Acute intermittent (hepatic) porphyria

This code represents a rare, inherited metabolic disorder characterized by acute neurovisceral attacks, often triggered by certain drugs, alcohol, or fasting. It results from a deficiency in hydroxymethylbilane synthase (HMBS), leading to an accumulation of porphyrin precursors in the body. Symptoms can include severe abdominal pain, psychiatric disturbances, and neurological dysfunction.

Use this code for patients diagnosed with acute intermittent porphyria (AIP) experiencing an acute attack or for chronic management of the condition. Documentation should clearly state the diagnosis of AIP, often supported by laboratory findings of elevated porphyrin precursors (e.g., ALA, PBG) during an attack. This code is appropriate for both initial diagnosis and subsequent encounters related to AIP.

• Distinguish from other types of porphyria; this code is specific to acute intermittent porphyria.
• Ensure documentation specifies "acute intermittent" to support this particular code.
• Consider sequencing this code as a primary diagnosis when the patient is admitted or seen for an acute porphyric attack.