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Hereditary Coproporphyria in Which the Patient's Course Improved after the Discontinuation of Givosiran
Nobuaki Ozaki1, Yuri Hayashi1, Atsushi Kiyota1
1Division of Endocrinology, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital, Japan.
Abstract:
Hereditary 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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