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The vascular phase, also known as vasospasm, is the initial stage of hemostasis, crucial for preventing excessive bleeding when a blood vessel is injured. After a vessel is cut, nerves in the damaged area trigger pain and other sensory impulses. Simultaneously, the smooth muscles in the vessel wall contract, resulting in a vascular spasm. This contraction reduces the vessel's diameter at the injury site, slowing or stopping blood loss through the vessel wall. Vascular spasms typically last...
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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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The inflammatory response is the body's defense against infection, injury, or irritation from bacteria, trauma, toxins, or heat. Inflammation helps locate and destroy pathogens and remove damaged tissue elements to heal the body. During this initial phase, fluid, blood products, and nutrients migrate to the injured area, resulting in redness, heat, swelling, ache, and loss of function. Moreover, signs of systemic inflammation include fever, increased WBC count, malaise, anorexia, nausea,...
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Longitudinal In Vivo Imaging of the Cerebrovasculature: Relevance to CNS Diseases
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Childhood vasculitis.

Seza Ozen1, Erdal Sag1

  • 1Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey.

Rheumatology (Oxford, England)
|April 30, 2020
PubMed
Summary
This summary is machine-generated.

Childhood vasculitis, including IgA-associated vasculitis and Kawasaki disease, presents unique challenges. This review details current diagnosis, treatment, and management strategies for pediatric vasculitis, comparing it to adult forms.

Keywords:
IgA vasculitisKawasakiPANSAVIchildhood vasculitisdeficiency of ADA2

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Area of Science:

  • Pediatric Rheumatology and Immunology
  • Systemic Inflammatory Diseases in Children

Background:

  • Vasculitis encompasses a spectrum of inflammatory conditions affecting blood vessels, posing significant diagnostic and therapeutic challenges in pediatric populations.
  • Common childhood vasculitides like IgA-associated vasculitis (Henoch-Schönlein purpura) and Kawasaki disease, while often self-limiting, can lead to serious complications.
  • Emerging research has identified monogenic forms of vasculitis that manifest in early childhood, requiring specialized diagnostic approaches.

Purpose of the Study:

  • To provide an updated overview of vasculitis in children, encompassing diagnostic criteria, therapeutic interventions, and long-term follow-up.
  • To compare the presentation and management of childhood vasculitides with their adult counterparts.
  • To discuss the evolving understanding and clinical implications of newly defined monogenic vasculitides in pediatric patients.

Main Methods:

  • Comprehensive literature review of recent advancements in pediatric vasculitis.
  • Comparative analysis of epidemiological data and clinical features between pediatric and adult vasculitis cohorts.
  • Synthesis of current guidelines for diagnosis, treatment, and management of various childhood vasculitides.

Main Results:

  • Significant progress has been made in understanding and managing common pediatric vasculitides, leading to improved prevention of complications.
  • Distinct differences exist in the epidemiology, clinical presentation, and treatment responses between childhood and adult vasculitis.
  • Monogenic vasculitides represent a growing area of focus, often presenting with severe systemic manifestations in infancy and early childhood.

Conclusions:

  • Effective management strategies for common pediatric vasculitides are established, improving patient outcomes.
  • Awareness of both common and rare forms, including monogenic vasculitides, is crucial for timely diagnosis and appropriate care in children.
  • Continued research is essential to further elucidate the pathogenesis and optimize treatment for all forms of vasculitis in pediatric patients.