CAUSES OF DEATH IN PATIENTS WITH HUNTINGTONS-DISEASE AND IN UNAFFECTED 1ST DEGREE RELATIVES

CAUSES OF DEATH IN PATIENTS WITH HUNTINGTONS-DISEASE AND IN UNAFFECTED 1ST DEGREE RELATIVES
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DOI:
10.1136/jmg.29.12.911
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发表时间:
1992-12-01
影响因子:
4
通讯作者:
FENGER, K
FENGER, K
中科院分区:
医学1区
文献类型:
--
作者:
SORENSEN, SA;FENGER, K

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研究了395名患有亨廷顿病(HD)的丹麦受试者和282名未受影响的同胞的死亡证明中的死亡原因,并与丹麦一般人群的死亡原因进行了比较。对于HD受试者和同胞,肺炎和心血管疾病是最常见的主要死亡原因。自杀占HD受试者所有死亡的5.6%,出乎意料的是,在兄弟姐妹中占5.3%,其中一些人可能是HD基因的携带者。两者均显著高于丹麦一般人群中的相应频率2.7%,但与一般人群相比,没有证据表明HD受试者和同胞的年龄特异性比例存在差异。一些导致同胞死亡的事故可能是隐藏的自杀。HD患者的癌症发生率较低,仅为5.3%,而同胞为31.2%。与一般人群相比,HD患者中神经系统疾病的报告频率增加,分别为6%和1%。
Causes of death were examined from death certificates for 395 Danish subjects with Huntington's disease (HD) and for 282 unaffected sibs and compared with the causes of death in the general Danish population. For both the HD subjects and the sibs, pneumonia and cardiovascular diseases were the most frequent primary causes of death. Suicides accounted for 5.6% of all deaths among the HD subjects and, unexpectedly, for 5.3% among the sibs, some of whom may have been carriers of the HD gene. Both were significantly higher than the corresponding frequency of 2.7% in the general Danish population, but there was no evidence of differences in the age specific proportions for the HD subjects and for the sibs compared to the general population. Some accidents leading to death in the sibs may have been hidden suicides. The rate of cancer was low for the HD patients, being only 5.3% compared with 31.2% for the sibs. Neurological diseases were reported with an increased frequency in the HD patients compared to the general population, 6% v 1%.