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題 名 | Dominant-Inherited Hypokalemic Periodic Paralysis in a Large Chinese Family=具顯性遺傳之低鉀周期性麻痺的一個華人大家族 |
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作 者 | 葉建宏; 孫明輝; 邱浩彰; | 書刊名 | 臺灣醫學會雜誌 |
卷 期 | 98:4 1999.04[民88.04] |
頁 次 | 頁277-282 |
分類號 | 415.597 |
關鍵詞 | 顯性遺傳; 低鉀周期性麻痺; 華人; Familial hypokalemic periodic paralysis; Chinese family; Permanent muscle weakness; Thyrotoxic periodic paralysis; Early onset; |
語 文 | 英文(English) |
英文摘要 | Farnilial hypokalemic periodic paralysis (HoPP) is a rare condition among Chinese. We studied a large Chinese family (48 members in six generations) with dominant-inheritcd HoPP, using incidental tracing of family history of a proband who presented with the typical features of HoPP. Fifteen family members were found to have the disease. We found the familial type of HoPP to differ from the sporadic type in Taiwan, in that the familial type has an equal gender distribution, earlier onset of paralytic attacks, and more severe clinical features in both frequency and extent. When these patients were compared with Caucasian families, the common features were the involvement of the respiratory" and the bulbar muscles, and the eye muscles in Chinese patients. Cold-induced attacks and permanent muscle weakness were not common in Chinese subjects. Age and history of paralytic attacks were not the major determinants for the development of permanent muscle weakness. Two family members died during attacks because of severe involvement of the respiratory and bulbar muscles. |
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