CELEBRATION, Fla. and ELK GROVE VILLAGE, Ill., June 18, 2024 /PRNewswire/ -- Orsini Specialty Pharmacy (Orsini), and Zevra Therapeutics, Inc. (NasdaqGS: ZVRA) (Zevra) today announced that Orsini is now the pharmacy partner for OLPRUVA® (sodium phenylbutyrate) for oral suspension.  OLPRUVA® is a prescription medicine used along with certain therapies, including changes in diet, for long-term management of certain adult and pediatric patients with urea cycle disorders (UCDs) involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC) or argininosuccinic acid synthetase (AS).

佛罗里达州庆祝会和伊利诺伊州埃尔克格罗夫村，2024年6月18日/PRNewswire/--Orsini Specialty Pharmacy（Orsini）和Zevra Therapeutics，Inc.（NasdaqGS:ZVRA）（Zevra）今天宣布Orsini现在是OLPRUVA®（苯丁酸钠）口服混悬液的药房合作伙伴。OLPRUVA®是一种处方药，与某些疗法（包括饮食变化）一起使用，用于长期治疗某些成人和儿科尿素循环障碍（UCD）患者，这些患者涉及氨基甲酰磷酸合成酶（CPS），鸟氨酸转氨甲酰酶（OTC）或精氨琥珀酸合成酶（AS）的缺乏。

Visit OLPRUVA-Prescribing-Information.pdf (olpruva.com) to view the full Prescribing Information, including Important Safety Information..

访问OLPRUVA-Prescribing-Information.pdf（OLPRUVA.com）查看完整的处方信息，包括重要的安全信息。。

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Zevra Therapeutics Transitions to Orsini as the Specialty Pharmacy Provider for OLPRUVA® (sodium phenylbutyrate)

Zevra Therapeutics转型为Orsini，成为OLPRUVA®（苯丁酸钠）的专业药房供应商

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UCDs are rare genetic disorders that impair the body's ability to remove excess ammonia. People living with UCDs often suffer from ammonia buildup in the blood, potentially resulting in brain damage and neurocognitive impairments, coma and even death, if untreated. OLPRUVA is a nitrogen scavenger that removes excess ammonia..

UCD是一种罕见的遗传性疾病，会损害身体清除过量氨的能力。患有UCD的人经常会遭受血液中氨的积累，如果不及时治疗，可能会导致大脑损伤和神经认知障碍，昏迷甚至死亡。OLPRUVA是一种氮清除剂，可以去除多余的氨。。

'Those living with UCDs can face tremendous long-term challenges including developmental delay and neurocognitive impairment, and we are committed to ensuring that they have access to OLPRUVA,' said Joshua Schafer, Zevra's Chief Commercial Officer.

Zevra首席商务官约书亚·谢弗（JoshuaSchafer）说：“患有UCD的人可能面临巨大的长期挑战，包括发育迟缓和神经认知障碍，我们致力于确保他们能够接触OLPRUVA。”。

'Orsini is built to serve rare disease communities, and we're excited to partner with Zevra to connect UCD patients with this important treatment option,' said Brandon Tom, Orsini's Chief Executive Officer.About Urea Cycle DisordersUCDs are a group of rare, genetic disorders that can cause harmful ammonia to build up in the blood, potentially resulting in brain damage and neurocognitive impairments if ammonia levels are not controlled.[i]  Any increase in ammonia over time is serious.

Orsini首席执行官布兰登·汤姆（BrandonTom）说：“Orsini旨在为罕见病社区服务，我们很高兴与Zevra合作，将UCD患者与这一重要的治疗选择联系起来。”。关于尿素循环障碍CDs是一组罕见的遗传性疾病，可导致血液中有害氨的积累，如果不控制氨水平，可能导致脑损伤和神经认知障碍。[i] 随着时间的推移，氨的任何增加都是严重的。

Therefore, it is important to adhere to any dietary protein restrictions and have alternative medication options to help control ammonia levels.About OLPRUVA®OLPRUVA (sodium phenylbutyrate) was approved for the treatment of certain UCDs in December 2022 and is marketed under the brand name, OLPRUVA®.

因此，重要的是要遵守任何饮食蛋白质限制，并有替代药物选择来帮助控制氨水平。关于OLPRUVA®OLPRUVA（苯基丁酸钠）于2022年12月被批准用于治疗某些UCD，并以OLPRUVA®品牌销售。

OLPRUVA (sodium phenylbutyrate) for oral suspension is a prescription medicine used along with certain therapies, including changes in diet, for the long-term management of adults and children weighing 44 pounds (20 kg) or greater and with a body surface area (BSA) of 1.2 m2 or greater, with UCDs, involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS).

用于口服混悬液的OLPRUVA（苯丁酸钠）是一种处方药，与某些疗法（包括饮食改变）一起使用，用于长期管理体重44磅（20公斤）或更重且体表面积（BSA）为1.2平方米或更大的成年人和儿童，患有UCD，涉及氨基甲酰磷酸合成酶（CPS），鸟氨酸转氨甲酰酶（OTC）或精氨琥珀酸合成酶（AS）的缺陷。

OLPRUVA is not used to treat rapid increase of ammonia in the blood (acute hyperammonemia), which can be life-threatening and requires emergency medical treatment.   For more information, please visit www.OLPRUVA.com.Important Safety InformationCertain medicines may increase the level of ammonia in your blood or cause serious side effects when taken during treatment with OLPRUVA.

OLPRUVA不用于治疗血液中氨的快速增加（急性高氨血症），这可能危及生命，需要紧急医疗。有关更多信息，请访问www.OLPRUVA.com.Important Safety information某些药物在服用OLPRUVA期间可能会增加血液中的氨含量或引起严重的副作用。

Tell your doctor about all the medicines you or your child take, especially if you or your child take corticosteroids, valproic acid, haloperidol, and/or probenecid.OLPRUVA can cause s.

告诉你的医生你或你的孩子服用的所有药物，特别是如果你或你的孩子服用皮质类固醇、丙戊酸、氟哌啶醇和/或丙磺舒。。