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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
A 4 year old girl presents with fever, rash, conjunctivitis and bilateral cervical lymph node enlargement. Chest radiograph demonstrates bilateral atelectasis and a small right pleural effusion. A diagnosis of Kawasaki disease is made.
Novel Treatments of Autoimmune Conditions
Published in Irun R. Cohen, Perspectives on Autoimmunity, 2020
Y. Shoenfeld, Y. Tomer, O. Ben-Yehuda
High-dose IVGG has also been studied in Kawasaki disease;152 though the disease is of unknown etiology, it is thought that immunological mechanisms play a major role. The treat posed by the disease is the development of coronary artery lesions. In a multicenter controlled trial in Japan, high-dose IVGG plus aspirin was compared with aspirin alone as treatment for Kawasaki disease: coronary artery lesions developed in 42% of the aspirin-treated group, compared with 15% in the IVGG plus aspirin group. 152A successful treatment of autoimmune hemolytic anemia with high-dose IVGG was recently reported.152a
Section 2
Published in Padmanabhan Ramnarayan, MCQs in Paediatrics for the MRCPCH, Part 1, 2017
The criteria to diagnose Kawasaki disease include fever > 39°C for > 5 days, non-purulent conjunctivitis, > 2 cm cervical adenopathy bilaterally, mucous membrane involvement in the form of strawberry tongue/lip cracking, skin rash and redness, swelling and peeling of the fingers/toes.
Plasma Cyclooxygenase-2 as a Potential Biomarker for Early Diagnosis of Kawasaki Disease
Published in Fetal and Pediatric Pathology, 2023
Kawasaki disease (KD) is a systemic vasculitis of medium and small-sized vessels characterized by febrile symptoms of unknown etiology [1]. KD mainly affects children under 5 years old, and can cause coronary artery lesions (CAL) including coronary artery dilatation, aneurysms, and giant aneurysms. In recent years, KD has become the major cause of acquired heart disease among pediatric diseases in countries with high medical standards [2]. The initial treatment for KD is a single high dose of intravenous immunoglobulin (IVIG) combined with acetylsalicylic acid (ASA), which is based on evidence from clinical trials. Approximately 10% to 20% of KD patients continue to have a fever at least 36 h after the initial treatment with IVIG, which is defined as IVIG resistant [3]. Numerous studies have demonstrated that patients who are resistant to standard IVIG treatment have a higher risk of developing CAL [1]. Biomarkers and better predictive models would help choose the appropriate initial treatment, preventing and reducing CAL.
Bilateral Anterior Uveitis Following Paediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 – Case Report and Focused Review
Published in Ocular Immunology and Inflammation, 2023
Aliénor Vienne-Jumeau, Antoine P. Brézin, Amina Debieb
Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS), also referred as Multi Inflammatory Syndrome in Children (MIS-C) in the USA, is a systemic hyperinflammatory state newly described in children recently infected with SARS-CoV-21 Clinical criteria include fever, disease severity and dysfunction of two or more organ systems.2–4 It shares common characteristics with Kawasaki disease.1,2,5 From the ophthalmologist perspective, non-purulent conjunctival injection is the most common symptom, which is found in both diseases.2,6–9 Anterior uveitis is another typical symptom of Kawasaki disease, which has raised interest as a potential help for early diagnosis.10,11 Here, we present a case of an anterior uveitis in a pre-adolescent boy diagnosed with PIMS-TS, whom we followed for six-month. We also review studies reporting uveitis in children with PIMS-TS to outline common history and evolution.
Interleukin-35 Enhances Regulatory T Cell Function by Potentially Suppressing Their Transdifferentiation into a T Helper 17-Like Phenotype in Kawasaki Disease
Published in Immunological Investigations, 2023
Kawasaki disease is an acute systemic vasculitis that predominantly affects children younger than five-years-old (Agarwal and Agrawal 2017). Typical Kawasaki disease is generally diagnosed based on the presence of fever persisting for at least five days, accompanied by any four of the following five findings: acute/subacute changes in the extremities, polymorphous exanthema, bilateral bulbar conjunctival injection without exudates, changes in the lips and oral cavity (e.g. erythema, strawberry tongue, lip cracking), and cervical lymphadenopathy (Singh et al. 2018). Although its etiopathogenesis remains unclear, it is thought to involve complex interactions among different genetic factors, infections, and immune responses mechanisms (Agarwal and Agrawal 2017). Superantigens or conventional antigens trigger the innate immune system at an early stage, leading to an increased number of activated neutrophils and monocytes (Matsubara et al. 2005). Both effector and regulatory T cells (Tregs) can be detected in the peripheral blood in the first week after the onset of fever, suggesting the involvement of the adaptive immune response in Kawasaki disease (Franco et al. 2010). Importantly, timely administration of intravenous immunoglobulin (IVIG) is essential for reducing the incidence of coronary artery lesions (CALs) (Rife and Gedalia 2020) and growth of CD4+forkhead box protein P3 (FoxP3)+ Tregs (Franco et al. 2014). Therefore, regulation of Treg function is important for the development of new therapeutic strategies for Kawasaki disease.