目的分析长QT综合征(LQTS)和尖端扭转性室性心动过速(TdP)的发病原因、临床表现,研究其诊断方法、治疗进展,强调患者临床特点的重要性。方法回顾总结16例已确诊为长QT综合征和尖端扭转性室性心动过速的临床患者,男2例,女14例,年龄9-78岁...目的分析长QT综合征(LQTS)和尖端扭转性室性心动过速(TdP)的发病原因、临床表现,研究其诊断方法、治疗进展,强调患者临床特点的重要性。方法回顾总结16例已确诊为长QT综合征和尖端扭转性室性心动过速的临床患者,男2例,女14例,年龄9-78岁,平均年龄(39±22．7)岁,最早发病年龄为3岁。对每例患者均进行详细的临床资料及心电图资料收集。结果①一般临床情况:10例诊断为原发性LQTS,6例诊断为继发性LQTS。4例有明确家族史,4例情绪激动或活动后诱发、2例劳累后诱发、2例休息或睡眠时发作,12例有晕厥、4例有黑朦症状。②心电图表现:QT时间>0．48 s 11例, QT时间0．46-0．47 s 3例,QT时间≤0．45 s 2例。③治疗情况:10例原发性LQTS患者均接受β-阻滞剂治疗、部分接受苯妥英钠和美西律治疗,3例植入起搏器。6例继发性LQTS患者中,3例因房室传导阻滞而行起搏器植入术,其余注意治疗原发病、纠正病因(如补钾等)。所有患者经治疗后未再出现TdP复发。结论LQTS主要是由于编码心肌细胞离子通道蛋白的基因突变引起钠、钾离子通道功能障碍所致。折返和早期后除极(EAD)两种机制在TdP发病中均起作用。LQTS诊断主要依靠家族史,不明原因的晕厥和心电图QTc延长,其中更重要的是心电图和家系调查．已有很有效的治疗措施,如β-阻滞剂、LCSD、心脏起搏、ICD治疗等,如果漏诊或误诊而未接受正确治疗则可能发生致命后果。展开更多
Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists’ opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster...Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists’ opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster headache in a female presenting to an eye department with recurrent episodes of severe unilateral periorbital swelling with a chronic history of headaches. Clinical features, review of literature, and therapeutic response to indomethacin helped to establish the diagnosis as CPH. This is the first report of severe orbital inflammation with CPH to our knowledge.展开更多
文摘目的分析长QT综合征(LQTS)和尖端扭转性室性心动过速(TdP)的发病原因、临床表现,研究其诊断方法、治疗进展,强调患者临床特点的重要性。方法回顾总结16例已确诊为长QT综合征和尖端扭转性室性心动过速的临床患者,男2例,女14例,年龄9-78岁,平均年龄(39±22．7)岁,最早发病年龄为3岁。对每例患者均进行详细的临床资料及心电图资料收集。结果①一般临床情况:10例诊断为原发性LQTS,6例诊断为继发性LQTS。4例有明确家族史,4例情绪激动或活动后诱发、2例劳累后诱发、2例休息或睡眠时发作,12例有晕厥、4例有黑朦症状。②心电图表现:QT时间>0．48 s 11例, QT时间0．46-0．47 s 3例,QT时间≤0．45 s 2例。③治疗情况:10例原发性LQTS患者均接受β-阻滞剂治疗、部分接受苯妥英钠和美西律治疗,3例植入起搏器。6例继发性LQTS患者中,3例因房室传导阻滞而行起搏器植入术,其余注意治疗原发病、纠正病因(如补钾等)。所有患者经治疗后未再出现TdP复发。结论LQTS主要是由于编码心肌细胞离子通道蛋白的基因突变引起钠、钾离子通道功能障碍所致。折返和早期后除极(EAD)两种机制在TdP发病中均起作用。LQTS诊断主要依靠家族史,不明原因的晕厥和心电图QTc延长,其中更重要的是心电图和家系调查．已有很有效的治疗措施,如β-阻滞剂、LCSD、心脏起搏、ICD治疗等,如果漏诊或误诊而未接受正确治疗则可能发生致命后果。
文摘Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists’ opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster headache in a female presenting to an eye department with recurrent episodes of severe unilateral periorbital swelling with a chronic history of headaches. Clinical features, review of literature, and therapeutic response to indomethacin helped to establish the diagnosis as CPH. This is the first report of severe orbital inflammation with CPH to our knowledge.
