기립성 못견딤증의 혈역학적 이해와 진단
Syndromes of Orthostatic Intolerance: Pathophysiology and Diagnosis
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기립성 못견딤증은 기립 자세에 의해서 악화되고 눕게 되면 증상이소실되는 여러 임상적인 상태를 포괄하는 용어로 사용된다. 기립성못견딤증의 정의 자체는 매우 단순하지만 새로운 개념의 기전 및 임상 유형들이 소개됨에 따라 명명법 또한 자주 바뀌고 있어 이를 임상에 적용하여 정확히 진단하는 것은 쉽지 않다. 기립 시 나타나는 혈액쏠림 현상을 극복하고 의식을 유지하기 위해서는 심혈관계의 해부학적 구조 및 반사 작용의 기능이 온전하고, 혈액량이 충분하며 근육펌프와 같은 물리적 기전이 제대로 작용해야 한다. 이러한 보상 기능 중어느 하나라도 불충분하거나 제대로 작용하지 않게 되면 기립성 못견딤증이 나타나게 된다. 소아청소년에서 자주 발생하는 기립성 못견딤증에는 초기 기립성 저혈압, 체위성 미주신경성 실신과 체위성 빈맥증후군이 있다. 초기 기립성 저혈압은 기립에 의한 초기 일련의 반응이 지연되어 나타나는 증후군으로 기립 직후 현기증, 어지럼증, 빈맥등의 증상이 나타나며 대부분의 경우 60초 이내에 증상이 소실되고혈압 및 맥박도 안정화된다. 체위성 미주신경성 실신은 자율신경계활동도의 갑작스러운 변화에 의해 혈압, 심박수, 뇌혈류량이 감소하여의식을 소실하는 실신으로 정의된다. 체위성 빈맥증후군은 기립에 의해 유발되는 과도한 동빈맥(sinus tachycardia)으로 정의되며 기립과관련한 어지럼증, 두통, 가슴 통증, 피곤등의 증상이 거의 매일 나타나는 만성 기립성 못견딤증으로 분류된다. 체위성 빈맥증후군은 저혈량, 카테콜아민 항진 상태(high catecholamine status), 비정상적 국소혈관 긴장도, 및 골격근의 근육 펌프 기능 저하와 같은 다양한 기전에의해 발생하므로 치료 역시 기전에 따라 개별화되어야 한다. 두통, 만성 피로 증후군, 불면증, 및 섬유근통 증후군과 같은 비특이적이고 치료가 잘 안 되는 만성 신경계 질환과 기립성 못견딤증간의 연관성이제시되고 있으므로 기립성 못견딤증에 대한 소아신경과 의사의 이해와 관심이 필요하다.
Abstract
Orthostasis means standing upright. Thus, orthostatic intolerance (OI) can be simply defined as “the development of symptoms during upright standing, that are relieved by recumbency.” However, OI might be a confusing topic in clinical practice because of the recent appreciation of the condition’s clinical variant, emerging understanding of its diverse mechanisms, and its nomenclature, which seems to change annually. OI is not fatal but should be differentiated from potentially lethal disorders, including seizures or cardiogenic syncope. Typical signs and symptoms include loss of consciousness, lightheadedness, and visual difficulties. However, patients also experience multiple and nonspecific symptoms that seem unrelated to orthostatic intolerance, such as headache, fatigue, nausea, abdominal pain, and exercise intolerance. This review was aimed at expanding the comprehension of this confusing and easily missed topic by providing better understanding of the normal hemodynamic response to orthostasis and the basic pathophysiological concepts of major syndromes of OI.
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