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婴儿获得性凝血酶原复合物缺乏所致出血综合征:93例患病婴儿的调查

A bleeding syndrome in infants due to acquired prothrombin complex deficiency: a survey of 93 affected infants.

作者信息

Bhanchet P, Tuchinda S, Hathirat P, Visudhiphan P, Bhamaraphavati N, Bukkavesa S

出版信息

Clin Pediatr (Phila). 1977 Nov;16(11):992-8. doi: 10.1177/000992287701601106.

Abstract

A bleeding syndrome due to severe prothrombin complex deficiency is reported in 93 infants. Most were breast fed (98 per cent), aged 2 weeks to 1 year and there were no serious preceding or associated diseases. Hemorrhagic diathesis, pallor and mild hepatomegaly were the major manifestations. The incidence of intracr anial bleeding was strikingly high (63 per cent) particularly with subdural and subarachnoid hemorrhage. Acute onset, short course and rapid clinical and laboratory improvement after vitamin K therapy were observed. Mortality rate was 35 per cent but has been reduced to 17 per cent since 1969. The location of bleeding, prompt diagnosis and early treatment are the major factors affecting prognosis. Severe prothrombin complex deficiency due to vitamin K deficiency accounted for the pathogenesis of bleeding. Possible causes of vitamin K deficiency were discussed but definite conclusions could not be drawn.

摘要

本文报道了93例因严重凝血酶原复合物缺乏所致的出血综合征患儿。多数患儿为母乳喂养(98%),年龄在2周龄至1岁之间,且无严重的前驱或伴发疾病。出血素质、面色苍白和轻度肝肿大是主要表现。颅内出血的发生率极高(63%),尤其是硬膜下和蛛网膜下腔出血。患儿起病急,病程短,维生素K治疗后临床和实验室指标迅速改善。死亡率为35%,但自1969年以来已降至17%。出血部位、及时诊断和早期治疗是影响预后的主要因素。维生素K缺乏导致的严重凝血酶原复合物缺乏是出血的发病机制。文中讨论了维生素K缺乏的可能原因,但未能得出明确结论。

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