JIMD Reports (Mar 2023)

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

  • Christopher D. Ma,
  • Denise Faust,
  • Herbert L. Bonkovsky

DOI
https://doi.org/10.1002/jmd2.12354
Journal volume & issue
Vol. 64, no. 2
pp. 146 – 149

Abstract

Read online

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.