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藥品參考

Glycerol phenylbutyrate

Glycerol phenylbutyrate is a medicine in the class miscellaneous.

  • 藥物類別: Miscellaneous

最後更新 來源: US FDA label, EMA SmPC

僅供醫療專業人員參考。本頁為簡要摘要,並非醫療建議,亦不能取代官方仿單或臨床判斷。開立處方或調劑前請務必核實。

本頁的臨床內容僅提供英文版。

重點資訊

藥物類別
Miscellaneous
ATC 代碼
A16AX09
常見劑型
溶液
常見規格
1.1 g/ml
已知交互作用
28 (嚴重: 3 · 中度: 12 · 輕微: 13)
商品名
2
國家與市場
2

劑型、規格與商品名稱會因國家而異。請核對您手上的產品。

作用方式

RAVICTI is a triglyceride containing 3 molecules of PBA. Its active moiety PAA conjugates with glutamine in the liver and kidneys to form PAGN, which the kidneys excrete. PAGN contains 2 moles of nitrogen, like urea, and provides an alternate route for waste nitrogen excretion.

成人常用劑量(仿單摘要)

UCD, phenylbutyrate-naive patients: 4.5 to 11.2 mL/m2/day (5 to 12.4 g/m2/day) orally, given in 3 equally divided doses in patients 2 years and older; starting dose 4.5 mL/m2/day for patients with some residual enzyme activity; maximum total daily dosage 17.5 mL

僅摘錄仿單中第一行劑量說明;完整的劑量內容請見 App。

懷孕與哺乳

懷孕
Unknown Limited human data are insufficient to inform risk. Animal studies showed maternal toxicity and, at higher doses in rats, reduced fetal weights and skeletal variations.
哺乳
Unsafe No human milk data. Because of potential for serious adverse reactions in the infant, breastfeeding is not recommended.

已通報的副作用

  • Diarrhea
  • Flatulence
  • Headache
  • Abdominal pain
  • Vomiting
  • Decreased appetite

禁忌症

  • Known hypersensitivity to phenylbutyrate.

哪些人需要特別留意

  • Neurotoxicity: PAA may be toxic; reduce dosage for neurotoxicity symptoms.
  • Pancreatic insufficiency or intestinal malabsorption may reduce drug absorption and ammonia control; monitor ammonia closely.
  • Not indicated for acute hyperammonemia.

請告知藥師或醫師您是否懷孕或哺乳、是否患有腎臟或肝臟疾病、是否有過敏,以及您正在使用的所有其他藥物或保健品。

重要交互作用 (28)

All 28 interactions, with severity and what to do

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  • Dosing by age, kidney and liver function, and further dose lines
  • Renal and hepatic dose adjustments
  • Paediatric dosing
  • Dosing in older adults
  • How to give it
  • Pharmacokinetics
  • Patient counselling points
  • Food interactions
  • IV preparation and dilution

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常見問題

What is Glycerol phenylbutyrate used for?

Glycerol phenylbutyrate is a medicine in the class miscellaneous.

What side effects have been reported with Glycerol phenylbutyrate?

Diarrhea, Flatulence, Headache, Abdominal pain, Vomiting, Decreased appetite.

Can Glycerol phenylbutyrate be taken in pregnancy or while breastfeeding?

Pregnancy: Unknown. Limited human data are insufficient to inform risk. Animal studies showed maternal toxicity and, at higher doses in rats, reduced fetal weights and skeletal variations. Breastfeeding: Unsafe. No human milk data. Because of potential for serious adverse reactions in the infant, breastfeeding is not recommended.

What does Glycerol phenylbutyrate interact with?

MediHelp lists 28 known interactions for Glycerol phenylbutyrate. Major: 3 · Moderate: 12 · Minor: 13. The most serious include Dexamethasone, Sodium valproate, Valproic acid.

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