbH, Wetzlar, Germany) to analyse and compare topometric, tomographic, pachymetric and Belin-Ambrósio Enhanced Ectasia Display III (BAD-D) data of both eyes of each patient. RESULTS: Most OI patients had type I disease (n = 24; 65%) but type III-VII patients were also included. Two patients had clinically overt bilateral keratoconus. OI patients had significantly higher maximum keratometry (45.2 ± 2.1 vs. 43.7 ± 1.2; p = 0.0416), front and back elevation (3.0 ± 3.3 vs. 2.1 ± 1.3, p = 0.0201; 11.1 ± 8.2 vs. 5.0 ± 3.7, p < 0.0001), index of surface variance (25.5 ± 13 vs. 17.4 ± 8.3; p = 0.0016), index of vertical asymmetry (0.21 ± 0.14 vs. 0.15 ± 0.06; p = 0.0215), index of height asymmetry (9.2 ± 14 vs. 6.0 ± 4.5; p = 0.0421), index of height decentration (0.02 ± 0.01 vs. 0.01 ± 0.01; p < 0.0001) and average pachymetric progression (1.01 ± 0.19 vs. 0.88 ± 0.14; p < 0.0001) readings. Thinnest corneal thickness and maximum Ambrósio relational thickness were significantly lower (477 ± 52 vs. 543 ± 26; 387 ± 95 vs. 509 ± 49; p < 0.0001). Two-thirds of OI patients had corneas with a minimum thickness < 500 µm. BAD-D value was significantly higher in OI patients (2.1 ± 1.4 vs. 0.9 ± 0.2; p < 0.0001). CONCLUSION: OI patients showed significant changes in corneal profiles compared with healthy subjects. A high proportion of patients had tomographically suspect corneas when using keratoconus diagnostic indices. Further studies are warranted to assess the true risk of corneal ectasia in OI patients.
Subject(s)
Keratoconus , Osteogenesis Imperfecta , Humans , Keratoconus/diagnosis , Corneal Topography/methods , Case-Control Studies , Corneal Pachymetry , Osteogenesis Imperfecta/complications , Osteogenesis Imperfecta/diagnosis , Dilatation, Pathologic , Cross-Sectional Studies , ROC Curve , Cornea , Tomography , Retrospective StudiesABSTRACT
Complex or hard-to-heal wounds continue to be a challenge because of the negative impact they have on patients, caregivers, and all the associated costs. This study aimed to identify prognostic factors for the delayed healing of complex wounds. Five databases and grey literature were the sources used to research adults with pressure ulcers/injuries, venous leg ulcers, critical limb-threatening ischaemia, or diabetic foot ulcers and report the prognostic factors for delayed healing in all care settings. In the last 5 years, a total of 42 original peer-reviewed articles were deemed eligible for this scoping review that followed the JBI recommendations and checklist PRISMA-ScR. The most frequent prognostic factors found with statistical significance coinciding with various wound aetiologies were: gender (male), renal disease, diabetes, peripheral arterial disease, the decline in activities of daily life, wound duration, wound area, wound location, high-stage WIfI classification, gangrene, infection, previous ulcers, and low ankle brachial index. It will be essential to apply critical appraisal tools and assessment risk of bias to the included studies, making it possible to make recommendations for clinical practice and build prognostic models. Future studies are recommended because the potential for healing through identification of prognostic factors can be determined, thus allowing an appropriate therapeutic plan to be developed.
Subject(s)
Diabetic Foot , Pressure Ulcer , Varicose Ulcer , Adult , Humans , Male , Diabetic Foot/therapy , Pressure Ulcer/therapy , Prognosis , Varicose Ulcer/therapy , Wound HealingABSTRACT
Objectives: Idiopathic inflammatory myopathies (IIM) are a group of rare disorders that can affect the heart. This work aimed to find predictors of cardiac involvement in IIM. Methods: Multicenter, open cohort study, including patients registered in the IIM module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) until January 2022. Patients without cardiac involvement information were excluded. Myo(peri)carditis, dilated cardiomyopathy, conduction abnormalities, and/or premature coronary artery disease were considered. Results: 230 patients were included, 163 (70.9%) of whom were females. Thirteen patients (5.7%) had cardiac involvement. Compared with IIM patients without cardiac involvement, these patients had a lower bilateral manual muscle testing score (MMT) at the peak of muscle weakness [108.0 ± 55.0 vs 147.5 ± 22.0, p=0.008] and more frequently had oesophageal [6/12 (50.0%) vs 33/207 (15.9%), p=0.009] and lung [10/13 (76.9%) vs 68/216 (31.5%), p=0.001] involvements. Anti-SRP antibodies were more commonly identified in patients with cardiac involvement [3/11 (27.3%) vs 9/174 (5.2%), p=0.026]. In the multivariate analysis, positivity for anti-SRP antibodies (OR 104.3, 95% CI: 2.5-4277.8, p=0.014) was a predictor of cardiac involvement, regardless of sex, ethnicity, age at diagnosis, and lung involvement. Sensitivity analysis confirmed these results. Conclusion: Anti-SRP antibodies were predictors of cardiac involvement in our cohort of IIM patients, irrespective of demographical characteristics and lung involvement. We suggest considering frequent screening for heart involvement in anti-SRP-positive IIM patients.
Subject(s)
Myocarditis , Myositis , Rheumatic Diseases , Female , Humans , Male , Cohort Studies , HeartABSTRACT
(1) Background: The high prevalence of persons with wounds and its consequences for a person's quality of life makes the issue a relevant focus of attention for healthcare professionals. Through prognostic factors for healing, the individual risk of complications can be predicted, is possible to predict imminent delays and guide decision-making, thus helping healthcare professionals. (2) Methods: A scoping review performed according to JBI methodology and guided by the Preferred Reporting Items for Systematic Reviews extension for Scoping Reviews (PRISMA-ScR) checklist will aim to identify the studies that meet predefined eligibility criteria. Five databases and gray literature will be the sources used to research adults with pressure ulcers, venous leg ulcers, arterial ulcers, or diabetic foot ulcers and report the prognostic factors for delayed healing in any care setting. (3) Results: This review will consider all quantitative and mixed studies in the last five years. The selection of articles will be carried out by two reviewers independently, using EndNoteWeb and Rayyan. Prognostic factors will be presented by design study, sampling, setting, outcome, wound type, and statistical methods. (4) Conclusions: Mapping prognostic factors for delayed healing could also be a starting point for a systematic review and meta-analyses to quantify the value of each factor.