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Related Concept Videos

Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

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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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Clot Retraction and Fibrinolysis01:16

Clot Retraction and Fibrinolysis

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After a fibrin clot is formed, the next step is clot retraction, a vital process facilitated by platelet contractile proteins, such as actin and myosin. These proteins pull the fibrin strands closer together and condense the clot. This action reduces the size of the clot, creating a smaller, denser structure that effectively seals off the damaged vessel. Clot retraction consolidates the clot and helps with wound healing by bringing the edges of the damaged blood vessel closer together.
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Fibril-associated Collagen01:11

Fibril-associated Collagen

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Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
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Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

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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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Rheumatic Heart Disease III: Medical Management01:21

Rheumatic Heart Disease III: Medical Management

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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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Rh Blood Group01:19

Rh Blood Group

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The Rhesus (Rh) antigen is crucial in determining blood groups and ensuring compatibility during blood transfusions.
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Related Experiment Video

Updated: Sep 29, 2025

An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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Cryofibrinogenemia: What Rheumatologists Should Know.

Mittermayer B Santiago1,2,3,4, Bartira Souza Melo1

  • 1Hospital Santa Izabel, Praça Almeida Couto 500, Salvador, Bahia, Brazil.

Current Rheumatology Reviews
|March 27, 2022
PubMed
Summary

Cryofibrinogenemia, a rare disorder causing skin lesions and thrombosis, is often underdiagnosed in rheumatology. Increased physician awareness is crucial for timely diagnosis and management of this condition.

Keywords:
Cryofibrinogenemiacryofibrinogencryoglobulinemiacryoproteinfibrinogenskin necrosis

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Generation of Two-color Antigen Microarrays for the Simultaneous Detection of IgG and IgM Autoantibodies
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Area of Science:

  • Rheumatology
  • Hematology
  • Dermatology

Background:

  • Cryofibrinogenemia is a rare condition characterized by cryofibrinogen precipitating at low temperatures.
  • Clinical manifestations include skin lesions (ulcers, necrosis, livedo reticularis), arthralgia, thrombosis, and limb ischemia.
  • It presents diagnostic challenges, often considered in the differential diagnosis of antiphospholipid syndrome, vasculitis, TTP, and cryoglobulinemia.

Purpose of the Study:

  • To increase physician awareness, particularly among rheumatologists, regarding the underdiagnosed condition of cryofibrinogenemia.
  • To highlight the clinical features and diagnostic considerations of cryofibrinogenemia.
  • To emphasize the need for further research into its prevalence and association with rheumatological disorders.

Main Methods:

  • This is a narrative review.
  • Literature search on cryofibrinogenemia, its clinical presentation, and diagnostic challenges.
  • Review of histopathological findings and current management strategies.

Main Results:

  • Cryofibrinogenemia presents with characteristic skin lesions and thrombotic events.
  • Histopathology typically shows thrombi in dermal blood vessels without vasculitis.
  • No validated classification criteria currently exist.

Conclusions:

  • Cryofibrinogenemia is an underdiagnosed condition in rheumatological practice.
  • Early recognition and diagnosis are essential for appropriate management, which may include corticosteroids, immunosuppressants, anticoagulants, and fibrinolytic agents.
  • Further epidemiological studies are needed to determine its prevalence in rheumatological disorders and its potential associations.