Unexpected Hypotension in a Female Patient with Fabry Disease: Switching from Agalsidase α to β after Long-term ERT

被引：0

作者：

Sugiura, Takuya ^{[1
]}

Muto, Reiko ^{[1
,2
]}

Amano, Tatsuaki ^{[3
]}

Kamiya, Fumitaka ^{[4
]}

Sato, Yuka ^{[1
]}

Maeda, Kayaho ^{[1
]}

Saito, Shoji ^{[1
]}

Katsuno, Takayuki ^{[5
]}

Kato, Noritoshi ^{[1
]}

Higashi, Michiko ^{[6
]}

Numaguchi, Atsushi ^{[6
]}

Matsuda, Naoyuki ^{[6
]}

Sugiura, Kazumitsu ^{[7
]}

Maruyama, Shoichi ^{[1
]}

机构：

[1] Nagoya Univ, Grad Sch Med, Dept Nephrol, Nagoya, Japan

[2] Nagoya Univ, Grad Sch Med, Dept Renal Replacement Therapy, Nagoya, Japan

[3] Handa City Hosp, Dept Nephrol, Handa, Japan

[4] Komaki City Hosp, Dept Nephrol, Komaki, Japan

[5] Aichi Med Univ, Med Ctr, Dept Nephrol & Rheumatol, Nagakute, Japan

[6] Nagoya Univ, Grad Sch Med, Dept Emergencyand Crit Care Med, Nagoya, Japan

[7] Fujita Hlth Univ, Sch Med, Dept Dermatol, Toyoake, Japan

来源：

INTERNAL MEDICINE | 2025年

关键词：

Fabry disease; Female; enzyme replacement therapy; infusion-related reactions; ENZYME REPLACEMENT THERAPY; PLASMA GLOBOTRIAOSYLSPHINGOSINE LEVELS;

D O I：

10.2169/internalmedicine.4685-24

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Fabry disease (FD) is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERT), such as agalsidase alpha and beta, are available treatment options. While infusion-related reactions (IRRs) are known to occur at the initiation of ERT owing to immune responses, there is limited information on IRRs during long-term ERT. We report the case of a female patient with Fabry disease who developed unexpected hypotension after six years of stable treatment with agalsidase alpha, leading to a switch to agalsidase beta. Continuous monitoring may be essential to identify potential IRRs in female patients with Fabry disease receiving long-term ERT.

引用

页数：6

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