Okura H, Gohma I, Hatta K, Imanaka T
Department of Internal Medicine, Tenri Hospital.
Intern Med. 1995 Jul;34(7):674-5. doi: 10.2169/internalmedicine.34.674.
A 57-year-old woman with Crow-Fukase syndrome presented thiamine deficiency and pulmonary hypertension of unknown etiology. After oral administration of prednisolone and thiamine, echocardiogram showed marked improvement of the pulmonary hypertension. To our knowledge, this is the first case of this syndrome associated with thiamine deficiency and precapillary pulmonary hypertension, which may play a role in the pathogenesis of polyneuropathy and heart failure of this syndrome.
一名患有克劳-深泽综合征的57岁女性出现了硫胺素缺乏和病因不明的肺动脉高压。口服泼尼松龙和硫胺素后，超声心动图显示肺动脉高压有明显改善。据我们所知，这是该综合征首例与硫胺素缺乏和毛细血管前性肺动脉高压相关的病例，这可能在该综合征的多发性神经病和心力衰竭的发病机制中起作用。
