Streeten D H, Dalakos T G, Fellerman H
J Clin Invest. 1971 Jan;50(1):142-55. doi: 10.1172/JCI106468.
In a 19 yr old male with familial hyperkalemic periodic paralysis, paralysis was consistently induced by the administration of potassium chloride, corticotropin-gel, and a variety of glucocorticoids (dexamethasone, 6-methylprednisolone, triamcinolone) but not by mineralocorticoids (D-aldosterone, deoxycorticosterone) or by adrenocorticotropin (ACTH)-gel plus metyrapone. Induced attacks were virtually identical with spontaneous attacks, being associated, after a latent period of a few hours, with a rise in plasma K(+) and HCO(3) (-) and a simultaneous fall in plasma Na(+) and Cl(-) concentrations to an extent implying exchange of 1 K(+) with 2 Na(+) and 2 Cl(-) between extracellular and intracellular fluid. ACTH-induced paralysis was preceded by rising serum inorganic P, and associated with increased plasma glucose, blood lactate, and serum creatine phosphokinase concentrations. In normal subjects ACTH, cortisol, and triamcinolone administration failed to change plasma electrolytes or strength, while ingestion of KCl produced no weakness and smaller changes in plasma K and Na than in the patient.Since the patient and normal subjects showed the same changes in renal excretion of K after the administration of cortisol and KCl, it seems likely that paralysis in the patient resulted from abnormally slow uptake (and/or excessive loss) of K by the muscle cells, possibly caused by an abnormal "ion-exchange pump." Normal adrenocortical function and absence of a peak in plasma 11-hydroxycorticoid (11-OHCS) concentration preceding spontaneous paralysis, indicated that spontaneous paralysis did not result from changes in cortisol secretion. Similar hyperkalemic paralysis was precipitated by ACTH-gel in a brother and first cousin of the propositus. Administration of acetazolamide and fludrocortisone reduced the rise in plasma K concentration and prevented the weakness which otherwise invariably followed KCl administration to the patient. He and two close relatives have been completely protected from severe attacks of paralysis in the past 14 months by treatment with these two medications.
在一名患有家族性高钾性周期性麻痹的19岁男性患者中，氯化钾、促肾上腺皮质激素凝胶以及多种糖皮质激素（地塞米松、6-甲基泼尼松龙、曲安奈德）的给药始终会诱发麻痹，但盐皮质激素（脱氧皮质酮、醛固酮）或促肾上腺皮质激素（ACTH）凝胶加甲吡酮则不会诱发。诱发的发作与自发发作几乎相同，在数小时的潜伏期后，与血浆钾（K⁺）和碳酸氢根（HCO₃⁻）升高以及血浆钠（Na⁺）和氯（Cl⁻）浓度同时下降有关，下降程度意味着细胞外液和细胞内液之间1个K⁺与2个Na⁺和2个Cl⁻发生了交换。ACTH诱发的麻痹之前血清无机磷升高，并伴有血浆葡萄糖、血乳酸和血清肌酸磷酸激酶浓度升高。在正常受试者中，给予ACTH、皮质醇和曲安奈德未能改变血浆电解质或肌力，而摄入氯化钾不会导致无力，且血浆钾和钠的变化比患者小。由于患者和正常受试者在给予皮质醇和氯化钾后肾脏钾排泄的变化相同，因此患者的麻痹似乎是由于肌肉细胞对钾的摄取异常缓慢（和/或过度丢失）所致，可能是由异常的“离子交换泵”引起的。正常的肾上腺皮质功能以及自发麻痹之前血浆11-羟皮质类固醇（11-OHCS）浓度无峰值，表明自发麻痹并非由皮质醇分泌变化引起。先证者的兄弟和一级表亲中，ACTH凝胶也会诱发类似的高钾性麻痹。给予乙酰唑胺和氟氢可的松可降低血浆钾浓度的升高，并预防了否则该患者在给予氯化钾后总会出现的无力。在过去14个月中，他和两名近亲通过使用这两种药物治疗，完全避免了严重的麻痹发作。
