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Hypokalemic Periodic Paralysis: Reports of Two Cases and Brief Review

机译:低血钾性周期性麻痹:两例报告并简述

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Hypokalemic periodic paralysis (HPP) is a rare autosomal dominant channelopathy characterized by skeletal muscle weakness or paralysis when there is a fall in potassium levels in blood. Weakness may be mild and limited to certain muscle groups or more severe causing generalized paralysis. During an attack, reflexes may be diminished or absent. Attacks may last for a few hours or persist for several days, ultimately resulting in complete recovery. Some patients may develop chronic muscle weakness later in life. Recurrent muscle weakness accompanied by hypokalemia and exclusion of other causes help establish the diagnosis. Potassium supplementation is the mainstay of treatment of acute illness. Lifestyle modification with or without pharmacotherapy in the form of carbonic anhydrase inhibitors and/ or spironolactone can prevent future attacks. Here, we present two cases of HPP, the first one had no positive family history and responded to oral potassium and spironolactone, while the second case had family history suggestive of HPP and was managed with potassium and eplerenone.
机译:低钾性周期性麻痹(HPP)是一种罕见的常染色体显性遗传性通道病,其特征是当血液中钾水平下降时,骨骼肌无力或麻痹。虚弱可能是轻度的,仅限于某些肌肉群,或更严重的则可引起全身性麻痹。在发作期间,反射可能会减弱或消失。攻击可能持续数小时或持续数天,最终导致完全恢复。一些患者可能在以后的生活中发展为慢性肌肉无力。反复出现肌肉无力并伴有低钾血症和其他原因的排除有助于建立诊断。补充钾是治疗急性疾病的主要手段。带有或不带有碳酸酐酶抑制剂和/或螺内酯的药物治疗可以改变生活方式,可以防止将来发作。在这里,我们介绍了2例HPP,第1例没有阳性家族史,对口服钾和螺内酯有反应,而第2例有家族史提示HPP,并用钾和依普利酮治疗。

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