Müller F M, Ehrenthal W, Hafner G, Schranz D
University Children's Hospital, Mainz, Germany.
Eur J Pediatr. 1996 Jan;155(1):20-5. doi: 10.1007/BF02115621.
This report describes the successful use of protein C concentrate to treat severe purpura fulminans in a homozygous protein C-deficient infant for 8 months until oral anticoagulation was initiated. While fresh frozen plasma was previously used in such cases to replace protein C in the acute phase, the availability of a monoclonal antibody purified protein C concentrate now allows specific replacement of protein C, avoiding problems of fluid overload. An occlusive-hydrocolloid bandage proved to be effective in local treatment of skin lesions. D-dimer, fibrin monomer, thrombin-antithrombin complex and prothrombin fragment 1 + 2 were useful markers in monitoring and optimizing protein C replacement therapy.
Diagnosis of protein C deficiency should be considered in a newborn with purpura fulminans. Early diagnosis and adequate replacement therapy is life-saving. Today, administration of protein C is the acute as well as long-term therapy of choice.
本报告描述了成功使用蛋白C浓缩物治疗一名纯合子蛋白C缺乏婴儿的严重暴发性紫癜8个月，直至开始口服抗凝治疗。虽然以前在此类病例的急性期使用新鲜冷冻血浆来替代蛋白C，但现在单克隆抗体纯化的蛋白C浓缩物的可用性允许特异性替代蛋白C，避免了液体超负荷问题。一种闭塞性水胶体绷带被证明对皮肤病变的局部治疗有效。D-二聚体、纤维蛋白单体、凝血酶-抗凝血酶复合物和凝血酶原片段1+2是监测和优化蛋白C替代疗法的有用标志物。
对于患有暴发性紫癜的新生儿，应考虑蛋白C缺乏的诊断。早期诊断和充分的替代疗法可挽救生命。如今，蛋白C给药是急性以及长期治疗的首选。
