| アイテムタイプ |
itemtype_ver1(1) |
| 公開日 |
2024-09-09 |
| タイトル |
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|
タイトル |
Atypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities |
|
言語 |
en |
| 著者 |
Sato, Yuka
Kato, Noritoshi
Asano, Marina
Shimizu, Hideaki
Tatematsu, Yoshitaka
Shimamura, Yuko
Horinouchi, Asuka
Maeda, Kayaho
Kato, Sawako
Kosugi, Tomoki
Maruyama, Shoichi
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| アクセス権 |
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|
アクセス権 |
open access |
|
アクセス権URI |
http://purl.org/coar/access_right/c_abf2 |
| 権利 |
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|
権利情報Resource |
http://creativecommons.org/licenses/by-nc-nd/4.0/ |
|
権利情報 |
Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International |
|
言語 |
en |
| キーワード |
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|
主題Scheme |
Other |
|
主題 |
atypical hemolytic uremic syndrome |
| キーワード |
|
|
主題Scheme |
Other |
|
主題 |
eculizumab |
| キーワード |
|
|
主題Scheme |
Other |
|
主題 |
anti-C5 antibody |
| キーワード |
|
|
主題Scheme |
Other |
|
主題 |
thrombotic microangiopathy |
| キーワード |
|
|
主題Scheme |
Other |
|
主題 |
complement |
| 内容記述 |
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|
内容記述タイプ |
Abstract |
|
内容記述 |
Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease often complicated by end-stage renal disease. Anti-C5 antibody agents have been developed for the treatment of aHUS: these are highly effective but limited in use owing to the difficulty of diagnosing aHUS in the acute clinical phase. The pathophysiology of aHUS is a thrombotic microangiopathy (TMA) caused by complement dysregulation triggered by environmental factors in susceptible individuals with genetic factors. Although several germline variants associated with aHUS have been identified, approximately half of patients with aHUS lack known pathogenic variants. It is essential to recognize the characteristic clinical features of aHUS. These include the triad of hemolytic anemia, thrombocytopenia, and renal impairment, without the presence of Shiga toxin-producing Escherichia coli infection, thrombotic thrombocytopenic purpura associated with ADAMTS13 deficiency, or TMA from secondary cause. In this case, plasma exchange could not be continued owing to allergy. Early diagnosis allowed for prompt administration of eculizumab at the time of relapse, with favorable outcomes. Based on the finding of no genetic abnormalities, eculizumab was discontinued after 12 months, with no recurrence for 3 years. On day 27 of hospitalization, renal biopsy revealed endothelial damage. Since a definitive diagnosis cannot be made with genetic testing in the acute stage and approximately half of patients have no genetic abnormalities, it is suggested to diagnose the condition as per the clinical definition and commence treatment with plasma exchange. If thrombotic thrombocytopenic purpura is excluded, switching to eculizumab is another treatment option according to clinical conditions. |
|
言語 |
en |
| 出版者 |
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出版者 |
Nagoya University Graduate School of Medicine, School of Medicine |
|
言語 |
en |
| 言語 |
|
|
言語 |
eng |
| 資源タイプ |
|
|
資源タイプresource |
http://purl.org/coar/resource_type/c_6501 |
|
タイプ |
departmental bulletin paper |
| 出版タイプ |
|
|
出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| ID登録 |
|
|
ID登録 |
10.18999/nagjms.87.3.573 |
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ID登録タイプ |
JaLC |
| 関連情報 |
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|
関連タイプ |
isVersionOf |
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|
識別子タイプ |
URI |
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|
関連識別子 |
https://www.med.nagoya-u.ac.jp/medlib/nagoya_j_med_sci/873.html |
| 収録物識別子 |
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|
収録物識別子タイプ |
PISSN |
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収録物識別子 |
0027-7622 |
| 収録物識別子 |
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|
収録物識別子タイプ |
EISSN |
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収録物識別子 |
2186-3326 |
| 書誌情報 |
en : Nagoya Journal of Medical Science
巻 87,
号 3,
p. 573-581,
発行日 2025-08
|
17_Sato.pdf (14.8 MB)
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