Abstract
Acute intermittent porphyria (AIP) is a rare, frequently misdiagnosed disease characterized by abdominal pain and psychiatric symptoms. Abdominal pain is the most common. At present, there is no radical cure for AIP. Hematin is considering the effective treatment to relieve the symptom. However, it is not available in china. A 25-year-old Chinese woman complained about intermittent abdominal pain for 2 years. He was misdiagnosed as acute abdominal disease many times. During attacks, abdominal pain relief always took more than 1 week without effective treatment. In our hospital, the patient was diagnosed AIP definitely and treated with intravenous cimetidine 400mg Q6h, symptoms especially abdomen pain relieved within 48-72 hours completely in each acute attack. Few cases of cimetidine to treat AIP have been reported. More clinical date is required in order to verify whether the routine treatment of cimetidine can replace hematin or be used as a preventive treatment. This is the first case to report that cimetidine has obvious effect during AIP acute attacks.
Highlights
Acute intermittent porphyria (AIP) is the most common type of porphyria which is a group of inherited or acquired metabolic diseases, attributed to defect of porphobilinogen deaminase (PBGD) in heme synthesis pathways resulting in overproduction of toxic hemin precursors such as hepatic δ-aminolevulinate (ALA) and porphobilinogen (PBG) [1]
We report the first case of a patient with recurrent AIP attacks who was treated with cimetidine successfully in China
The patient was presented to our Department of General Surgery with complain of abdominal pain accompanied by cessation of exhaustion and defecation on December 7, 2008 for the first time
Summary
Acute intermittent porphyria (AIP) is the most common type of porphyria which is a group of inherited or acquired metabolic diseases, attributed to defect of porphobilinogen deaminase (PBGD) in heme synthesis pathways resulting in overproduction of toxic hemin precursors such as hepatic δ-aminolevulinate (ALA) and porphobilinogen (PBG) [1]. There is no radical treatment for the disease and hematin is the first choice when acute AIP attacks. Cimetidine has been shown effective in the treatment of AIP.
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