Publications by authors named "Maria V Mastrolia"
Article Synopsis
- - Behçet's syndrome (BS) is a rare, chronic inflammatory disorder mostly affecting adults in their 30s and 40s, but young patients can also experience it, requiring ongoing medical attention as they grow.
- - Transitional care programs are designed to support young patients with chronic conditions like BS, helping them gain independence and self-management skills for their health.
- - There is a lack of established guidelines on the timing and effectiveness of these transition plans for BS patients, but tailored approaches could enhance care continuity and improve quality of life for young patients.
Article Synopsis
- The study investigates the role of CD38/HLA-DRCD8 lymphocytes in distinguishing hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) from conditions like sepsis and systemic juvenile idiopathic arthritis.
- Researchers found that while CD38/HLA-DRCD8 frequencies were higher in HLH/MAS patients compared to other febrile conditions, the difference was not statistically significant.
- The findings suggest that CD38/HLA-DRCD8 levels are not exclusive to HLH, as they also increase in response to infections, highlighting their limited utility as an HLH-specific marker.
Article Synopsis
- The study investigates treatment options for children with chronic non-infectious uveitis (cNIU) who don't respond to the initial anti-TNF therapy, focusing on whether to switch to another anti-TNF or another biologic.
- A systematic review and meta-analysis was done, analyzing data from January 2000 to August 2023, with 23 articles covering 150 children, to evaluate treatment efficacy based on intraocular inflammation improvement.
- Results indicate that both tocilizumab and infliximab show a higher response rate compared to other treatments, suggesting these might be better options for managing cNIU after the first anti-TNF fails.
Uveitis as predictor of disease flare after the first anti-TNF withdrawal in oligoarticular and polyarticular juvenile idiopathic arthritis: a multicentric Italian experience.
Clin Exp Rheumatol
September 2024
Article Synopsis
- The study investigates the effects of stopping TNF inhibitors (TNFi) in children with juvenile idiopathic arthritis (JIA) who show persistent disease inactivity and identifies factors related to relapse.
- A multicentric analysis of medical records from 136 JIA patients who discontinued TNFi treatment revealed that 79.4% experienced a relapse within a median of 5 months after stopping therapy.
- Key predictors for relapse include younger age at onset, presence of uveitis, duration of treatment tapering, and not having persistent oligoarticular JIA.
Objectives: To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children.
Methods: Prospective collection of demographics, clinical and treatment data. Assessment of type 1 interferon (IFN) score, CXCL9, CXCL10, Interleukin (IL)18, IFNγ, IL6, IL1b at disease onset and at recovery.
Introduction: Vascular events account for a considerable burden of morbidity and mortality in Behçet syndrome (BS). Thrombosis occurs in 1.8-21 % pediatric BS patients, even if the real prevalence is still largely unknown.
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- Childhood Mixed Connective Tissue Disease (cMCTD) is a rare pediatric condition displaying symptoms of various connective tissue diseases, making it difficult to diagnose.
- A systematic literature review examined 39 articles involving 215 patients to identify early clinical features of cMCTD and to evaluate symptoms not included in existing diagnostic criteria.
- Findings revealed a diverse range of symptoms at onset, with common manifestations like Raynaud's phenomenon, arthritis, and muscular involvement, suggesting the need for new diagnostic approaches for earlier detection of cMCTD.
This narrative review aims to report the main clinical manifestations, therapeutic strategies, outcomes, and complications of acute SARS-CoV-2 infection in childhood and to summarize the data relating the SARS-CoV-2 vaccination efficacy and safety in pediatric age. SARS-CoV-2 infection mostly occurs asymptomatically in the pediatric population, while multisystem inflammatory syndrome in children (MIS-C) represents the most severe coronavirus disease 2019 (COVID-19)-related illness, a life-threatening event with a high morbidity rate. After the development of SARS-CoV-2 vaccines and their subsequent approval in children, the rate of infection as well as the number of its related complications have shown a drastic decrease.
View Article and Find Full Text PDF: Childhood chronic non-infectious uveitis (cNIU) is a challenging disease that needs close monitoring. Slit lamp evaluation (SLE) is the cornerstone of ophthalmological evaluation for uveitis, but it is affected by interobserver variability and may be problematic in children. Laser flare photometry (LFP), a novel and objective technique, might be used in children with uveitis.
View Article and Find Full Text PDFBackground: The treatment of multisystem inflammatory syndrome in children unresponsive to first-line therapies (IVIG and/or steroids) is challenging. The effectiveness of IL-1 receptor antagonist, anakinra, is debated.
Patients And Methods: We conducted an anonymous retrospective multicenter study on MIS-C patients treated with anakinra in Italy from January 2020 to February 2021.
Objective: The main aim of this study was to define the best treatment option for multisystem inflammatory syndrome in children (MIS-C) and to analyse the role of anakinra.
Methods: This is a multicentre retrospective cohort study. Patients were treated according to the attending physician's decision.
Objective: Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement, including interstitial lung disease (ILD). Available data on JIIM-associated ILD are very limited. We performed a systematic review of the available clinical, laboratory, and radiological features of JIIM-associated ILD.
View Article and Find Full Text PDFIntroduction: Interleukin-17 (IL-17) is a family of cytokines that plays a key role in several rheumatic diseases in both adults and children. In the last few years, several drugs targeting IL-17 have been developed.
Areas Covered: We present a review on the current state of the art regarding the use of anti-IL17 in childhood chronic rheumatic diseases.
Multisystem Inflammatory Syndrome in Children (MIS-C) is a systemic hyperinflammatory disorder that is associated with a hypercoagulable state and a higher risk of thrombotic events (TEs). We report the case of a 9-year-old MIS-C patient with a severe course who developed a massive pulmonary embolism that was successfully treated with heparin. A literature review of previous TEs in MIS-C patients was conducted (60 MIS-C cases from 37 studies).
View Article and Find Full Text PDFIntroduction: Deficiency of adenosine deaminase 2 (DADA2) is a rare monogenic autoinflammatory disease, whose clinical phenotype was expanded since the first cases, originally described as mimicker of polyarteritis nodosa, with immunodeficiency and early-onset stroke.
Methods: A systematic review according to PRISMA approach, including all articles published before the 31st of August 2021 in Pubmed and EMBASE database was performed.
Results: The search identified 90 publications describing 378 unique patients (55.
Challenges and management of childhood noninfectious chronic uveitis.
Expert Rev Clin Immunol
June 2023
Article Synopsis
- Childhood uveitis is a serious eye condition that can cause complications and blindness if not recognized and treated properly, making diagnosis and management challenging.
- This review covers the causes, diagnostic methods, risk factors for noninfectious uveitis in children, and the difficulties of performing eye exams on young patients.
- Expert opinions emphasize the importance of accurate diagnosis and appropriate treatment timing, as well as exploring new techniques and clinical trials to improve outcomes for affected children.
Among the 365 children diagnosed as having Kawasaki disease (KD), only 5 children (1.4%) presented with splenomegaly: 2 complicated by macrophage activation syndrome and 3 ultimately received a diagnosis of alternative systemic illness. Splenomegaly is atypical in KD and a potential marker of an underling complication, namely macrophage activation syndrome, or diagnosis other than KD.
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