دورية أكاديمية
Annual trends in atypical haemolytic uremic syndrome management in Japan and factors influencing early diagnosis and treatment: a retrospective study.
| العنوان: | Annual trends in atypical haemolytic uremic syndrome management in Japan and factors influencing early diagnosis and treatment: a retrospective study. |
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| المؤلفون: | Tatematsu Y; Department of Nephrology, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya, 466-8550, Japan., Imaizumi T; Department of Nephrology, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya, 466-8550, Japan.; Department of Advanced Medicine, Nagoya University Hospital, Nagoya, Japan., Michihata N; Department of Health Services Research, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Kato N; Department of Nephrology, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya, 466-8550, Japan., Kumazawa R; Center for Clinical Sciences, National Center for Global Health and Medicine, Tokyo, Japan., Matsui H; Department of Clinical Epidemiology and Health Economics School of Public Health, The University of Tokyo, Tokyo, Japan., Fushimi K; Department of Health Policy and Informatics, Tokyo Medical and Dental University Graduate School, Tokyo, Japan., Yasunaga H; Department of Clinical Epidemiology and Health Economics School of Public Health, The University of Tokyo, Tokyo, Japan., Maruyama S; Department of Nephrology, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya, 466-8550, Japan. marus@med.nagoya-u.ac.jp. |
| المصدر: | Scientific reports [Sci Rep] 2024 Aug 06; Vol. 14 (1), pp. 18265. Date of Electronic Publication: 2024 Aug 06. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: London : Nature Publishing Group, copyright 2011- |
| مواضيع طبية MeSH: | Atypical Hemolytic Uremic Syndrome*/diagnosis , Atypical Hemolytic Uremic Syndrome*/therapy , Atypical Hemolytic Uremic Syndrome*/epidemiology , Early Diagnosis* , Plasma Exchange* , Antibodies, Monoclonal, Humanized*/therapeutic use, Humans ; Japan/epidemiology ; Female ; Retrospective Studies ; Male ; Adult ; Middle Aged ; Adolescent ; ADAMTS13 Protein ; Young Adult ; Aged ; Child ; Child, Preschool ; Renal Dialysis |
| مستخلص: | Atypical haemolytic uremic syndrome (aHUS) is a rare disorder characterised by complement-mediated thrombotic microangiopathy (TMA). Despite clinical guidelines, the diagnosis and treatment of aHUS in its early stages remains challenging. This study examined the annual trends in aHUS clinical practices in Japan and explored factors influencing early diagnosis and treatment. Using data from the 2011-2020 Diagnosis Procedure Combination database, 3096 cases with the HUS disease code were identified, of which 217 were confirmed as aHUS and treated with eculizumab or plasma exchange. Early initiation, defined as starting eculizumab or plasma exchange within 7 days of admission, was the focus of the study. Our study revealed no significant changes over time in the number of aHUS diagnoses, cases treated with eculizumab, or early initiation cases. Early initiation cases underwent haemodialysis earlier and had ADAMTS13 activity measured earlier, shorter hospital stays, and lower hospitalisation costs than late initiation cases. In conclusion, we found no increase in the number of newly diagnosed aHUS cases or early treatment initiation over time. Early recognition of TMA and differentiation of the causative disease are crucial for identifying potential aHUS cases, which may lead to better patient prognoses. (© 2024. The Author(s).) |
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| فهرسة مساهمة: | Keywords: A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13); Atypical haemolytic uremic syndrome (aHUS); Eculizumab; Haemolytic anaemia; Plasma exchange; Thrombocytopenia; Thrombotic microangiopathy (TMA) |
| المشرفين على المادة: | A3ULP0F556 (eculizumab) 0 (Antibodies, Monoclonal, Humanized) EC 3.4.24.87 (ADAMTS13 Protein) EC 3.4.24.87 (ADAMTS13 protein, human) |
| تواريخ الأحداث: | Date Created: 20240806 Date Completed: 20240806 Latest Revision: 20240812 |
| رمز التحديث: | 20240813 |
| مُعرف محوري في PubMed: | PMC11303750 |
| DOI: | 10.1038/s41598-024-68736-6 |
| PMID: | 39107421 |
| قاعدة البيانات: | MEDLINE |
Find this article in full text from Springer Verlag. 
Full Text Finder | تدمد: | 2045-2322 |
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| DOI: | 10.1038/s41598-024-68736-6 |