Acute porphyria management options?

[Manami]

A 33-year-old previously healthy woman presented with abdominal pain and behavioral symptoms. She developed hyponatremia, seizures, and rapidly progressive weakness leading to respiratory failure, quadriplegia, and autonomic instability. Cerebrospinal fluid was acellular with normal protein and glucose. Initial nerve conduction studies showed prolonged F waves, and follow-up studies demonstrated absent sensory and motor responses with signs of profuse active denervation on needle examination (fibrillation potentials and reduced recruitment). Quantitative urine testing for porphyria was abnormal and in keeping with AIP. The patient had severe axonal porphyric neuropathy resulting in significant disability. What are the management optons?

[Samik]

@manami The management of patients with attacks of AIP can be challenging because the disease manifestations are diverse and potentially life-threatening. Timely intervention can resolve and prevent attacks; any other condition (eg, infection, medication exposure) that may have triggered the acute attack must also be addressed. Close long-term monitoring for complications is also needed.