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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Autoimmune Disorders01:29

Autoimmune Disorders

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Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune...
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Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
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Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

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Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
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Overview of Systemic Arteries01:11

Overview of Systemic Arteries

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The human body is a complex, well-organized machine, and at the heart of its operations lies the circulatory system. This network of blood vessels, which includes systemic arteries, plays a vital role in maintaining life by transporting nutrients, oxygen, and waste products to and from cells throughout the body.
Systemic circulation is the part of the cardiovascular system that carries oxygenated blood away from the heart to the body's tissues and returns deoxygenated blood back to the...
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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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Updated: Aug 19, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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系统性硬化

Elizabeth R Volkmann1, Kristofer Andréasson2, Vanessa Smith3

  • 1Department of Medicine, Division of Rheumatology, University of California, Los Angeles, CA, USA; David Geffen School of Medicine, Los Angeles, CA, USA.

Lancet (London, England)
|November 28, 2022
PubMed
概括
此摘要是机器生成的。

系统性硬化症 (硬皮症) 是一种复杂的自身免疫性疾病. 现在的进展使得人们能够更好地了解,及早诊断,并针对炎症,纤维化和血管疾病进行定制治疗.

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科学领域:

  • 关节病学
  • 免疫学
  • 内部医学

背景情况:

  • 系统性硬化症 (硬皮症) 是一种罕见的复杂自身免疫性结合组织疾病.
  • 在历史上被认为是无法治愈的,最近的进展改善了对其病因和临床表现的理解.
  • 目前的研究重点是早期检测,风险分层和综合治疗策略.

研究的目的:

  • 审查系统性硬化症的多方面的临床方面,强调早期检测器官参与.
  • 根据核心病理特征总结管理策略:炎症,纤维化和血管病变.
  • 突出系统性硬化症的未满足需求和未来的研究方向.

主要方法:

  • 对系统性硬化病原体,临床表型和治疗的当前文献的综述.
  • 对诊断和风险分层方法的分析.
  • 综合基于疾病特征的管理考虑.

主要成果:

  • 更好地了解全身性硬化病原和临床表型.
  • 扩大治疗选择,专注于个性化治疗策略.
  • 强调早期诊断和风险分层来预测器官参与和进展.

结论:

  • 早期和准确的诊断对于治疗全身性硬化症至关重要.
  • 一个整体的,量身定制的治疗方法, 考虑患者的目标和疾病的特征, 是必不可少的.
  • 仍有大量未得到满足的需求,为系统性硬化症的治疗提供了未来研究和发现的机会.