Acute Intermittent Porphyria

Common Name(s)

Acute Intermittent Porphyria

Acute intermittent porphyria (AIP) is one of the liver (hepatic) porphyrias. AIP is caused by low levels of porphobilinogen deaminase (PGBD), an enzyme also often called hydroxymethylbilane synthase. The low levels of PGBD are generally not sufficient to cause symptoms; however, activating factors such as hormones, drugs, and dietary changes may trigger symptoms. Although most individuals with AIP never develop symptoms, symptomatic individuals typically present with abdominal pain. Treatment is dependent on the symptoms.  
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Acute Intermittent Porphyria" for support, advocacy or research.

American Porphyria Foundation

The mission of the American Porphyria Foundation is to enhance awareness and education about the Porphyrias and to aid in advancing treatment of this group of disorders.

Last Updated: 2 Dec 2009

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Acute Intermittent Porphyria" for support, advocacy or research.

American Porphyria Foundation

The mission of the American Porphyria Foundation is to enhance awareness and education about the Porphyrias and to aid in advancing treatment of this group of disorders.

http://www.porphyriafoundation.com

Last Updated: 2 Dec 2009

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Acute Intermittent Porphyria" returned 179 free, full-text research articles on human participants. First 3 results:

A novel 55-basepair deletion of hydroxymethylbilane synthase gene found in a Chinese patient with acute intermittent porphyria and her family: A case report.
 

Author(s): Yi Ren, Lin-Xin Xu, Yun-Feng Liu, Chen-Yu Xiang, Fei Gao, Yan Wang, Tao Bai, Jian-Hong Yin, Yang-Lu Zhao, Jing Yang

Journal: Medicine (Baltimore). 2018 Sep;97(37):e12295.

 

Acute intermittent porphyria (AIP) is caused by hydroxymethylbilane synthase (HMBS) gene mutation.

Last Updated: 31 Dec 1969

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Acute Intermittent Porphyria in the North of China: The Acute Attack Effect on Quality of Life and Psychological Condition.
 

Author(s): Jing Yang, Tienan Zhu, Yongqiang Zhao, Xuezhong Yu, Huadong Zhu, Yinan Jiang, Xiaoqing Li

Journal:

 

Acute intermittent porphyria (AIP) is an autosomal recessive disorder with intermittent attacks. Patients with AIP are susceptible to impaired quality of life and psychological distress.

Last Updated: 31 Dec 1969

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Acute Intermittent Porphyria Presenting with Posterior Reversible Encephalopathy Syndrome, Accompanied by Prolonged Vasoconstriction.
 

Author(s): Tadayuki Takata, Kodai Kume, Yohei Kokudo, Kazuyo Ikeda, Masaki Kamada, Tetsuo Touge, Kazushi Deguchi, Tsutomu Masaki

Journal: Intern. Med.. 2017 ;56(6):713-717.

 

A 20-year-old Japanese woman had an attack of acute intermittent porphyria (AIP). Magnetic resonance imaging (MRI) revealed symmetrical lesions in the cerebrum and cerebellar hemisphere, corresponding to posterior reversible encephalopathy syndrome (PRES). Our administration of heme ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Acute Intermittent Porphyria" returned 6 free, full-text review articles on human participants. First 3 results:

Acute intermittent porphyria presenting with seizures and posterior reversible encephalopathy syndrome: Two case reports and a literature review.
 

Author(s): Xueping Zheng, Xuejun Liu, Yan Wang, Renliang Zhao, Lintao Qu, Haitao Pei, Miao Tuo, Yong Zhang, Yuqiang Song, Xiaojun Ji, Hongyun Li, Liou Tang, Xinbao Yin

Journal: Medicine (Baltimore). 2018 Sep;97(36):e11665.

 

Acute intermittent porphyria (AIP) is a rare and challenging hereditary neurovisceral disease with no specific symptoms. Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome with bilateral reversible posterior gyriform lesions that can be associated ...

Last Updated: 31 Dec 1969

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Acute Intermittent Porphyria in children: A case report and review of the literature.
 

Author(s): Manisha Balwani, Preeti Singh, Anju Seth, Ekta Malik Debnath, Hetanshi Naik, Dana Doheny, Brenden Chen, Makiko Yasuda, Robert J Desnick

Journal: Mol. Genet. Metab.. 2016 12;119(4):295-299.

 

Acute Intermittent Porphyria (AIP), an autosomal dominant inborn error of heme metabolism, typically presents in adulthood, most often in women in the reproductive age group. There are limited reports on the clinical presentation in children, and in contrast to the adults, most of ...

Last Updated: 31 Dec 1969

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[Acute intermittent porphyria presenting as spontaneous hemothorax].
 

Author(s): Juliana Buitrago, Sandra Viviana Santa

Journal: Biomedica. 2009 Sep;29(3):339-47.

 

The porphyrias are inherited disorders of the heme biosynthetic pathway. They are relatively rare and often misdiagnosed; however, acute episodes can be curtailed by early administration of heme arginate. Acute intermittent porphyria is the commonest of acute forms of porphyria. Here, ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Dental Health, Diet, Inflammation and Biomarkers in Patients With Acute Intermittent Porphyria(AIP)
 

Status: Not yet recruiting

Condition Summary: Acute Intermittent Porphyria

 

Last Updated: 8 Jun 2012

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Clinical Diagnosis of Acute Porphyria
 

Status: Recruiting

Condition Summary: Hereditary Coproporphyria (HCP); Acute Intermittent Porphyria (AIP); Variegate Porphyria (VP)

 

Last Updated: 4 May 2018

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Controlled Trial of Panhematin in Treatment of Acute Attacks of Porphyria
 

Status: Recruiting

Condition Summary: Acute Porphyrias

 

Last Updated: 25 Jul 2018

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