Cyclophosphamide treatment with a comparison in both pediatric rheumatology and pediatric nephrology practices.

Background: Cyclophosphamide (CYC) is an inactive alkylating agent that transforms the alkyl radicals into other molecules and is used in combination with systemic corticosteroids in the treatment of many childhood rheumatic diseases, such as systemic lupus erythematosus (SLE), and ANCA-associated vasculitis (AAV). In recent years, rituximab (RTX), a B-cell-targeting anti-CD20 monoclonal antibody, has emerged as a new alternative treatment modality over CYC for induction therapy of childhood-onset rheumatic diseases. Clinicians adopt different practices for using CYC particularly in relation to indications, posology, pre-treatment laboratory work-up, post-treatment follow-up, and screening pre- and post-treatment vaccination status.

Evaluation of ILAR and PRINTO classifications for juvenile idiopathic arthritis: oligoarticular JIA vs early-onset ANA-positive JIA.

Objectives: The International League of Associations for Rheumatology (ILAR) juvenile idiopathic arthritis (JIA) classification was revisited by the Pediatric Rheumatology International Trials Organization (PRINTO) in 2018. Classifications should establish uniform groups to assist physicians in providing optimal care. Therefore, we evaluated changes proposed by PRINTO to highlight their impact on forming consistent groups regarding uveitis and treatment responses, particularly focusing on early-onset anti-nuclear antibody (ANA)-positive JIA.

Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management.

Background: Pediatric patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) are at an increased risk of arterial and venous thromboembolism (AVTE). Although the exact mechanisms underlying AVTE remain unclear, eosinophils play a pivotal role in AVTE.

Main Body: Current guidelines lack evidence-based recommendations, particularly concerning anticoagulant and antiplatelet treatments for this condition.

Juvenile idiopathic arthritis management: insights into the utilization of intra-articular corticosteroid injections.

Background: Juvenile idiopathic arthritis (JIA) is a common chronic rheumatic disease in children, requiring careful management to reduce both short- and long-term morbidity. In this study, our objective was to assess the clinical features of patients diagnosed with JIA who received intra-articular corticosteroid injections (IACI).

Methods: In this retrospective study, we evaluated the clinical and laboratory characteristics of 225 JIA patients monitored from January 2012 to October 2023 at a tertiary care center.

Clinical Associations of E148Q Heterozygosity: What to Expect From E148Q?

Objective: The exact effects of MEFV variants on inflammation are still under investigation, and reports on variants of unknown significance, particularly the E148Q variant, have been conflicting. Therefore, this study aims to investigate patients exhibiting E148Q heterozygosity, focusing on diagnoses and disease courses to assist physicians in interpreting the variant.

Methods: Data of pediatric patients presenting to the Pediatric Rheumatology clinic between November 2016 and September 2023, exhibiting only E148Q heterozygosity in MEFV gene analysis, were extracted.

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Unlabelled: Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey.

Thymic carcinoma presenting with overlap polyarthritis and myositis: A rare paraneoplastic syndrome in childhood.

Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient.

Adhesive small-bowel obstruction as a challenging complication of familial Mediterranean fever: A case-based review.

Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disorder, characterized by recurrent and self-limiting episodes of fever and serosal inflammation. Recurrent serositis may rarely lead to the formation of adhesions in the peritoneum, which may result in mechanical bowel obstruction. The symptoms, such as abdominal pain and vomiting, may mimic typical FMF attacks, resulting in misdiagnosis and severe morbidity, including strangulation and intestinal necrosis.

Comparison of clinical and laboratory features and treatment responses in patients with clinically amyopathic juvenile dermatomyositis and classical juvenile dermatomyositis.

Aim: The aim of this study was to compare the clinical and laboratory features, treatment choices and responses, and outcomes between patients with clinically amyopathic juvenile dermatomyositis (CAJDM) and classical juvenile dermatomyositis (JDM).

Methods: We retrospectively reviewed the medical records of patients with CAJDM and JDM, and compared the 2 groups' clinical and laboratory data, treatment agents and responses, and outcomes.

Results: There were 38 JDM and 12 CAJDM patients, with female dominance.

Venlafaxine intoxication in an adolescent presenting with severe lactic acidosis.

Venlafaxine is a selective serotonin noradrenaline reuptake inhibitor and commonly prescribed antidepressant in adults. Most patients overdosing with venlafaxine develop only mild symptoms. Severe toxicity is reported with the most common symptoms being CNS depression, serotonin toxicity, seizure, or cardiac conduction abnormalities.