The Enigma of Severe Factor XI Deficiency without Hemorrhagic Symptoms
The Enigma of Severe Factor XI Deficiency without Hemorrhagic Symptoms
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无出血症状的严重 XI 因子缺乏之谜
DOI：
10.1055/s-0038-1655041
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发表时间：
1967
影响因子：
6.7
通讯作者：
W. Krivit
中科院分区：
文献类型：
--
作者：
J. R. Edson;J. White;W. Krivit
总结1例非常严重的P. T。A.缺乏无出血症状的描述，和出血素质的缺乏可能有一个解释，目前未知的相互关系之间的3个“接触相”凝血因子的建议。“弗莱彻因子”缺陷作为一个实体不同于因子XI和因子XII缺陷的存在得到证实。建立肺结核诊断标准存在的问题。A.不足之处进行了讨论。作者认为，用已知先天缺陷的血浆进行测定是诊断P。T。A.缺陷先证者家庭的3名成员有轻微的肺结核。A.该家系的遗传方式与Rapaport等的假说一致。
Summary A case of very severe P. T. A. deficiency without hemorrhagic symptoms is described, and the possibility that the absence of a bleeding diathesis may have an explanation in the presently unknown interrelationship between 3 “contact phase” coagulation factors is suggested. The existence of “Fletcher factor” deficiency as an entity distinct from factor XI and XII deficiency is confirmed. The problems in establishing a diagnosis of P. T. A. deficiency are discussed. The authors believe that assay using known congenitally deficient plasma is the only certain method of diagnosing P. T. A. deficiency. The 3 members of the propositus’s family have minor P. T. A. deficiency, and the mode of inheritance in this family is consistent with the hypothesis of Rapaport et al.