• Internal medicine · Nov 2024

    Case Reports

    A Case of Hereditary Coproporphyria in which the Patient's Course Improved after the Discontinuation of Givosiran.

    • Nobuaki Ozaki, Yuri Hayashi, and Atsushi Kiyota.
    • Division of Endocrinology, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital, Japan.
    • Intern. Med. 2024 Nov 1; 63 (21): 296129642961-2964.

    AbstractHereditary coproporphyria (HCP) is caused by a partial deficiency of coproporphyrinogen oxidase during heme biosynthesis. Givosiran is approved for the treatment of acute hepatic porphyria. We herein report the case of a 47-year-old woman with HCP. Monthly givosiran administration improved her subjective symptoms and reduced her δ-aminolevulinic acid (ALA) and porphobilinogen (PBG) levels to the normal range. However, givosiran was discontinued after six months due to a decreased renal function. The patient's ALA and PBG levels remained within the normal ranges, and her HCP-related symptoms resolved more than 2 years after the discontinuation of givosiran.

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