Advances in the treatment of acute hepatic porphyria

Jiajia LEI; Shuang LI; Baixue DONG; Jing YANG; Yi REN

doi:10.12449/JCH240430

Volume 40 Issue 4

Apr. 2024

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Article Navigation > Journal of Clinical Hepatology > 2024 > 40(4): 828-833

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Advances in the treatment of acute hepatic porphyria

DOI: 10.12449/JCH240430

Jiajia LEI¹,
Shuang LI¹,
Baixue DONG¹,
Jing YANG²,
Yi REN^{2
,
,
,}

1.
The First Clinical Medical College of Shanxi Medical University，Taiyuan 030001，China
2.
Department of Endocrinology，The First Hospital of Shanxi Medical University，Taiyuan 030001，China

Research funding:

Key Research and Development Project of Shanxi Province (201903D321127)

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Corresponding author: REN Yi， renyi_0_0@163.com （ORCID： 0000-0002-1163-3642）
Received Date: 2023-07-07
Accepted Date: 2023-07-26
Published Date: 2024-04-25

Abstract

Abstract

Acute hepatic porphyria （AHP） is a rare disease with abnormal heme metabolism， and breakthroughs have been made in the treatment of this disease in recent years. In addition to conventional treatment methods， this article reviews new therapies for AHP that are in the stage of initial clinical application or are still in the research stage， including RNAi therapy， enzyme replacement therapy， genetic supplementation of DNA or mRNA， drug molecular chaperones， and glycine transporter inhibitors for reducing heme synthesis. Moreover， this article also reviews the treatment of AHP-related comorbidities and complications， such as hyponatremia and posterior reversible encephalopathy syndrome. High glucose infusion is the main treatment method for AHP in China， and the improvement in diagnosis and increased attention to rare diseases in China has promoted the development of the diagnosis and treatment of AHP， and it is expected to explore more suitable treatment methods for AHP in the Chinese population in the future.
- Porphyrias， Hepatic,
- Heme,
- Therapeutics

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References(45)

References

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Advances in the treatment of acute hepatic porphyria

DOI: 10.12449/JCH240430

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Advances in the treatment of acute hepatic porphyria

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