Publications by authors named "Ilaria Pagnini"

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.
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  • 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.
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  • 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.
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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.

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Article Synopsis
  • 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.
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The burden of group A streptococcus (GAS) infection and its rheumatic sequelae remains dramatically high, especially in low-income countries. Recently, an increased number of Acute Rheumatic Fever (ARF) cases was documented in many regions of Italy. The diagnosis of rheumatic sequelae relies on clinical signs and on the evaluation of the Antistreptolysin O titre (ASO), whose variations are globally reported.

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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.

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: 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.

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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.

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Introduction: 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.

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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).

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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.
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Patients with cystic fibrosis often complain of joint manifestations. However, only a few studies have reported the association between cystic fibrosis and juvenile idiopathic arthritis and addressed the therapeutic challenges of these patients. We describe the first paediatric case of a patient affected by cystic fibrosis, Basedow's disease and juvenile idiopathic arthritis who was contemporarily treated with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and anti-tumor necrosis factor α (anti-TNFα).

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Unlabelled: Leishmaniasis is a cause of infection associated with hemophagocytic lymphohistiocytosis (HLH). The measurement of the CD8 CD38/HLA-DR T cells in children presenting with acute onset of shock and multisystem organ failure represents an important parameter to distinguish HLH from sepsis or healthy control.

Conclusion: We report a case series of 4 Italian children suffering from HLH secondary to visceral Leishmaniasis in which the lymphocyte subset assay suggests a potential role of CD38/HLA-DR CD8 T cells as HLH diagnostic biomarkers.

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Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare genetic disease characterized by tetrad camptodactyly, noninflammatory arthropathy, coxa vara deformity, and pericardial effusion. Arthropathy typically affects large joints and presents with joint swelling in the absence of other signs of inflammation. We described the case of a girl affected by CACP syndrome caused by a novel compound heterozygous variant in proteoglycan 4 gene (c.

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Neurological manifestations related to SARS-CoV-2 infection in adults have been largely reported since the beginning of the pandemic. Subsequent large-scale studies involving children confirmed the occurrence of neurological symptoms associated with SARS-CoV-2 infection also among paediatric patients, especially in the context of paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS). At this regard, we report the challenging case of a 10-month-old baby with PIMS-TS complicated by acute cerebral oedema successfully treated with intravenous immunoglobulins, corticosteroids and anakinra.

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