Introduction: Pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) is a relatively new but controversial diagnosis affecting hundreds of children and their families. It is generally thought to be an autoimmune disorder resulting from a streptococcal infection that causes significant and bizarre behavioral changes in children. Currently no definitive diagnostic or treatment modalities exist, which has led to misdiagnoses, ineffective treatments, and delayed care.
Methods: A qualitative study was conducted that included 60 families with at least one child diagnosed with PANDAS. The purpose was to explore how families experience the disorder and what nurses can do to provide effective care.
Results: Using paradigmatic analysis of transcribed interviews, three themes were identified: fear, frustration, and not being heard.
Discussion: Results from this study suggest that more information is needed to better understand this challenging phenomenon from both medical and nursing perspectives. The study also reaffirms the importance of practicing the art of nursing, especially when the science is not yet established.
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http://dx.doi.org/10.1016/j.pedhc.2015.03.005 | DOI Listing |
J Clin Invest
January 2025
Institute for Research in Biomedicine (IRB), Bellinzona, Switzerland.
Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease characterized by the presence of autoantibodies, including those targeting O-phosphoseryl-tRNA:selenocysteine-tRNA synthase (SepSecS), also known as soluble liver antigen (SLA). Anti-SepSecS antibodies have been associated with a more severe phenotype, suggesting a key role for the SepSecS autoantigen in AIH. To analyze the immune response to SepSecS in patients with AIH at the clonal level, we combined sensitive high-throughput screening assays with the isolation of monoclonal antibodies (mAbs) and T cell clones.
View Article and Find Full Text PDFNarra J
December 2024
Department of Pediatrics, Faculty of Medicine, Universitas Brawijaya, Malang, Indonesia.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse manifestations, requiring long-term treatment that can have side effects, particularly in pediatric patients. has shown potential for improving SLE symptoms due to its anti-inflammatory and immunomodulatory effects. The aim of this study was to investigate the immunomodulatory effect of oil (NSO) on disease activity, T lymphocyte activity and inflammatory cytokine profiles in pediatric SLE patients.
View Article and Find Full Text PDFJ Rheumatol
January 2025
Jessica K. Gordon, Division of Rheumatology, Hospital for Special Surgery, New York City, NY; Department of Medicine, Weill Cornell Medicine, New York City, NY.
Objective: To evaluate the psychometric properties of the Scleroderma Skin Questionnaire (SSQ), a novel patient-reported outcome (PRO) to assess systemic sclerosis (SSc) related skin symptoms.
Methods: The SSQ was administered to 799 adults (mean age 52.7; 82% female) enrolled in the SSc Collaborative National Quality and Efficacy Registry (CONQUER).
J Rheumatol
January 2025
Florenzo Iannone, Rheumatology Unit - Department of Precision and Regenerative Medicine of Jonian Area University of Bari, Bari, Italy.
Objective: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry.
Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included.
J Clin Immunol
January 2025
Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA, USA.
Reduced function or hypomorphic variants in recombination-activating genes (RAG) 1 or 2 result in a broad clinical phenotype including common variable immunodeficiency (CVID) and even adult-onset disease. Milder RAG variants are less characterized. Here we describe the longitudinal course of a milder combined RAG deficiency in 3 of 7 siblings sharing the same RAG2 mutations over a 50-year study.
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