Publications by authors named "SIMONINI G"
Background: Atraumatic avascular necrosis (AVN) is a severe condition that may complicate the course of rheumatic diseases and contribute to long-term damage. However, there is a lack of evidence on this rare event in pediatric rheumatology. The aim of our study was to evaluate the occurrence of avascular necrosis in the context of rheumatologic diseases in Italy and to describe the main demographic and clinical features of AVN patients, with a particular focus on treatment background.
View Article and Find Full Text PDFObjectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.
Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.
Background And Aims: The aim of the present study was to assess prevalence and disease outcomes of arthritis in a nationwide cohort of pediatric patients with inflammatory bowel disease (IBD).
Methods: We collected data of pediatric IBD patients experiencing arthritis from the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition IBD registry. We gathered baseline and one-year follow-up data on concomitant IBD and arthritis diagnosis.
Uveitis in paediatric Behçet disease: a large multicentric Italian cohort.
Ther Adv Musculoskelet Dis
December 2024
Background: Behçet disease (BD) is a rare disease in childhood and its uveitis may lead to blindness if not properly treated.
Objectives: We aim to describe a cohort of paediatric BD patients with uveitis.
Design: This is a multicentric retrospective study.
Brain MRI White Matter Abnormalities in Pediatric Non-Infectious Uveitis.
Ocul Immunol Inflamm
October 2024
Article Synopsis
- Childhood chronic non-infectious uveitis (cNIU) is a complex condition that can mimic other diseases, like demyelinating disorders, and this study focuses on the brain MRI findings in affected children.
- A total of 123 children with cNIU were analyzed, revealing that 26.8% exhibited white matter abnormalities (WMA), particularly among those with non-anterior uveitis and more frequently in males.
- The study suggests that while brain MRIs might be beneficial for monitoring cNIU, the clinical implications of the observed WMAs remain unclear, highlighting the need for interdisciplinary assessment and further long-term studies.
Acute necrotizing encephalopathy (ANE) is a severe neurological condition that is diagnosed clinically and upon specific radiological imaging. Medical treatment of this condition is uncertain, and timing is likely very important to minimize brain damage and systemic inflammation. The present case series describes three patients suffering from ANE secondary to influenza A/H1N1 infection during a recent outbreak, treated with increasingly aggressive anti-inflammatory approach, and with significantly different outcomes.
View Article and Find Full Text PDFArticle 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.
Background: Artificial intelligence (AI) has become a popular tool for clinical and research use in the medical field. The aim of this study was to evaluate the accuracy and reliability of a generative AI tool on pediatric familial Mediterranean fever (FMF).
Methods: Fifteen questions repeated thrice on pediatric FMF were prompted to the popular generative AI tool Microsoft Copilot with Chat-GPT 4.
Article Synopsis
- Juvenile Idiopathic Arthritis (JIA) is a common chronic childhood condition that can lead to uveitis in 20-25% of cases, requiring regular screening for early detection.
- Uveitis is typically chronic, asymptomatic, and involves inflammation in the front part of the eye, making collaboration between paediatric rheumatologists and ophthalmologists essential for effective management.
- Treatment usually begins with methotrexate, and if ineffective, may progress to adalimumab, with alternative options like anti-IL6 and anti-TNF agents available for persistent cases, though evidence for these is limited.
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.
Article Synopsis
- The study analyzes 20 years' worth of data on Acute Hemorrhagic Edema of Infancy (AHEI) from a national referral center, involving a retrospective look at patients from 2004 to 2023.
- Out of 21 diagnosed patients, most were young children (median age of 18 months) and presented with prodromal symptoms and cutaneous findings such as targetoid purpuric plaques primarily on the face and upper limbs.
- The results showed a mostly benign course of AHEI with a hospitalization rate of 62%, and there were no significant complications related to the condition.
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: The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome.
Study Design: Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected.
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.
Article Synopsis
- - The study aimed to establish and validate specific cutoff values for the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) to differentiate between various disease activity levels in children with systemic juvenile idiopathic arthritis.
- - Researchers used data from 400 patients across multiple countries, applying different methods to determine these cutoffs, ensuring robust validation through comparison of physician assessments.
- - The identified cutoffs were found to effectively separate inactive disease, minimal disease activity, moderate disease activity, and high disease activity, making them reliable for clinical use and research purposes.
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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- - A significant outbreak of influenza A(H1N1pdm09) affected seven young children in Tuscany, Italy, between December 2023 and January 2024, with a median age of 52 months.
- - The children exhibited a range of symptoms, from mild encephalopathy to severe acute necrotizing encephalopathy (ANE), resulting in coma and multiorgan failure, with one child sadly passing away.
- - This report emphasizes the necessity for healthcare providers to quickly recognize and treat acute encephalopathy linked to H1N1 influenza and highlights the importance of vaccination to prevent severe illness in young children.
Inflammatory bowel diseases (IBD) encompass a group of chronic inflammatory disorders primarily affecting the gastrointestinal tract but capable of impacting various organs, including the eye, with uveitis being the most common ocular condition. We assessed uveitis prevalence and clinical features in a nationwide cohort of pediatric IBD. Among 4229 cases, six patients (four Crohn's disease, one ulcerative colitis, and one unclassified IBD) were identified, resulting in an overall prevalence rate of 141.
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