Recurrent symptoms of acute intermittent porphyria after biochemical normalization with givosiran-An ongoing clinical conundrum

Christopher D Ma; Denise Faust; Herbert L Bonkovsky

doi:10.1002/jmd2.12354

Recurrent symptoms of acute intermittent porphyria after biochemical normalization with givosiran-An ongoing clinical conundrum

JIMD Rep. 2022 Dec 13;64(2):146-149. doi: 10.1002/jmd2.12354. eCollection 2023 Mar.

Authors

Christopher D Ma¹, Denise Faust¹, Herbert L Bonkovsky¹

Affiliation

¹ Department of Internal Medicine, Section on Gastroenterology and Hepatology Wake Forest University School of Medicine Winston-Salem North Carolina USA.

Abstract

A 47-year-old woman with acute intermittent porphyria (AIP) has had recurring symptoms after achieving biochemical normalization of her urinary 5-aminolevulinic acid (ALA), porphobilinogen (PBG), and total porphyrins with givosiran. She has had normal liver tests, mildly decreased renal function, and sustained normal urinary ALA, PBG, and porphyrins with no rebound in her laboratory test results throughout treatment. She continues to tolerate monthly givosiran injections with no adverse effects, but she still experiences what she believes are acute porphyric attacks every 1-2 months.

Publication types

Case Reports