Cerebral Venous Thrombosis in Pediatric Age: Risk Factors and Prognosis.

INTRODUCTION: Cerebral venous thrombosis (CVT) is a rare but potentially fatal disease in pediatric age with an important morbimortality. In adults several factors have been associated with worse outcomes, however there are still few studies in children. This study aims to identify risk factors associated with clinical manifestations and long-term sequelae in pediatric CVT. METHODS: Retrospective analysis of pediatric inpatients admitted to a tertiary-care hospital due to CVT between 2008 and 2020. RESULTS: Fifty-four children were included, 56% male, median age of 6.5 years (9 months-17.3 years). Permanent risk factors were identified in 13 patients (malignancy, 8; hematologic condition, 5) and transient risk factors in 47, including head and neck infections (57%) and head trauma (15%). Multiple venous sinuses involvement was present in 65% and the deep venous system was affected in four patients. Seventeen percent had intracranial hemorrhage and 9% cerebral infarction. Sixty-four percent of patients with multiple venous sinuses involvement presented with severe clinical manifestations: impaired consciousness, intracranial hypertension, acute symptomatic seizures or focal deficits. Regarding long-term prognosis, six patients had major sequelae: epilepsy (n = 3), sensory motor deficits (n = 2), and cognitive impairment (n = 3). Permanent risk factors were associated with severe clinical manifestations (p = 0.043). Cerebral infarction and intracranial hemorrhage were associated with major sequelae (p = 0.006 and p = 0.03, respectively, adjusted for age and sex). CONCLUSION: Permanent risk factors, involvement of multiple venous sinuses, intracranial hemorrhage, and cerebral infarction, were related to worse prognosis. Detection and early management of risk factors may limit CVT extension and reduce its morbimortality.

Safety of direct oral provocation test to delabel reported mild beta-lactam allergy in infants.

BACKGROUND: Around 10% of people report a drug allergy and avoid some medications because of fear of allergic reactions. However, only after a proper diagnostic workup can some of these reactions be confirmed as allergic or nonallergic hypersensitivities. Beta-lactams (BLs) are the most common medication suspected of being involved in drug hypersensivity reactions (DHRs) in children. Recently, direct oral provocation tests (DPT) with BLs gained popularity within pediatric populations as a tool for delabeling children with suspected BL allergies. This study aimed to evaluate the safety of direct provocation tests in infants with mild cutaneous non-immediate reactions to BLs. METHODS: The authors retrospectively analyzed the data of 151 infants between 2015 and 2022, referred for evaluating a suspected allergy to BLs that occurred before age 24 months. RESULTS: The mean age of the children, including 55% male kids, at the suspected reaction was 15.9 months and the mean age at the time of the DPT was 39.6 months. In most cases, antibiotics were prescribed to treat common upper respiratory infections, such as acute otitis (54.3%) and acute tonsillitis (27.2%). Amoxicillin was considered the culprit drug in 62.9% of the cases, and the combination of amoxicillin-clavulanic acid in the case of 33.8% of children. The most frequent associated cutaneous clinical manifestations were maculopapular exanthema in 74.8% and delayed urticaria/angioedema in 25.2%. Of the 151 infants evaluated, parents of 149 infants agreed for a direct DPT, and only three had a positive test (2%). Symptoms resulting from the DPT were mild and easily treatable. CONCLUSIONS: A direct DPT without prior tests is a safe and effective procedure to delabel BL allergy, even in infants. The authors wish to emphasize the importance of properly validating BL allergy suspicions by promoting appropriate diagnostic procedures in infants as, in most cases, DHRs can be excluded and there is no need for further therapeutic restrictions.

A standardised protocol for blood and cerebrospinal fluid collection and processing for biomarker research in ataxia.

The European Spinocerebellar Ataxia Type 3/Machado-Joseph Disease Initiative (ESMI) is a consortium established with the ambition to set up the largest European longitudinal trial-ready cohort of Spinocerebellar Ataxia Type 3/Machado-Joseph Disease (SCA3/MJD), the most common autosomal dominantly inherited ataxia worldwide. A major focus of ESMI has been the identification of SCA3/MJD biomarkers to enable future interventional studies. As biosample collection and processing variables significantly impact the outcomes of biomarkers studies, biosampling procedures standardisation was done previously to study visit initiation. Here, we describe the ESMI consensus biosampling protocol, developed within the scope of ESMI, that ultimately might be translated to other neurodegenerative disorders, particularly ataxias, being the first step to protocol harmonisation in the field.

Task Force for a rapid response to an outbreak of severe acute hepatitis of unknown aetiology in children in Portugal in 2022.

On 5 April 2022, the United Kingdom reported an increase of cases of severe acute hepatitis of unknown aetiology in children, several needing hospitalisation and some required liver transplant or died. Thereafter, 35 countries reported probable cases, almost half of them in Europe. Facing the alert, on 28 April, Portugal created a multidisciplinary Task Force (TF) for rapid detection of probable cases and response. The experts of the TF came from various disciplines: clinicians, laboratory experts, epidemiologists, public health experts and national and international communication. Moreover, Portugal adopted the European Centre for Disease Prevention and Control (ECDC) and the World Health Organization (WHO) case definition and recommendations. By 31 December 2022, 28 probable cases of severe acute hepatitis of unknown aetiology were reported: 16 male and 17 aged under 2 years. Of these cases, 23 were hospitalised but none required liver transplant or died. Adenovirus was detected from nine of 26 tested cases. No association was observed between adenovirus infection and hospital admission after adjusting for age, sex and region in a binomial regression model. The TF in Portugal may have contributed to increase awareness among clinicians, enabling early detection and prompt management of the outbreak.

Static and Dynamic Ocular Motor Abnormalities as Potential Biomarkers in Spinocerebellar Ataxia Type 3.

While dynamic ocular motor abnormalities (e.g., gaze-evoked nystagmus (GEN), low optokinetic nystagmus (OKN), pursuit and vestibulo-ocular reflex (VOR) gains, and dysmetric saccades) have been shown to be potential biomarkers in spinocerebellar ataxia type 3 (SCA3), the value of static abnormalities (e.g., convergent [esodeviation] and divergent strabismus [exodeviation]) is unknown. Moreover, studies on dynamic abnormalities in SCA3 usually do not take into account the existence of potential abduction-adduction asymmetries in patients with degenerative ataxia. Thirty-eight patients with genetically confirmed SCA3 (24 females; mean age ± SD, 49.8± 12.2 years) and 22 healthy controls (12 females, p = 0.589; mean age ± SD, 50.7± 12.5 years, p = 0.651) underwent clinical and video-oculographic assessment. A p value < 0.002 (between- and within-group analyses) and < 0.001 (correlation analysis) was considered significant. Patients showed larger esodeviation at distance (p < 0.001), became more esodeviated in lateral gaze (p < 0.001), and their near exodeviation correlated with scale for the assessment and rating of ataxia (SARA) score (p = 0.004). Pursuit, OKN, and VOR gains were lower in patients, both for their adducting and abducting components (p < 0.001). Saccades showed higher velocities (p < 0.001), abducting saccades showed lower amplitude (p < 0.001), and adducting saccades tended to show greater vertical bias (p = 0.018) in patients. Abducting saccades showed relatively lower velocity (p < 0.001) and lower amplitude (p = 0.015) than abducting saccades within patients. All dynamic ocular motor abnormalities except saccades correlated with SARA score, CAG repeat number, and/or disease duration (p < 0.001). Static and dynamic ocular motor abnormalities are potential biomarkers in SCA3. SCA3 studies using saccades should take into account the existence of potential abduction-adduction asymmetries.

Sporadic Creutzfeldt-Jakob disease presenting as epilepsia partialis continua and non-ictal nystagmus.

BACKGROUND AND PURPOSE: Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive neurodegenerative disorder. Seizures are uncommon in the early stage of CJD, increasing diagnostic difficulty. METHODS: An autopsy-proven case of CJD presenting initially as an epilepsia partialis continua is reported, in which the initial workup was unremarkable. Retrospectively, the presence of nystagmus, which proved to be non-epileptic, pointed to a cerebellar lesion before a diagnosis of clinically probable CJD was made. RESULTS: A 70-year-old man presented with a 3-week history of intermittent rhythmic jerking tremors in his left limbs, interfering with his gait. Examination showed left body clonic movements. Electroencephalography revealed an ictal right centroparietal pattern of focal status epilepticus. Video-oculography revealed right-beating nystagmus (mean slow phase velocity [SPV] 3.4º/s) in the dark and left-beating nystagmus (SPV 2.6º/s) in the light, left-beating nystagmus after head shaking (SPV 4º/s) and during mastoid vibration (SPV 11º/s) and mildly hypoactive horizontal head impulses. Search for occult malignancy, serologies, cerebrospinal fluid analyses, anti-onconeural antigen, auto-immune panel and brain magnetic resonance imaging were unrevealing. Rapid neurological decline was observed. Three weeks later, cerebrospinal fluid was positive for 14.3.3 protein, electroencephalography showed generalized periodic sharp wave complexes and brain magnetic resonance imaging revealed diffusion restriction and T2/fluid-attenuated inversion recovery hyperintensities in the cerebellum, basal ganglia, thalamus and cortex. He died 1 month later. Neuropathological study confirmed the diagnosis of CJD. CONCLUSION: This case highlights that CJD should be considered in the differential diagnosis of new onset epilepsia partialis continua and that neuro-ophthalmological examination can be helpful in pointing to early asymmetric cerebellar involvement.

DAS28, CDAI and SDAI cut-offs do not translate the same information: results from the Rheumatic Diseases Portuguese Register Reuma.pt.

OBJECTIVES: . The 28-joint DAS (DAS28), clinical disease activity index (CDAI) and simplified disease activity index (SDAI) are indices frequently used to assess disease activity in RA patients. Cut-off values were defined to classify the states of RA disease activity: remission, low, moderate and high. The aim of this work was to assess disease activity states classified by DAS28, CDAI and SDAI and to analyse their agreement in the Rheumatic Diseases Portuguese Register Reuma.pt. METHODS: . A total of 2795 patients and 14 440 visits were selected from Reuma.pt for analysis. Pearson's correlation coefficients (PCCs) were calculated for the three indices. McNemar's chi-squared tests, PCCs and kappa statistics were performed to analyse and compare the distribution of visits among all disease activity states and indices. RESULTS: A strong correlation was found between the three indices throughout the 14 440 visits: r = 0.874 for DAS28/CDAI, r = 0.877 for DAS28/SDAI and r = 0.984 for CDAI/SDAI (all PCCs with P < 0.0001). However, when categorization in the different disease activity states was analysed, McNemar's chi-squared tests and PCCs revealed significant disagreement between the cut-offs of the three indices. CONCLUSION: DAS28, CDAI and SDAI cut-offs do not translate into the same clinical information in Reuma.pt. Although this might be expected for the original DAS28 cut-offs, when compared with CDAI and SDAI significant disagreement was also found for the DAS28 modified cut-offs. For visits where patients are in CDAI or SDAI remission, we also find disagreement between these two indices, which may contradict previous conclusions that acute phase reactants add little to composite disease activity indices for RA.

An Unusual Complication of Lisinopril: A Report of Iatrogenic Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, is a hypersensitivity vasculitis characterized by palpable purpuric lesions associated with polyarthralgia, abdominal discomfort, and renal involvement. We present the case of a 41-year-old man who was admitted to the emergency department due to generalized purpuric lesions and abdominal pain. During the complementary study, there was no evidence of thrombocytopenia or coagulopathy but confirmed microscopic haematuria. The diagnosis of HSP was supported by the presence of leukocytoclastic vasculitis with perivascular IgA deposits in the skin biopsy. After excluding infectious, autoimmune, and neoplastic pathologies, the possibility of HSP associated with taking lisinopril, which had been recently initiated after hospitalization for acute heart failure, was assumed. Angiotensin-converting enzyme (ACE) inhibitor suspension and treatment with systemic corticosteroids lead to significant clinical regression, supporting our suspicion.

Schizophreniform Disorder Related Hospitalizations: A Clinical and Demographic Analysis of a National Hospitalization Database.

INTRODUCTION: Schizophreniform disorder manifests itself with similar symptoms to schizophrenia, but it is distinguished from the latter by its shorter duration, varying between at least one and six months. This study aimed to describe and analyze schizophreniform disorder related hospitalizations in a national hospitalization database. METHODS: We planned a descriptive retrospective study using a nationwide hospitalization database containing all hospitalizations registered in Portuguese mainland public hospitals from 2008 to 2015. Hospitalizations with a primary diagnosis of schizophreniform disorder were selected based on the International Classification of Diseases version 9, Clinical Modification (ICD-9-CM) code of diagnosis 295.4x. Data regarding birth date, sex, residence address, diagnoses, length of stay, discharge status, and hospital charges were obtained. Comorbidities were analyzed using the Charlson Index Score. Independent Sample t tests were performed to assess differences in continuous variables with a normal distribution and Mann-Whitney-U tests when no normal distribution was registered. RESULTS: In Portuguese mainland public hospitals, a total of 594 hospitalizations with a primary diagnosis of schizophreniform disorder occurred during the eight-year study period. Most, 72.1% (n = 428), were observed in male patients. The mean age at admission was 34.34 years in male patients and 40.19 years in female patients. The median length of stay was 17.00 days and in-hospital mortality was 0.5% (n = 3). Only 6.1% (n = 36) of the hospitalization episodes had one or more registered comorbidities. Forty-one readmissions were documented. CONCLUSION: Hospitalizations with a primary diagnosis of schizophreniform disorder occur more frequently in young male patients. This is, to the best of our knowledge, the first nationwide study analyzing all hospitalizations due to this diagnosis in Portugal.

Colouterine Fistula as a Rare Cause of Postmenopausal Abnormal Uterine Bleeding: A Case Report.

Postmenopausal abnormal uterine bleeding is a common clinical problem addressed in gynaecological practice and should prompt clinical investigation due to the significant prevalence of malignant and premalignant lesions of the endometrium in this age group. Nevertheless, other causes should be considered, since its diagnostic and therapeutic management may differ considerably. Here, we present a case of a colouterine fistula due to chronic diverticulitis presenting with postmenopausal abnormal uterine bleeding. This is an infrequent occurrence and is caused by the rupture of a diverticular abscess into the uterine wall, resulting in an inflammatory adhesion of the colon and uterus, with necrosis and subsequent fistula formation. The clinical presentation is variable and may include abdominal pain, gastrointestinal tract symptoms, vaginal discharge, and abnormal uterine bleeding. The laboratory and imaging techniques may be not completely conclusive and definitive diagnosis can be made intraoperatively. There are different treatment options, with en bloc resection and primary anastomosis being used most often, allowing complete treatment. The prognosis for a colouterine fistula secondary to diverticulitis is excellent after surgery. This case highlights the importance of clinical suspicion of an unusual cause of uterine bleeding and an effective and multidisciplinary approach that allowed complete surgical treatment and patient recovery.

Limits of Viability: Perspectives of Portuguese Neonatologists and Obstetricians.

INTRODUCTION: Advances in neonatal care have improved the prognosis in extremely preterm infants. The gestational age considered for active treatment has decreased globally. Despite implemented guidelines, several studies show variability in practice. The aim of this study was to understand theperspectives of Portuguese neonatologists and obstetricians regarding the management of extremely preterm infants. METHODS: An online survey was sent through the Portuguese Neonatology Society and the Portuguese Society of Obstetrics and Maternal-Fetal Medicine from August to September 2023. RESULTS: We obtained 117 responses: 53% neonatologists, 18% pediatricians, and 29% obstetricians, with 62% having more than 10 years of experience. The majority (80%) were familiar with the Portuguese Neonatology Society consensus on the limits of viability and 46% used it in practice; 62% were unaware of Portuguese morbidity-mortality statistics associated with extremely preterm infants. Most (91%) informed parents about morbiditymortality concerning the gestational age more frequently upon admission (64%) and considered their opinion in the limit of viability situations (95%). At 22 weeks gestational age, 71% proposed only comfort care, while at 25 and 26 weeks, the majority suggested active care (80% and 96%, respectively). Less consensus was observed at 23 and 24 weeks. At 24 weeks, most obstetricians offered active care with the option of comfort care by parental choice (59%), while the neonatology group provided active care (65%), p < 0.001. Regarding the lower limit of gestational age for in utero transfer, corticosteroid administration, cesarean section for fetal indication, neonatologist presence during delivery, and endotracheal intubation; neonatologists considered a lower gestational age than obstetricians (23 vs 24 weeks; p = 0.036; p < 0.001; p < 0.001; p = 0.021; p < 0.001, respectively). CONCLUSION: Differences in perspectives between obstetricians and neonatologists in limits of viability situations were identified. Neonatologists considered a lower gestational age in various scenarios and proposed active care earlier. Standardized counseling for extremely preterm infants is crucial to avoid ambiguity, parental confusion, and conflicts in perinatal care.

eHealth in pediatric respiratory allergy.

PURPOSE OF REVIEW: This review explores the relevance of eHealth technologies to address unmet needs in pediatric respiratory allergies, particularly allergic rhinitis (AR) and asthma. Given the increasing burden of these conditions, there is a pressing need for effective solutions to enhance disease surveillance, diagnosis, and management. RECENT FINDINGS: Recent literature highlights the potential of eHealth tools to transform pediatric respiratory allergy care. The use of digital data for infodemiology, application of machine learning models to improve diagnostic sensitivity, smartphone apps with digital patient reported outcome measure (PROMs) and embedded sensors to monitor disease, healthcare professional dashboards with real-time data monitoring and clinical decision support systems (CDSS) are advances emerging to optimize pediatric respiratory allergy care. SUMMARY: Integrating eHealth technologies into the pediatric respiratory allergy care pathway is a potential solution for current healthcare challenges to better meet the needs of children with AR and asthma. However, while the potential of eHealth is evident, its widespread implementation in real-world practice requires continued research, collaboration, and efforts to overcome existing barriers.

Quality of Life and Hormonal Impairment in Pediatric Patients With Craniopharyngiomas.

INTRODUCTION: Craniopharyngiomas (CP) are tumors in the sellar region that, despite a high survival rate, are associated with significant morbidity, including hypothalamic, hormonal, and visual dysfunction. This study aimed to assess the quality of life (QoL) in pediatric patients with CP and to evaluate its relationship with various factors, with a focus on the impact of endocrine dysfunction. METHODS: In this observational cross-sectional study, patients with CP aged between 0 and 18 years, currently followed up in a tertiary hospital by a multidisciplinary team, were included. QoL was assessed using the validated PEDS-QL4.0 questionnaire, which was administered to parents. This tool estimates Global QoL (QoL-G), further divided into Physical (QoL-P) and Psychosocial (QoL-PS) dimensions, including Emotional (QoL-Em), Social (QoL-S), and School (QoL-Sc) aspects. In Portugal, the estimated average QoL-G is 79.8, QoL-P is 83.5, and QoL-PS is 78.2. Variables studied included gender, current and diagnostic age, follow-up time, presence of hydrocephalus, hypothalamic involvement, type of resection (total or subtotal), radiotherapy, visual impairment, hormonal deficits, and therapy. RESULTS: The study included 11 patients with a median age of 15.2 years (interquartile ratio (IQR), 9.7-17.9 years) and a mean age at diagnosis of 9.3±4.1 years. Of these patients, 54.5% were male, and 36.4% were obese. Subtotal resection was performed in 72.7% of cases. Hydrocephalus was present in 54.5% of the patients, hypothalamic involvement in 63.7%, radiotherapy was received by 81.8%, and visual impairment was noted in 54.5%. All patients presented with at least one hormonal deficit. The average QoL-G was 69.9±22.5, with QoL-P at 66.9±30.0 and QoL-PS at 70.9±21.4. A worse QoL-S was associated with female gender (p=0.030) and subtotal resection (p=0.048). Worse QoL-G, QoL-P, QoL-Em, and QoL-PS were linked to hypothalamic involvement (p values 0.008, 0.025, 0.015, and 0.009, respectively). Irradiated patients had worse QoL-G (p=0.006). Treatment with sexual hormones enhanced QoL-Global (p=0.035) and QoL-Emotional (p=0.020), while treatment for adrenal insufficiency and diabetes insipidus improved QoL-Emotional (p=0.021 and p=0.013). No significant associations with visual deficit or obesity were found. CONCLUSIONS: Pediatric patients with CP appear to have poorer QoL-G, QoL-P, and QoL-PS compared to the healthy Portuguese population. However, the small sample size limits statistically significant associations with many of these variables. Predictors of worse QoL include female gender, hypothalamic involvement, subtotal resection, and radiotherapy. The results may be biased due to the small sample size, questionnaire administration to parents, and possible inadequacy of the questionnaire for the studied population. There is a need for a more suitable tool to enable a more precise assessment of QoL in these patients.

Carotid Intima-Media Thickness and Cardiometabolic Profile in Turner Syndrome: A Cross-Sectional Study.

INTRODUCTION: Turner syndrome (TS), one of the most common chromosomal abnormalities in females, often results in adult cardiovascular and metabolic complications. Information on pediatric age is scarce. This study aimed to compare the presence of cardiometabolic risk factors in children with TS and healthy controls. METHODS: This is a cross-sectional study comparing patients with TS to age-matched healthy controls, regarding cardiometabolic risk factors including lipid profile, fasting glucose, insulin resistance, body composition, body mass index, blood pressure, and carotid intima-media thickness (cIMT). RESULTS: We included nine TS patients and nine controls with a median age of 13 years (9-14 years). Three TS patients and three controls were prepubertal. All TS patients received growth hormone treatment (GHT), median treatment of six years (3-10 years); four patients underwent treatment with estradiol. No statistically significant differences were detected between TS patients and controls regarding body mass index (BMI), cholesterol levels, and insulin resistance. cIMT indexed to body surface area showed no significant differences between TS patients and controls (0.37 vs 0.35 mm/m2, respectively, p=0.605). TS patients had lower body fat levels (7.2% vs 34.9%, p=0.004). On the other hand, TS patients had higher levels of systolic (z-score 1.04 vs -0.08, p=0.001) and diastolic (z-score 1.08 vs 0.33, p=0.031) blood pressure (BP) and aspartate (AST) and alanine (ALT) aminotransferase levels (26 vs 20 U/L, p=0.008 and 19 vs 14 U/L, p=0.004, respectively). CONCLUSION: Patients with TS, all submitted to GHT, had lower body fat levels compared with controls, despite similar BMI. Although we found no differences in cIMT between the two groups, young girls with TS had higher BP and transaminase levels. Early anthropometric, cardiovascular, and analytical monitoring of patients with TS is essential to detect abnormalities and prevent further complications.

Telemonitoring of pediatric asthma in outpatient settings: A systematic review.

Telemonitoring technologies are rapidly evolving, offering a promising solution for remote monitoring and timely management of asthma acute episodes. We aimed to describe current pediatric asthma telemonitoring technologies. A systematic review was conducted until September 2023 on Medline, Scopus, and Web of Science. We included studies of children (0-18 years) with asthma or recurrent wheezing whose respiratory condition was telemonitored outside the healthcare setting. A narrative synthesis was performed. We identified 40 telemonitoring technologies described in 40 studies. The more frequently used technologies for telemonitoring were mobile applications (n = 21) and web-based systems (n = 14). Telemonitoring duration varied between 2 weeks and 32 months. Data collection included asthma symptoms (n = 30), patient-reported outcome measures (PROMs) (n = 11), spirometry/peak flow readings (n = 20), medication adherence (n = 17), inhaler technique (n = 3), air quality (n = 2), and respiratory sounds (n = 2). Both parents and children were the technology target users in most studies (n = 23). Technology training was reported in 23 studies of which 3 provided ongoing support. Automatic feedback was found in 30 studies, mostly related with asthma control. HCP were involved in data management in 27 studies. Technologies were tested in samples from 4 to 327 children, with most studies including school-aged children and/or adolescents (n = 38) and eight including preschool children. This review provides an overview of existing technologies for the outpatient telemonitoring of pediatric asthma. Specific technologies for preschool children represent a gap in the literature that needs to be specifically addressed in future research.

Newest Vital Sign as a proxy for medication adherence in older adults.

OBJECTIVE: To assess the utility of the Newest Vital Sign (NVS) as a proxy for medication adherence in community-dwelling older adults. DESIGN: Descriptive cross-sectional study. SETTING: 12 adult day care centers in the Lisbon metropolitan area, Portugal, between March and May 2009. PARTICIPANTS: 100 white community-dwelling older adults. INTERVENTION: Participants were administered the NVS, Single Item Literacy Screener (SILS), and self-reported Measure of Adherence to Therapy (MAT). MAIN OUTCOME MEASURES: Health literacy and medication adherence. RESULTS: The mean (±SD) age of the respondents was 73.3 ± 7.8 years and 71% were women. The NVS score was 0.81 ± 0.10 (of 6 possible points), and 95% of the respondents scored in the three lowest possible scores, indicating a notable floor effect. Age was found to be inversely correlated with NVS score (P = 0.003). The MAT score was 36.2 ± 4.7 (range 17-42). No statistically significant association between the NVS and level of education (P = 0.059 [Kruskal-Wallis]), gender (P = 0.700 [Mann-Whitney]), SILS (P = 0.167), or MAT (P = 0.379) was identified. CONCLUSION: The utility of the NVS as a proxy for medication adherence in community-dwelling older adults is limited because of a floor effect that hinders its predictive power for medication adherence.

Fig Tree-Induced Phytophotodermatitis: A Case Report on the Perils of a Hobby.

Phytophotodermatitis, a condition that results from sequential skin exposure to phototoxic chemicals contained within plants, followed by exposure to solar ultraviolet radiation, has been described with several plants and plant-based foods, namely members of the Moraceaefamily, which include Ficus carica L. This tree's branches, leaves, and fruit skin exude a milky sap or latex containing proteolytic enzymes and furocoumarins known to be photoirritants, easily absorbed upon skin contact. Oxygen-dependent and independent toxic reactions subsequent to sun exposure promote cell membrane damage and oedema, consequently leading to cell death. The diagnosis is confirmed with a detailed anamnesis, and photopatch testing is often useful to rule out a differential diagnosis. It is typically a self-limited condition, with few cases requiring treatment with topical or systemic corticosteroids. We report on a 55-year-old male patient who, following picking figs and pruning a fig tree while exposed to sunlight, developed erythematous and pruritic maculopapular lesions that progressed to blisters with residual hyperpigmentation. The diagnosis was further corroborated through photopatch testing, and the patient was recommended to avoid this recreational activity without symptoms' relapse. This case highlights the importance of considering phytophotodermatitis as a differential diagnosis when evaluating cases of dermatitis on exposed body surfaces and the importance of an exhaustive anamnesis. Identification of specific plant triggers and the performance of photopatch tests are essential to help confirm the diagnosis and guide avoidance recommendations.

Impact of the COVID-19 Pandemic on the Prescribing of Antiasthmatic Treatments in Portugal: A Nationwide Study.

PURPOSE: The coronavirus disease 2019 (COVID-19) pandemic has had a profound impact on everyday life, the environment, and health care services. A shift from in-person medical appointments to telemedicine was a main adjustment. Such changes can have repercussions on the control and management of chronic respiratory diseases, such as asthma. The available data suggest that there was an overall decrease in asthma-related morbidities during the first year of the pandemic. Therefore, the goal of this study was to quantify the effects of the pandemic on the prescribing of antiasthmatic treatments in outpatient care (public and private health care). METHODS: This before-after study used a time series approach based on data from monthly prescriptions of antiasthmatic drugs (anti-inflammatory drugs and bronchodilators) dated between April 2018 and March 2021. An interrupted time series (ITS) design was used for assessing changes in antiasthmatic prescribing patterns in the short and long terms after COVID-19 was recognized as a pandemic. The results are complemented with seasonal autoregressive integrated moving average (sARIMA) models. FINDINGS: The ITS analysis showed a non-significant increase in antiasthmatic prescribing in the short term. In the long term, after the pandemic was declared, a statistically significant decrease was observed in the prescribing of antiasthmatics (in anti-inflammatory drugs and, more pronounced, in bronchodilators). In the sARIMA model, the mean monthly volume of antiasthmatic prescriptions was 18.1% lower than predicted. The numbers of months outside of the 95% CIs were different between anti-inflammatory drugs (1 month) and bronchodilators (7 months). IMPLICATIONS: The prescribing of antiasthmatic drugs in the long term was significantly decreased with the COVID-19 pandemic, with a greater effect in the case of bronchodilators.

Effectiveness of biosimilar infliximab CT-P13 compared to originator infliximab in biological-naïve patients with rheumatoid arthritis and axial spondyloarthritis: data from the Portuguese Register.

OBJECTIVES: To compare the effectiveness of the infliximab biosimilar CT-P13 with originator infliximab over 24 months of follow-up in biological-naïve patients with rheumatoid arthritis (RA) and axial spondyloarthritis (axSpA). METHODS: Biological-naïve patients from the Rheumatic Diseases Portuguese Register (Reuma.pt), with a clinical diagnosis of RA or axSpA, who were starting either the infliximab biosimilar CT-P13 or the originator infliximab after 2014 (date of market entry of CT-P13 in Portugal), were included. Patients on biosimilar and originator were compared regarding different response outcomes at 3 and 6 months, adjusting for age, sex and baseline C-reactive protein (CRP). The main outcome was the change in DAS28-erytrocyte sedimentation rate (ESR) for RA and the ASDAS-CRP for axSpA. Additionally, the effect of infliximab biosimilar vs originator on different response outcomes over 24 months of follow-up was tested with longitudinal generalized estimating equations (GEE) models. RESULTS: In total, 140 patients were included, 66 (47%) of which with RA. The distribution of patients starting the infliximab biosimilar and the originator was the same between the two diseases (approximately 60% and 40%, respectively). From the 66 patients with RA, 82% were females, mean age was 56 years (SD 11) and mean DAS28-ESR 4.9 (1.3) at baseline. As for the patients with axSpA, 53% were males, mean age was 46 years (13) and mean ASDAS-CRP 3.7 (0.9) at baseline. There were no differences in efficacy between RA patients treated with the infliximab biosimilar and the originator, either at 3 months (∆DAS28-ESR: -0.6 (95% CI -1.3; 0.1) vs -1.2 (-2.0; -0.4)), or at 6 months (∆DAS28-ESR: -0.7 (-1.5; 0.0) vs -1.5 (-2.4; -0.7)). This was also true for patients with axSpA (∆ASDAS-CRP at 3 months: -1.6 (-2.0; -1.1) vs -1.4 (-1.8; -0.9) and at 6 months: -1.5 (-2.0; -1.1) vs -1.1 (-1.5; -0.7)). Results were similar with the longitudinal models over 24 months. CONCLUSION: There are no differences in effectiveness between the infliximab biosimilar CT-P13 and the infliximab originator in the treatment of biological-naïve patients with active RA and axSpA in clinical practice.

Influence of the timing of biological treatment initiation on Juvenile Idiopathic Arthritis long-term outcomes.

BACKGROUND: Juvenile idiopathic arthritis (JIA) treatment is aimed at inducing remission to prevent joint destruction and disability. However, it is unclear what is the long-term impact on health-related outcomes of the timing of biological disease-modifying antirheumatic drug (bDMARD) initiation in JIA. Our aim was to evaluate the long-term impact of the time between JIA onset and the initiation of a bDMARD in achieving clinical remission, on physical disability and health-related quality of life (HRQoL). METHODS: Adult JIA patients registered in the Rheumatic Diseases Portuguese Register (Reuma.pt) and ever treated with bDMARD were included. Data regarding socio-demographic, JIA-related characteristics, disease activity, physical disability (HAQ-DI), HRQoL (SF-36), and treatments were collected at the last visit. Patients were divided into 3 groups (≤ 2 years, 2-5 years, or > 5 years), according to the time from disease onset to bDMARD initiation. Regression models were obtained considering remission on/off medication, HAQ-DI, SF-36, and joint surgeries as outcomes and time from disease onset to bDMARD start as an independent variable. RESULTS: Three hundred sixty-one adult JIA patients were evaluated, with a median disease duration of 20.3 years (IQR 12.1; 30.2). 40.4% had active disease, 35.1% were in remission on medication, and 24.4% were in drug-free remission; 71% reported some degree of physical disability. Starting a bDMARD > 5 years after disease onset decreased the chance of achieving remission off medication (OR 0.24; 95% CI 0.06, 0.92; p = 0.038). Patients who started a bDMARD after 5 years of disease onset had a higher HAQ and worse scores in the physical component, vitality, and social function domains of SF-36, and more joint surgeries when compared to an earlier start. CONCLUSION: Later initiation of bDMARDs in JIA is associated with a greater physical disability, worse HRQoL, and lower chance of drug-free remission in adulthood.