Studies on hyperkalemic periodic paralysis. Evidence of changes in plasma Na and Cl and induction of paralysis by adrenal glucocorticoids

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Hyperkalemic Periodic Paralysis Synonym: HyperPP

Hyperkalemic periodic paralysis (hyperPP) is characterized by attacks of flaccid limb weakness (which may also include weakness of the muscles of the eyes, throat, and trunk), hyperkalemia (serum potassium concentration >5 mmol/L) or an increase of serum potassium concentration of at least 1.5 mmol/L during an attack of weakness and/or provoking/worsening of an attack by oral potassium intake, ...

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A case of hyperkalemic periodic paralysis.

た.し か し発熱,筋 ・関節痛はな く,知 覚異常も 自覚 しなかつた.筋 脱力は四肢に限局し,頭 頚部 およ び躯幹の異常はなかつた.発 病後約1週 間徐 徐に増悪 したのち快方に向い,約1ヵ 月間の通院 治療で全治した.そ の後は特別の症状なく普通に 生活 していたがが,昭 和43年(15才 時)8月 初旬, 野球大会に投手 として出場のための練習で過労がが 続いたのち,同 月12日,最 終試合を 終えて 帰宅 後,夕 食前,午 後6時 頃から両手の脱力を覚え, 翌13日 朝は両下肢 の脱力が加わ り,さ らに夕刻に は軽度の嚥下障害も認められ,14日 には腰腹筋脱 力,15日 には起坐 ・歩行全 く不能 とな り,8月17 日当科に入院 した. 入院時現症:意 識清明.起 坐 ・歩行全 く不能 で受動的背臥位であるが,心 ・肺お よび腹部内臓 には異常なく,軽 度の嚥下障害を...

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Effects of local anesthetics on Na+ channels containing the equine hyperkalemic periodic paralysis mutation.

We examined the ability of local anesthetics to correct altered inactivation properties of rat skeletal muscle Na+channels containing the equine hyperkalemic periodic paralysis (eqHPP) mutation when expressed in Xenopusoocytes. Increased time constants of current decay in eqHPP channels compared with wild-type channels were restored by 1 mM benzocaine but were not altered by lidocaine or mexile...

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Na+,K+-pump stimulation improves contractility in isolated muscles of mice with hyperkalemic periodic paralysis

In patients with hyperkalemic periodic paralysis (HyperKPP), attacks of muscle weakness or paralysis are triggered by K(+) ingestion or rest after exercise. Force can be restored by muscle work or treatment with β(2)-adrenoceptor agonists. A missense substitution corresponding to a mutation in the skeletal muscle voltage-gated Na(+) channel (Na(v)1.4, Met1592Val) causing human HyperKPP was targ...

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Hyperkalemic periodic paralysis M1592V mutation modifies activation in human skeletal muscle Na+ channel.

Mutations in the human skeletal muscle Na+ channel underlie the autosomal dominant disease hyperkalemic periodic paralysis (HPP). Muscle fibers from affected individuals exhibit sustained Na+ currents thought to depolarize the sarcolemma and thus inactivate normal Na+ channels. We expressed human wild-type or M1592V mutant α-subunits with the β1-subunit in Xenopus laevis oocytes and recorded Na...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 1971

ISSN: 0021-9738

DOI: 10.1172/jci106468