Chessells J M, Wigglesworth J S
Arch Dis Child. 1970 Aug;45(242):539-43. doi: 10.1136/adc.45.242.539.
Clinical, laboratory, and necropsy findings are presented in 5 newborn infants who died with a haemorrhagic diathesis developing within 24 hours after birth. 4 babies were born prematurely; 3 suffered birth asphyxia; and 3 became hypothermic. Laboratory studies showed fibrin degradation products, low platelet counts, and deficient coagulation factors. At necropsy extensive subarachnoid haemorrhages of a characteristic type were seen in all cases, and intravascular fibrin deposits in all but one. Haemorrhage in these babies is considered to be due to consumption of platelets and coagulation factors as a consequence of disseminated intravascular coagulation. Predisposing conditions and possible pathogenic mechanisms are discussed. Platelet counts aid early diagnosis of this form of haemorrhagic disease. Treatment by exchange transfusion using fresh heparinized blood is suggested.
本文报告了5例新生儿的临床、实验室及尸检结果。这些新生儿在出生后24小时内出现出血性素质并死亡。4例为早产儿；3例有出生窒息；3例体温过低。实验室检查显示有纤维蛋白降解产物、血小板计数低及凝血因子缺乏。尸检发现所有病例均有特征性的广泛蛛网膜下腔出血，除1例外在所有病例中均可见血管内纤维蛋白沉积。这些婴儿的出血被认为是由于弥散性血管内凝血导致血小板和凝血因子消耗所致。文中讨论了易感因素及可能的发病机制。血小板计数有助于早期诊断这种出血性疾病。建议采用新鲜肝素化血液进行换血治疗。
