患有杜氏肌力发育不良的青少年的扩散活性心肌炎 - 一份病例报告
Mohammad Mahdavi1, Shahla Meshgi2, Golnar Mortaz Hejri1
1Congenital Heart Disease Research Center, Rajaie Cardiovascular Institute, Tehran, Iran.
BMC cardiovascular disorders
|May 19, 2025
概括
杜氏肌力发育不良 (DMD) 患者可能会由于活性心肌炎而经历快速的心脏衰退. 心脏MRI可以检测到这种炎症,有助于早期诊断和管理DMD中的心脏功能障碍.
科学领域:
- 心脏病学 心脏病学
- 遗传学 遗传学 是一个
- 神经肌肉疾病 神经肌肉疾病
背景情况:
- 杜恩肌肉发育不良 (DMD) 是一种X链接遗传疾病,由于缺乏营养不良素,导致肌肉逐渐恶化.
- 心脏参与 (心肌病) 在DMD中很常见,往往默默地进展到晚期.
- 急性心脏功能下降是DMD患者罕见但严重的并发症.
研究的目的:
- 报告一个儿科DMD患者急性心脏病恶化的罕见病例.
- 为了调查左心室 (LV) 功能快速下降的原因.
- 突出心脏成像在识别活跃心肌炎的诊断实用性.
主要方法:
- 一个13岁的男孩患有DMD,胸部疼痛和心电图变化.
- 实验室检测包括托波水平和冠状动脉CT血管造影 (CTA).
- 心脏磁共振成像 (CMR) 评估心肌炎症和纤维化.
主要成果:
- 患者出现了暗示急性心脏损伤的症状.
- 热素水平升高,但CTA排除了冠状动脉疾病.
- CMR检测显示有异常纤维化模式的扩散性活性心肌炎,表明心肌炎症.
结论:
- 扩散性活性心肌炎可以在杜申心肌病症中导致LV功能快速恶化.
- 心脏磁共振成像 (CMR) 有效检测DMD患者的活跃心肌炎.
- 识别活跃心肌炎对于管理DMD心脏功能障碍至关重要.
相关概念视频
Satellite Stem Cells and Muscular Dystrophy
1.9K
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...
1.9K
Disorders of the Skeletal Muscle
849
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...
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...
849
Myasthenia Gravis: Diagnostic Tests
593
Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
593
Myasthenia Gravis: Overview and Treatment
1.1K
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
1.1K


