Diagnosis and management of lymphatic vascular disease

Diagnosis and management of lymphatic vascular disease
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DOI:
10.1016/j.jacc.2008.06.005
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发表时间:
2008-09-02
影响因子:
24
通讯作者:
Rockson, Stanley G.
Rockson, Stanley G.
中科院分区:
医学1区
文献类型:
--
作者:
Rockson, Stanley G.

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淋巴管系统由一个血管网络组成,该网络对液体动态平衡和调节局部免疫反应都是必不可少的。在健康情况下,淋巴管系统具有必要的运输能力,以适应施加在其上的液体负荷。淋巴管功能不全最容易辨认的特征是组织肿胀,称为淋巴水肿,这是由于淋巴运输不足引起的。淋巴管病的诊断在很大程度上依赖于体检。如果诊断仍有疑问,可以通过成像,包括间接放射性核素淋巴核素扫描来确定淋巴管功能不全的存在。除了淋巴核素扫描,临床上相关的成像手段包括磁共振成像和计算机轴位断层扫描。淋巴水肿的最先进的治疗方法依赖于物理治疗技术。复杂的减充血理疗是一种经验性的、有效的、多组分的技术,旨在减少肢体体积并保持皮肤和支持结构的健康。值得注意的是,淋巴管功能不全状态的管理策略中缺少药物治疗的应用。总的来说,基于药物的方法充其量也是有争议的。通过血管再吻合改善淋巴流动的手术方法在很大程度上是不成功的,但受控抽脂在选定的患者中提供了持久的好处。在未来,专门设计的分子疗法可能会被设计成促进受损、功能障碍或闭塞的淋巴管系统的受控再生,以绕过或减轻导致浮肿和组织破坏的血管不足。
The lymphatic vasculature is comprised of a network of vessels that is essential both to fluid homeostasis and to the mediation of regional immune responses. In health, the lymphatic vasculature possesses the requisite transport capacity to accommodate the fluid load placed upon it. The most readily recognizable attribute of lymphatic vascular incompetence is the presence of the characteristic swelling of tissues, called lymphedema, which arises as a consequence of insufficient lymph transport. The diagnosis of lymphatic vascular disease relies heavily upon the physical examination. If the diagnosis remains in question, the presence of lymphatic vascular insufficiency can be ascertained through imaging, including indirect radionuclide lymphoscintigraphy. Beyond lymphoscintigraphy, clinically-relevant imaging modalities include magnetic resonance imaging and computerized axial tomography. The state-of-the-art therapeutic approach to lymphatic edema relies upon physiotherapeutic techniques. Complex decongestive physiotherapy is an empirically-derived, effective, multicomponent technique designed to reduce limb volume and maintain the health of the skin and supporting structures. The application of pharmacological therapies has been notably absent from the management strategies for lymphatic vascular insufficiency states. In general, drug-based approaches have been controversial at best. Surgical approaches to improve lymphatic flow through vascular reanastomosis have been, in large part, unsuccessful, but controlled liposuction affords lasting benefit in selected patients. In the future, specifically engineered molecular therapeutics may be designed to facilitate the controlled regrowth of damaged, dysfunctional, or obliterated lymphatic vasculature in order to circumvent or mitigate the vascular insufficiency that leads to edema and tissue destruction.