Publications by authors named "Marrani E"
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.
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.
Article Synopsis
- * The review aims to gather and assess existing MRI scoring systems to create an evidence-based foundation for a universal standardized system that can be used in both research and clinical settings.
- * A systematic search of electronic databases will be conducted to consolidate information on MRI scanning protocols for evaluating muscle involvement in IIMs, with the goal of producing guidelines for consistent clinical and research practices.
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.
Article Synopsis
- This study investigates the clinical features and treatment outcomes of three patients with juvenile idiopathic arthritis (JIA) and inborn errors of immunity (IEI) treated with biological DMARDs.
- It highlights the cases of three young patients, two with 22q11.2 deletion syndrome and one with X-linked agammaglobulinemia, who had previously unresponsive JIA but achieved remission with specific biological therapies (etanercept, Adalimumab, and sirolimus plus abatacept).
- The findings indicate that biological therapies can effectively manage disease activity in patients with these conditions without any observed adverse drug reactions.
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.
Introduction: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.
View Article and Find Full Text PDFThis 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 PDFObjective: The COVID-19 pandemic has affected patient care in general. We aimed to analyze the impact of the pandemic on pediatric rheumatology practice.
Methods: An online survey including 22 questions was created by the representatives of the Emerging RheumatoloGists and rEsearchers (EMERGE) group of the Pediatric Rheumatology European Society (PReS) on SurveyMonkey.
: 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 PDFObjectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis.
View Article and Find Full Text PDFSjögren's disease (SD) is a chronic autoimmune disease primarily affecting lacrimal and salivary glands. The diagnosis of pediatric SD mostly relies on clinical suspect, resulting in a significant diagnostic delay. Recently, ultrahigh-frequency ultrasound (UHFUS) of labial glands has been proposed as a diagnostic method in adults with suspected SD.
View Article and Find Full Text PDFObjective: 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: Juvenile localized scleroderma (JLS) is a rare sclerosing disorder of childhood which can result in permanent morbidity and functional disability, if not effectively treated. Treatment should be started in the inflammatory phase before the development of any complication and/or damage.
Areas Covered: In this review, we will discuss how to assess disease activity and damage in JLS, and propose an escalation plan for systemic treatment, according to a treat-to-target concept.
Objective: Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR.
Methods: Consenting children or young adults with CNO were enrolled into CHOIR.