AIP

Q: Which organ transplantation can be considered in extreme cases pf Acute Intermittent porphyria (AIP)? - select one

A) Pancreas

B) Liver

Answer: B

Interestingly, hepatic transplant is considered very effective in most cases of AIP where other therapies failed and were exhausted. It should be considered if patient does not have advanced motor neuropathy like quadriplegic patients.

Indications for liver transplant are frequent hospitalizations, unresponsiveness to hemin or givosiran, and poor quality of life. It should be highly considered in patients of AIP who develop primary liver cancer or have other acute hepatic porphyrias. 

It should be noted that traditionally used model for end-stage liver disease [MELD] score for liver transplantation does not apply to patients with AIP because they usually do not have advanced parenchymal liver damage even when neuropathic manifestations are severe. Patients with AIP and severe neuropathic symptoms who are being considered for liver transplant receive exception points for expedited waitlist priority. In the case of a living-related donor, the donor should be ruled out for AIP or latent AIP. Sibling donors may have asymptomatic AIP.

#hepatology

#transplantation

References:

1. Soonawalla ZF, Orug T, Badminton MN, et al. Liver transplantation as a cure for acute intermittent porphyria. Lancet 2004; 363:705.

2. Seth AK, Badminton MN, Mirza D, et al. Liver transplantation for porphyria: who, when, and how? Liver Transpl 2007; 13:1219.

3. Lissing M, Nowak G, Adam R, et al. Liver Transplantation for Acute Intermittent Porphyria. Liver Transpl 2021; 27:491.