Objectives: To examine the descriptive epidemiology of the patient population referred to paediatric rheumatology centres (PRCs) in Southeast Asia (SEA) and to compare the frequency of conditions encountered with other PRC populations.
Methods: A web-based Registry for Childhood Onset Paediatric Rheumatic Diseases was established in 2009 and seven PRCs in four SEA countries, where paediatric rheumatologists are available, participated in a prospective 24 month data collection (43 months for Singapore).
Results: The number of patients analysed was 4038 (788 from Malaysia, 711 from the Philippines, 1943 from Singapore and 596 from Thailand). Over 70% of patients evaluated in PRCs in Malaysia, the Philippines and Thailand had rheumatic diseases (RDs), as compared with one-half of the proportion seen in Singaporean PRCs, which was similar to the Western PRC experience. Among RDs diagnosed (n = 2602), JIA was the most common disease encountered in Malaysia (41%) and Thailand (61%) as compared with systemic vasculitides in the Philippines (37%) and Singapore (35%) among which Henoch-Schönlein purpura was the most prevalent. SLE and related diseases were more common, but idiopathic pain syndrome and abnormal immunological laboratory tests were rarer than those seen in the West. JIA subtype distributions were different among countries. Among non-RDs (n = 1436), orthopaedic and related conditions predominated (21.7-59.4%).
Conclusion: The frequencies of RDs seen by SEA PRCs were different from those in the West. Systemic vasculitides and SLE were common in addition to JIA. Paediatric rheumatologist availability and healthcare accessibility partially explain these observed discrepancies.
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http://dx.doi.org/10.1093/rheumatology/kew446 | DOI Listing |
Sci Rep
December 2024
International Collaboration On Repair Discoveries, School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada.
Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still's disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS.
View Article and Find Full Text PDFPediatr Rheumatol Online J
December 2024
Translational Genetics Research Group, La Fe Health Research Institute (IIS La Fe), Avenida Fernando Abril Martorell nº 106 Tower A, 7th Floor, Valencia, Spain.
Background: Aicardi-Goutières Syndrome is a monogenic type 1 interferonopathy with infantile onset, characterized by a variable degree of neurological damage. Approximately 7% of Aicardi-Goutières Syndrome cases are caused by pathogenic variants in the ADAR gene and are classified as Aicardi-Goutières Syndrome type 6. Here, we present a new homozygous pathogenic variant in the ADAR gene.
View Article and Find Full Text PDFPediatr Rheumatol Online J
December 2024
Section of Rheumatology, Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Canada.
Background: Primary small vessel CNS vasculitis (sv-cPACNS) is a challenging inflammatory brain disease in children. Brain biopsy is mandatory to confirm the diagnosis. This study aims to develop and validate a histological scoring tool for diagnosing small vessel CNS vasculitis.
View Article and Find Full Text PDFBMC Med Educ
December 2024
Graduate Program in Child and Adolescent Health, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Background: Effective communication with patients and their families is a fundamental skill for medical students to cultivate during their undergraduate training. However, communicating with pediatric patients presents unique challenges. This study investigated the perceptions, attitudes, and confidence levels of undergraduate medical students regarding communication skills in pediatrics.
View Article and Find Full Text PDFJ Allergy Clin Immunol Pract
December 2024
Servicio de Alergia, Hospital Clínico San Carlos, IdISSC, Madrid, Spain; Facultad de Medicina, Universidad Complutense, Madrid, Spain. Electronic address:
Background: Fish allergy affects children and adults worldwide and there are transient and persistent phenotypes.
Objective: We aimed to analyze persistence, severity and reactivity thresholds in challenge-confirmed fish allergic patients sensitized to parvalbumin.
Methods: Patients 12-65 years-old reporting immediate reactions to fish, with fish skin prick test ≥5 mm and IgE to cod and carp β-parvalbumins ≥0.
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