Abstract
INTRODUCTION-BACKGROUND: Cognitive, learning and/or behavioral disorders are common (up to 70%) complications of neurofibromatosis (NF). The first multidisciplinary-clinic for ...neurocutaneous-disorders was established at the Aghia Sophia Children’s Hospital in Athens, Greece, in 2016. Since then, more than 200 children and adolescents with NF have been examined. SCOPE: Acknowledging and indicating awareness on the devastating life-long consequences (poor academic performance, behavioral problems, and limited career prospectives) that can result from cognitive impairment, a research collaboration with educational specialists was recently implemented to examine the neurocognitive functions of children and adolescents with NF. MATERIALS: Children and adolescents aged 7-14 years who suffer from NF type I or type II, were eligible for study entry. The third edition of the Wechsler Intelligence Scale (WISC-III) was used to measure participants’ cognitive function. RESULTS: Preliminary results of this ongoing study are presented. Patients’ recruitment was limited by the coronavirus disease 2019 (COVID-19) restrictions. At this stage, the research involved 10 participants suffering from NF, with mean (± SD) age of 11.55 (± 1.80) years and a male-to-female ratio of 1. The mean (± SD) full-scale intelligence quotient (IQ) was 85.50 (± 18.80), corresponding to the 0.3rd to 73th percentile range. The mean (± SD) scores of performance IQ and verbal IQ were 84.90 (± 17.43) and 89.40 (± 17.23) respectively, corresponding to the 1st to 73rd percentile range for both subscales. CONCLUSION: Significant cognitive deficits, according to the percentile scores of WISC-III, were demonstrated in the small number of children and adolescents suffering from NF (type I or type II). Cognitive assessment, as part of the multidisciplinary approach of these patients is warranted, to aid timely educational interventions and improve patient learning outcomes.
Pediatric cancer and its treatment may have an impact on the neurocognitive functions of childhood cancer survivors (CCS). The aim of the present meta-analysis was to compare the intelligence ...quotient (IQ) scores between CCS of acute lymphoblastic leukemia (ALL) and controls. A comprehensive electronic search identified original research articles that reported scores of the Wechsler Intelligence Scale (WISC; WISC-III, WISC-IV and WISC-R) for children and adolescents, aged 6-16 years at evaluation, survivors of ALL and healthy controls. The included CCS had completed anticancer treatment and were in remission at the time of assessment. A total of 16 studies were included in the meta-analysis, out of 128 extracted studies, and involved a total of 1,676 children and adolescents: 991 CCS (ALL) and 685 healthy controls. Among the studies, a random effects model revealed a moderate estimate of effect size standardized mean difference (SMD), -0.78; 95% CI, -1.05 to -0.50, indicating that the WISC scores for total IQ were significantly lower in the CCS than in the controls. The mean total IQ range was 85.2-107.2 in the CCS and 88.4-114.1 in the controls. The difference in the mean total IQ between controls and CCS ranged from -13.8 to 20.6. As regards the WISC scores for verbal IQ, 11 studies were included. A random effects model revealed a moderate estimate of effect size (SMD, -0.71; 95% CI, -1.05 to -0.38), indicating that the WISC scores for verbal IQ were significantly lower in the CCS than in the controls. Among the 9 studies that had available data for performance IQ scores, a fixed effect model revealed a moderate estimate of effect size (SMD, -0.80; 95% CI, -1.09 to -0.52), indicating that the WISC scores for performance IQ were significantly lower in the CCS than in the controls. As the survival rates of children and adolescents with ALL are steadily increasing, regular, lifelong follow-up for neurocognitive late effects is imperative in order to improve their education and employment prospects and overall, their quality of life.
Pediatric cancer and its treatment may have an impact on the neurocognitive functions of childhood cancer survivors (CCS). The aim of the present meta-analysis was to compare the intelligence ...quotient (IQ) scores between CCS of acute lymphoblastic leukemia (ALL) and controls. A comprehensive electronic search identified original research articles that reported scores of the Wechsler Intelligence Scale (WISC; WISC-III, WISC-IV and WISC-R) for children and adolescents, aged 6-16 years at evaluation, survivors of ALL and healthy controls. The included CCS had completed anticancer treatment and were in remission at the time of assessment. A total of 16 studies were included in the meta-analysis, out of 128 extracted studies, and involved a total of 1,676 children and adolescents: 991 CCS (ALL) and 685 healthy controls. Among the studies, a random effects model revealed a moderate estimate of effect size standardized mean difference (SMD), -0.78; 95% CI, -1.05 to -0.50, indicating that the WISC scores for total IQ were significantly lower in the CCS than in the controls. The mean total IQ range was 85.2-107.2 in the CCS and 88.4-114.1 in the controls. The difference in the mean total IQ between controls and CCS ranged from -13.8 to 20.6. As regards the WISC scores for verbal IQ, 11 studies were included. A random effects model revealed a moderate estimate of effect size (SMD, -0.71; 95% CI, -1.05 to -0.38), indicating that the WISC scores for verbal IQ were significantly lower in the CCS than in the controls. Among the 9 studies that had available data for performance IQ scores, a fixed effect model revealed a moderate estimate of effect size (SMD, -0.80; 95% CI, -1.09 to -0.52), indicating that the WISC scores for performance IQ were significantly lower in the CCS than in the controls. As the survival rates of children and adolescents with ALL are steadily increasing, regular, lifelong follow-up for neurocognitive late effects is imperative in order to improve their education and employment prospects and overall, their quality of life. Key words: cancer, acute lymphoblastic leukemia, survivors, learning difficulties, cognitive, IQ, intelligence, children, adolescents, chemotherapy, radiotherapy, WISC, education
Objective
To describe the rate and type of adverse effects (AEs) and the frequency of disease flares after COVID-19 vaccination and to assess the reasons for vaccination hesitancy (non-vaccination) ...in SRD patients.
Methods
Telephone interviews were conducted of SRD patients consecutively enrolled (15/06/2021–1/7/2021). Participants were asked about the type of AEs and disease flare after vaccination. Reasons for vaccination hesitancy were recorded. Univariate and mutivariable analyses examined associations of demographic, clinical and other features, with occurrence of AEs, disease flare and non-vaccination. For the latter, association with negative vaccination behaviour (not influenza vaccinated for the last 2 years) and nocebo-prone behaviour (denoting AEs attributed to negative expectations Q-No questionnaire) was also tested.
Results
561 out of 580 contacted patients were included in the study. 441/561 (78.6%) patients were vaccinated 90% (Pfizer, Moderna), 10% (Astra-Zeneca). AEs were reported by 148/441 (33.6%), with rates being comparable between the three vaccines. AEs were more common in females and those with chronic obstructive pulmonary disease OR, 95% CI; females: 2.23 (1.30–3.83); COPD: 3.31 (1.24–8.83). Disease flare was reported in 9/441 (2%) patients. For those unvaccinated, fear that the vaccine would be harmful (53.3%), could cause disease flare (24.2%) and/or could cause thrombosis (21.7%) were the main reasons to do so. Multivariable analysis identified as independent variables for non-vaccination: nocebo-prone behaviour (OR; 95% CI, 3.88; 1.76–8.55), negative vaccination behaviour (6.56; 3.21–13.42) and previous COVID-19 infection (2.83; 1.13–7.05). Higher educational status was protective (0.49; 0.26–0.92).
Conclusion
No new safety signals for COVID-19 vaccination were observed. Vaccination campaign should target SRD patients with nocebo-prone and negative influenza vaccination behaviour.
To assess non-compliance and potential changes in seasonal flu vaccination coverage before and during the Covid-19 pandemic in patients with autoimmune rheumatic diseases (ARDs). Consecutive patients ...with ARDs followed-up in 2 tertiary hospitals were telephone-interviewed (December 12–30, 2020) regarding seasonal flu vaccination during the 2019/20 and 2020/21 time periods. Self-reported disease flares that occurred after flu vaccination, as well as reasons for non-vaccination were recorded. One thousand fifteen patients were included. The rate of flu vaccination increased from 76% before to 83% during the COVID-19 pandemic (
p
= 0.0001). The rate of self-reported disease flares was < 1% among vaccinated patients. Reasons for not vaccination in both periods, respectively, included: ‘was not recommended by their rheumatologists’ (35.0vs.12.2%,
p
< 0.0001), ‘did not feel that they would have any benefit’ (36.9 vs. 32.6%), felt unsafe to do so (27.5 vs. 30.2%), or other reasons (18.9 vs. 23.8%). By multivariate analysis, age OR = 1.03 (95% CI 1.02–1.04) vs. 1.04 (95% CI 1.02–1.05) and treatment with biologics OR = 1.66 (95% CI 1.22–2.24) vs. 1.68 (95% CI 1.19–2.38) were independent factors associated with vaccination in both periods. These findings, although are temporally encouraging, emphasize the need for continuous campaigns aiming at increasing patients’ and physicians’ awareness about the benefits of vaccination.
To present the characteristics of patients with potential difficult-to-treat (D2T) psoriatic arthritis (PsA).
We used data from the Greek multicentre registry of PsA patients. D2T-PsA was defined as ...follows: patients with at least 6-months disease duration, who have failed to at least 1 csDMARD and at least 2 bDMARDs/tsDMARDs with a different mechanism of action and have either at least moderate disease activity (MODA) defined as DAPSA > 14, and/or are not at minimal disease activity (MDA). Demographic and clinical characteristics were compared between D2T and non-D2T PsA patients. In two sensitivity analyses, patients classified as D2T solely according to the MODA or MDA criterion were examined separately.
Among 467 patients included, 77 (16.5%) were considered D2T and 390 non-D2T PsA. Compared with non-D2T, patients with D2T PsA presented more commonly with extensive psoriasis (p< 0.0001) and were more likely to have higher BMI (p= 0.023) and a history of inflammatory bowel disease (p= 0.026). In the MODA and MDA sensitivity analyses, 7.5% and 12.5% of patients were considered D2T, respectively. In both sensitivity analyses, extensive psoriasis was again identified as an independent variable for D2T PsA (p= 0.001 and p= 0.008, respectively). Moreover, female gender (p= 0.034) in the MODA analysis and axial disease (p= 0.040) in the MDA analysis were independent variables for D2T PsA.
Despite the availability of therapies, D2T PsA is common in real-life cohorts of patients with PsA and extensive psoriasis. High BMI, female gender, axial-disease, and history of IBD were also associated with D2T PsA.
Psoriatic arthritis (PsA) is a heterogenous chronic inflammatory disease affecting skin, joints, entheses, and spine with various extra-musculoskeletal manifestations and comorbidities. The reported ...patient, disease and treatment characteristics in the modern therapeutic era are limited.
In this cross-sectional, multi-centre, nationwide study, we recorded the demographic, clinical, and therapeutic characteristics as well as the comorbidities of patients with PsA seen for 1 year (1/1/2022-31/12/2022).
923 patients (55% females) with a median (IQR) age of 57 (48-65) years and a mean disease duration of 9.5 years were enrolled. Family history of psoriasis and PsA was noted in 28.3% and 6.3%, respectively. Most patients had limited psoriasis (BSA<3: 83%) while enthesitis, dactylitis, nail and axial involvement reported in 48.3%, 33.2%, 43% and 25.9% of patients, respectively. Regarding comorbidities, approximately half of patients had dyslipidaemia (42%) or hypertension (45.4%), 36.8% were obese and 17% had diabetes while 22.7% had a depressive disorder. Overall, 60.1% received biologics and among them more patients treated with anti-IL-17 or -12/23 agents were on monotherapy (64.2%) compared to those on TNFi monotherapy (49.4%, p=0.0001). The median PsA activity as assessed by the DAPSA score was 6 (IQR: 2.3 - 13.1) with 46% of patients reaching minimal disease activity status (MDA).
In this large, real life, modern cohort of patients with PsA with frequent comorbidities who were treated mainly with biologics, almost half achieved minimal disease activity. These results show the value of existing therapeutic approaches while at the same time highlight the existing unmet needs.
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