What is Acute Intermittent Porphyria ?

Acute Intermittent Porphyria (AIP) is the most frequent occurring porphyria among the acute and cutaneous porphyrias, based on Anderson, Sassa, Bishop and Desnick’s (2001) research. Hence, this blog will focus on AIP rather than other porphyrias. AIP is a disorder which is caused by the deficit in the number of enzyme known as porphobilinogen deaminase or hydroxymethylbilane synthase (American Porphyria Foundation, 2015). This enzyme can result in the buildup of porphyrin precursors in human body. Its inheritance is autosomal dominant. However, insufficiency of hydroxymethylbilane synthase cannot trigger AIP by itself, the presence of other risk factors are also essential (American Porphyria Foundation, 2015). These leading factors may take account of smoking, medications and dietary changes, but some roots of triggering the disorder are unknown. Both males and females have equivalent probability of being affected, but females have higher chance to experience acute attack as compared to males (British Porphyria Association, 2016). However, the accurate number of people who have AIP is not identified, but American Porphyria Foundation (2015) predicts that about 5.9 from one million people suffer from the disorder. AIP does not occur in any specific country or ethnic, it happens in all ethnics and race. The most common age of onset for AIP is from 20 to 40 years old (Elder, Harper, Badminton, Sandberg, & Deybach, 2013). According to Ramanujam and Anderson (2015), the acute attacks can occur in children before puberty, but generally it will only start between the ages of 20 to 40.

Figure 6. Introduction on Acute Intermittent Porphyria in point-form