Dysautonomia in the joint hypermobility syndrome

Dysautonomia in the joint hypermobility syndrome
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DOI:
10.1016/s0002-9343(03)00235-3
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发表时间:
2003-07-01
影响因子:
5.9
通讯作者:
Jacob, G
Jacob, G
中科院分区:
医学2区
文献类型:
--
作者:
Gazit, Y;Nahir, AM;Jacob, G

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目的:关节过度活动综合征的关节外表现可能包括周围神经系统。本研究的目的是调查该综合征患者的自主神经功能。 方法:48 名符合 1998 年布莱顿标准的关节过度活动综合征患者和 30 名健康对照受试者填写了一份临床问卷,旨在评估与自主神经系统相关的主诉频率。接下来,27 名患者和 21 名对照者接受了自主神经评估:直立性测试、心血管迷走神经和交感功能、儿茶酚胺水平和肾上腺素受体反应性。 结果:与自主神经系统相关的症状,如晕厥和先兆晕厥、心悸、胸部不适、疲劳和耐热不耐受,在患者中明显更常见。 78% (21/27) 的患者存在直立性低血压、直立性心动过速综合征和未分类的直立性不耐受,而对照组的这一比例为 10% (2/21)。与对照组相比,患有该综合征的患者在过度通气期间收缩压平均 (+/- SD) 下降幅度更大(-11 +/- 7 mm Hg 对比 -5 +/- 5 mm Hg,P = 0.02),并且冷加压试验后收缩压升高幅度更大(19 +/- 10 mm Hg 对比 11 +/- 13 mm Hg,P = 0.06)。该综合征患者还存在α-肾上腺素能(通过给予去氧肾上腺素评估)和β-肾上腺素能高反应性(通过给予异丙肾上腺素评估)的证据。结论:患者的自主神经系统相关症状具有病理生理学基础,这表明自主神经功能障碍是关节过度活动综合征的关节外表现。 (C) 2003 年,Excerpta Medica Inc.
PURPOSE: Extraarticular manifestations of the joint hypermobility syndrome may include the peripheral nervous system. The purpose of this study was to investigate autonomic function in patients with this syndrome.METHODS: Forty-eight patients with the joint hypermobility syndrome who fulfilled the 1998 Brighton criteria and 30 healthy control subjects answered a clinical questionnaire designed to evaluate the frequency of complaints related to the autonomic nervous system. Next, 27 patients and 21 controls underwent autonomic evaluation: orthostatic testing, cardiovascular vagal and sympathetic functions, catecholamine levels, and adrenoreceptor responsiveness.RESULTS: Symptoms related to the autonomic nervous system, such as syncope and presyncope, palpitations, chest discomfort, fatigue, and heat intolerance, were significantly more common among patients. Orthostatic hypotension, postural orthostatic tachycardia syndrome, and uncategorized orthostatic intolerance were found in 78% (21/27) of patients compared with in 10% (2/21) of controls. Patients with the syndrome had a greater mean (+/- SD) drop in systolic blood pressure during hyperventilation than did controls (-11 +/- 7 mm Hg vs. -5 +/- 5 mm Hg, P = 0.02) and a greater increase in systolic blood pressure after a cold pressor test (19 +/- 10 mm Hgvs. 11 +/- 13 mm Hg, P = 0.06). patients with the syndrome also had evidence of alpha-adrenergic (as assessed by administration of phenylephrine) and beta-adrenergic hyperresponsiveness (as assessed by administration of isoproterenol).CONCLUSION: The autonomic nervous system-related symptoms of the patients have a pathophysiological basis, which suggests that dysautonomia is an extraarticular manifestation in the joint hypermobility syndrome. (C) 2003 by Excerpta Medica Inc.