KERR C B
Br J Ind Med. 1961 Jan;18(1):47-52. doi: 10.1136/oem.18.1.47.
The main features of Christmas disease, a hereditary disorder of blood coagulation, are outlined, and a family of coal-miners who suffer from this disorder is described. The affected male members of two generations have spent their working lives underground. Three of the eight affected miners were temporarily severely disabled by episodes associated with their bleeding diathesis, and one died in 1894 from haemorrhage following an injury. A mild degree of Christmas disease caused an increased occupational morbidity in some members of this family, but did not shorten their overall working life-span. Twelve further cases of Christmas disease are briefly described. One affected man had an episode of cerebral haemorrhage, possibly associated with the physiological effects of contact with nitroglycerine. A review of the literature suggests that nitroglycerine may have a significant aetiological action in the development of cerebral haemorrhage in men with a defective haemostatic mechanism. Finally, it is suggested that an analogous situation may exist in patients under anticoagulant treatment, who use nitrates or other vasodilator drugs therapeutically.
本文概述了一种遗传性血液凝固障碍疾病——克里斯马斯病的主要特征,并描述了一个患有这种疾病的煤矿工人家庭。两代受影响的男性成员都在地下度过了他们的职业生涯。八名受影响的矿工中有三名因与他们的出血素质相关的发作而暂时严重残疾,其中一人于1894年因受伤后出血死亡。轻度的克里斯马斯病在这个家庭的一些成员中导致了职业发病率的增加,但并没有缩短他们的总体工作寿命。文中还简要描述了另外12例克里斯马斯病病例。一名受影响的男子发生了脑出血,可能与接触硝酸甘油的生理效应有关。对文献的回顾表明,硝酸甘油可能在具有止血机制缺陷的男性发生脑出血的过程中具有重要的病因学作用。最后,有人提出,在接受抗凝治疗并在治疗中使用硝酸盐或其他血管扩张药物的患者中可能存在类似情况。