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Joints form during embryonic development in conjunction with the formation and growth of the associated bones. The embryonic tissue that gives rise to all bones, cartilage, and connective tissues of the body is called mesenchyme.
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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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AssessmentA comprehensive assessment is essential in managing a patient with rheumatic heart disease (RHD). Begin with obtaining a detailed medical history, including recent streptococcal infections, a history of rheumatic fever, or previously diagnosed rheumatic heart disease. Assess the patient for symptoms such as fever, chest pain, widespread joint pain (arthralgia), tachycardia, pericardial friction rub, muffled heart sounds, heart murmurs, peripheral edema, subcutaneous nodules, and...
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Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...
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Juvenile Inflammatory Rheumatism Real-Life Clinical Practice Strategies (JIR-CliPS) network: methodology and the

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This summary is machine-generated.

The Juvenile-onset Inflammatory Rheumatisms Clinical Practice Strategies (JIR-CliPS) project gathered global physician insights on rare childhood rheumatic diseases. Findings will inform practical, locally adapted treatment strategies, improving care delivery worldwide.

Keywords:
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Area of Science:

  • Pediatric Rheumatology
  • Systemic Inflammatory Diseases
  • Clinical Practice Research

Background:

  • Juvenile-onset Inflammatory Rheumatisms (JIR) are rare childhood systemic inflammatory diseases.
  • Clinical practice guidelines exist, but real-world implementation varies significantly.
  • The JIR-Clinical Practice Strategies (CliPS) project aims to investigate and understand these variations.

Purpose of the Study:

  • To describe the rationale and methodology of the JIR-CliPS project.
  • To establish a foundation for future disease-specific publications on JIR clinical practices.
  • To investigate real-world clinical practices for JIR diseases.

Main Methods:

  • The JIR-CliPS network was established in 2020, collecting data on five JIR conditions.
  • Questionnaires targeted physician demographics, disease management approaches, and local healthcare constraints.
  • Data collection involved 534 physicians from 66 countries between June 2022 and April 2024.

Main Results:

  • Broad international physician participation was achieved, with 1,193 questionnaires completed.
  • Respondents included experienced pediatricians and rheumatology specialists from diverse settings.
  • Few countries have developed JIR-specific recommendations, indicating variable awareness and adherence.

Conclusions:

  • The JIR-CliPS network successfully engaged physicians globally.
  • A library of JIR Clinical Practice Strategies (CliPS) is being developed, tailored to local constraints.
  • This initiative bridges the gap between guidelines and practical, everyday JIR patient care.