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去氨加压素(DDAVP)在儿童出血性疾病中的应用。

Desmopressin (DDAVP) in bleeding disorders of childhood.

作者信息

Sutor A H

机构信息

Universitäts-Kinderklinik, Freiburg, Germany.

出版信息

Semin Thromb Hemost. 1998;24(6):555-66. doi: 10.1055/s-2007-996055.

Abstract

As in adults, desmopressin (DDAVP) can be used in children for prophylaxis of bleeding and to stop bleeding in many hereditary and acquired bleeding disorders. DDAVP is the treatment of choice in children with mild hemophilia and type 1 von Willebrand's disease (vWD). It is effective in some variants of vWD and in many patients with platelet function defects. It reduces the bleeding diathesis of children with uremia and drug-induced bleeding complications. In any case, a test dose of DDAVP has to be given to the patient to predict the hemostatic effect before relying on this drug for treatment. The response can be measured by shortening of the bleeding time (BT) and of partial thromboplastin time (PTT), indicating a rise of Factor (F) VIII or von Willebrand factor (vWF). Side effects such as facial flushing, transient headache, increased pulse rate, and drop in systolic blood pressure are mild and transient. They can be minimized when the dose is not exceeding 0.3 microg/kg body weight, and the infusion lasts at least 20 to 30 minutes. The strong antidiuretic action of DDAVP has some potential problems that are negligible in adults and older children when water intake is restricted. In infants and small children under the age of 18 months, however, DDAVP should be used with caution and with close surveillance in order to prevent water intoxication and electrolyte imbalance. The danger is increased when the patients receive parenteral fluid substitution. The advantages of DDAVP include the reduction in the use of plasma factor concentrates, thereby minimizing the danger of immunological or infectious complications, as well as the considerable reduction of costs realized by treatment with this form of medication. Fortunately, it can be applied successfully in the most frequent hereditary bleeding disorder, namely vWD type 1.

摘要

与成人一样,去氨加压素(DDAVP)可用于儿童预防出血,并在许多遗传性和获得性出血性疾病中止血。DDAVP是轻度血友病和1型血管性血友病(vWD)患儿的首选治疗药物。它对某些vWD变异型和许多血小板功能缺陷患者有效。它可减轻尿毒症患儿的出血素质以及药物引起的出血并发症。无论如何,在依靠这种药物进行治疗之前,必须给患者注射试验剂量的DDAVP以预测止血效果。可通过出血时间(BT)和部分凝血活酶时间(PTT)的缩短来衡量反应,这表明因子(F)VIII或血管性血友病因子(vWF)升高。诸如面部潮红、短暂头痛、脉搏率增加和收缩压下降等副作用轻微且短暂。当剂量不超过0.3微克/千克体重且输注持续至少20至30分钟时,这些副作用可降至最低。DDAVP的强大抗利尿作用存在一些潜在问题,在限制水摄入时,这些问题在成人和大龄儿童中可忽略不计。然而,在18个月以下的婴儿和幼儿中,使用DDAVP时应谨慎并密切监测,以防止水中毒和电解质失衡。当患者接受胃肠外液体替代时,这种风险会增加。DDAVP的优点包括减少血浆因子浓缩物的使用,从而将免疫或感染并发症的风险降至最低,以及通过这种药物治疗可大幅降低成本。幸运的是,它可成功应用于最常见的遗传性出血性疾病,即1型vWD。

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