Severe homozygous protein C deficiency.
Severe homozygous protein C deficiency.
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严重的纯合蛋白 C 缺乏症。
DOI：
10.1016/s0022-3476(84)80013-x
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发表时间：
1984
期刊：
影响因子：
--
通讯作者：
Humbert,JR
中科院分区：
文献类型：
--
作者：
Sills,RH;Marlar,RA;Montgomery,RR;Deshpande,GN;Humbert,JR
一名患有复发性暴发性紫癜的婴儿在出生后一年内被发现有严重的纯合子蛋白C缺乏症（<正常水平的1%）。通过输注含有蛋白C的新鲜冷冻血浆控制了暴发性紫癜的发作。然而，频繁血浆输注的要求最终导致了继发于高蛋白血症的几种并发症。然后给予富含蛋白质C的因子IX浓缩物，以维持足够的因子水平，同时最大限度地减少外来蛋白质的量。患者在每48小时接受这些浓缩物的同时保持无症状和无并发症> 10个月。
An infant with recurrent purpura fulminans in the first year of life was found to have severe homozygous deficiency of protein C (<1% of normal levels). The episodes of purpura fulminans were controlled by infusions of fresh frozen plasma containing protein C. The requirement of frequent plasma infusions, however, eventually resulted in several complications secondary to hyperproteinemia. Factor IX concentrates rich in protein C were then given to maintain adequate levels of the factor while minimizing the amount of extraneous proteins. The patient has remained asymptomatic and free of complications for > 10 months while receiving these concentrates every 48 hours.