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1957年Jervell和Lange-Nielsen首次报道了4例家族性伴有先天性耳聋、持续性心电图Q-T间期延长,发作性意识丧失和猝死。1963年Romano等相继报道了类似的病例,但无先天性耳聋。前者称为听力-心脏综合征或Jervell and Lange-Nielsen氏综合征(简称J-L-N综合征)。后者称为Romano-Ward综合征(简称R-W综合征)。这些综合征除先天性耳聋的有、无和遗传形式不同等外,临床特点大致相同,统称为遗传性Q-T间期延长综合征。现将我院于1979年5~9月先后收治的2例报道如下:
In 1957, Jervell and Lange-Nielsen first reported 4 cases of congenital deafness in familial companion, prolongation of Q-T of persistent electrocardiogram, loss of episodic consciousness and sudden death. 1963 Romano, etc. have reported a similar case, but no congenital deafness. The former is called hearing-heart syndrome or Jervell and Lange-Nielsen’s syndrome (J-L-N syndrome). The latter is called Romano-Ward syndrome (R-W syndrome for short). In addition to these syndromes of congenital deafness, no and genetic forms of different, the clinical features are roughly the same, collectively referred to as hereditary Q-T prolongation syndrome. Now our hospital in 1979 5 to September has been treated 2 cases reported as follows: