治疗小儿急性间歇性卟啉症的吉沃西兰。

IF 0.9 4区 医学 Q4 HEMATOLOGY Journal of Pediatric Hematology/Oncology Pub Date : 2024-10-01 Epub Date: 2024-08-22 DOI:10.1097/MPH.0000000000002941
Kenneth E Bujold, Nicole Kasher, Christine McKiernan
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引用次数: 0

摘要

急性间歇性卟啉症(AIP)会引起神经内脏症状和器官毒性,导致急性和慢性健康问题。传统的治疗方法包括避免诱发因素、使用碳水化合物和海明输液治疗急性发作。Givosiran 是一种经美国食品及药物管理局批准的小干扰 RNA,已在成人中显示出减少发作的效果。然而,它在儿科的应用却极为有限。我们介绍了一名患有AIP的儿科患者,她需要频繁输注hemin来治疗严重的发作,在开始使用吉沃西兰治疗后,她的疾病状态和症状得到了缓解。
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Givosiran for the Treatment of Pediatric Acute Intermittent Porphyria.

Acute intermittent porphyria (AIP) causes neurovisceral symptoms and organ toxicity resulting in acute and chronic health conditions. Treatment has traditionally involved avoiding triggers and utilizing carbohydrates and hemin infusions for acute attacks. Givosiran, an FDA-approved small interfering RNA, has shown benefit in adults in reducing attacks. However, its usage in pediatrics is extremely limited. We present a pediatric patient with AIP, requiring frequent hemin infusions for severe attacks, which have a resolution of her disease state and symptoms with the initiation of givosiran therapy.

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来源期刊
CiteScore
1.90
自引率
8.30%
发文量
415
审稿时长
2.5 months
期刊介绍: ​Journal of Pediatric Hematology/Oncology (JPHO) reports on major advances in the diagnosis and treatment of cancer and blood diseases in children. The journal publishes original research, commentaries, historical insights, and clinical and laboratory observations.
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