Successful Treatment with Activated Protein C Concentrates during and after Implementation of Ventriculoperitoneal Shunt in an Infant with Congenital Protein C Deficiency.

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  • 乾燥濃縮ヒト活性型プロテインＣ製剤の投与下に脳室腹腔短絡術を行った先天性プロテインＣ欠乏症

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Protein C (PC) is a precursor of a vitamin K-dependent serine protease that is activated by thrombin-thrombomodulin complex. In the absence of activated PC (APC), the inactivation of activated factors V and VIII is impaired, and the fibrinolytic capacity of the circulating blood is reduced. We report a newborn female infant who developed purpura fulminans, disseminated intravascular coagulation, and intracranial and vitreous hemorrhaging. She had undetectable levels of PC activity and antigen, and decreased levels of PC activity and antigen were observed in her parents. The patient was clinically diagnosed with complete PC deficiency and treated with APC concentrates, fresh-frozen plasma (FFP) and warfarin. During hospitalization, she underwent ophthalmic and neurosurgical operations with the use of APC concentrates. In general, the major clinical signs and symptoms of severe PC deficiency have been purpura fulminans on the extremities and scalp, and the major complications have been ophthalmic and neurological with resultant vitreous and intracranial hemorrhaging. At the onset of these signs and symptoms, the measurement of PC activity and antigen and the administration of the initial treatment are both required immediately. Although APC, FFP, warfarin, and prothrombin complex concentrate have been used successfully to treat severe PC deficiency, all have substantial drawbacks and limitations. Ultimately, other methods of treatment, such as liver transplantation or gene therapy, will need to be pursued in the future.

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