与非典型溶血尿毒综合征相关的链球菌感染后肾小球肾炎：是否用依库珠单抗治疗？
Post-streptococcal glomerulonephritis associated with atypical hemolytic uremic syndrome: to treat or not to treat with eculizumab?
作者信息
Kakajiwala Aadil, Bhatti Tricia, Kaplan Bernard S, Ruebner Rebecca L, Copelovitch Lawrence
机构信息
Division of Nephrology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Division of Pathology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
出版信息
Clin Kidney J. 2016 Feb;9(1):90-6. doi: 10.1093/ckj/sfv119. Epub 2015 Nov 26.
Abstract
A 7-year-old male with poststreptococcal glomerulonephritis (PSGN) developed hemolytic uremic syndrome (HUS) and achieved remission. He was treated with eculizumab for 1 year. The eculizumab was discontinued and the patient remained in remission. This is the 10th reported case of PSGN associated with HUS. The histopathological feature observed at the 1-year follow-up was indistinguishable from the expected findings in an individual with healed PSGN without associated HUS. The relatively good prognosis in most prior cases and the absence of any reported recurrences strongly suggest that this form of atypical HUS does not warrant long-term eculizumab therapy.
摘要
一名患有链球菌感染后肾小球肾炎（PSGN）的7岁男性发展为溶血性尿毒症综合征（HUS）并实现缓解。他接受依库珠单抗治疗1年。依库珠单抗停药后，患者仍处于缓解状态。这是第10例报道的与HUS相关的PSGN病例。1年随访时观察到的组织病理学特征与无相关HUS的已治愈PSGN个体的预期发现没有区别。大多数先前病例相对良好的预后以及没有任何复发报道强烈表明，这种非典型HUS形式无需长期依库珠单抗治疗。
相似文献
1
Post-streptococcal glomerulonephritis associated with atypical hemolytic uremic syndrome: to treat or not to treat with eculizumab?
Clin Kidney J. 2016 Feb;9(1):90-6. doi: 10.1093/ckj/sfv119. Epub 2015 Nov 26.
2
Poststreptococcal glomerulonephritis with atypical hemolytic uremic syndrome: An unusual presentation.
Saudi J Kidney Dis Transpl. 2018 May-Jun;29(3):728-731. doi: 10.4103/1319-2442.235201.
3
Case report: Short-term eculizumab use in atypical HUS associated with Lemierre's syndrome and post-infectious glomerulonephritis.
Front Med (Lausanne). 2023 May 3;10:1167806. doi: 10.3389/fmed.2023.1167806. eCollection 2023.
4
Atypical Hemolytic Uremic Syndrome: A Meta-Analysis of Case Reports Confirms the Prevalence of Genetic Mutations and the Shift of Treatment Regimens.
Ther Apher Dial. 2018 Apr;22(2):178-188. doi: 10.1111/1744-9987.12641. Epub 2017 Dec 17.
5
Hemolytic Uremic Syndrome and Kidney Transplantation: A Case Series and Review of the Literature.
Nephron. 2017;136(3):245-253. doi: 10.1159/000468528. Epub 2017 Apr 19.
6
Safety and effectiveness of eculizumab for adult patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillance.
Clin Exp Nephrol. 2019 Jan;23(1):65-75. doi: 10.1007/s10157-018-1609-8. Epub 2018 Jun 29.
7
Atypical hemolytic uremic syndrome and eculizumab therapy in children.
Korean J Pediatr. 2018 Feb;61(2):37-42. doi: 10.3345/kjp.2018.61.2.37. Epub 2018 Feb 28.
8
Safety and effectiveness of eculizumab for pediatric patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillance.
Clin Exp Nephrol. 2019 Jan;23(1):112-121. doi: 10.1007/s10157-018-1610-2. Epub 2018 Jul 23.
9
Efficacy and safety of eculizumab in adult patients with atypical hemolytic uremic syndrome: A single center experience from Turkey.
Transfus Apher Sci. 2016 Dec;55(3):357-362. doi: 10.1016/j.transci.2016.09.017. Epub 2016 Sep 30.
10
Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab.
Exp Hematol Oncol. 2017 Jan 13;6:4. doi: 10.1186/s40164-017-0064-7. eCollection 2017.
引用本文的文献
1
Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.
Nephrology (Carlton). 2025 Sep;30(9):e70116. doi: 10.1111/nep.70116.
2
Rare Histologic Coexistence of Infection-Related Glomerulonephritis and Thrombotic Microangiopathy.
Indian J Nephrol. 2025 Jan-Feb;35(1):109-110. doi: 10.25259/IJN_304_2024. Epub 2024 Aug 29.
3
Case report: Short-term eculizumab use in atypical HUS associated with Lemierre's syndrome and post-infectious glomerulonephritis.
Front Med (Lausanne). 2023 May 3;10:1167806. doi: 10.3389/fmed.2023.1167806. eCollection 2023.
4
Genetic analysis of CFH and MCP in Egyptian patients with immune-complex proliferative glomerulonephritis.
Front Immunol. 2022 Sep 23;13:960068. doi: 10.3389/fimmu.2022.960068. eCollection 2022.
5
Renal diseases and the role of complement: Linking complement to immune effector pathways and therapeutics.
Adv Immunol. 2021;152:1-81. doi: 10.1016/bs.ai.2021.09.001. Epub 2021 Nov 19.
本文引用的文献
1
Anti-complement therapy for glomerular diseases.
Adv Chronic Kidney Dis. 2014 Mar;21(2):152-8. doi: 10.1053/j.ackd.2013.12.001.
2
A time for reappraisal of "atypical" hemolytic uremic syndrome: should all patients be treated the same?
Eur J Pediatr. 2012 Oct;171(10):1519-25. doi: 10.1007/s00431-012-1763-z. Epub 2012 Jun 7.
3
Post-streptococcal acute glomerulonephritis in children: clinical features and pathogenesis.
Pediatr Nephrol. 2011 Feb;26(2):165-80. doi: 10.1007/s00467-010-1554-6. Epub 2010 Jul 23.
4
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome.
Blood. 2006 Aug 15;108(4):1267-79. doi: 10.1182/blood-2005-10-007252. Epub 2006 Apr 18.
5
An adult with acute poststreptococcal glomerulonephritis complicated by hemolytic uremic syndrome and nephrotic syndrome.
Am J Kidney Dis. 2005 Oct;46(4):e59-63. doi: 10.1053/j.ajkd.2005.06.010.
6
Studies on renal biopsy specimens, with the aid of the electron microscope. II. Glomerulonephritis and glomerulonephrosis.
Am J Clin Pathol. 1962 Sep;38:280-96. doi: 10.1093/ajcp/38.3.280.
7
NATURAL HISTORY OF ACUTE GLOMERULONEPHRITIS.
Am J Med. 1963 Aug;35:213-30. doi: 10.1016/0002-9343(63)90213-4.
8
Post-streptococcal glomerulo-nephritis: histopathologic and clinical studies of the acute, subsiding acute and early chronic latent phases.
J Clin Invest. 1961 Aug;40(8 Pt 1-2):1525-95. doi: 10.1172/JCI104382.
9
Incidental healed postinfectious glomerulonephritis: a study of 1012 renal biopsy specimens examined by electron microscopy.
Hum Pathol. 2003 Jan;34(1):3-10. doi: 10.1053/hupa.2003.53.
10
Simultaneous occurrence of the haemolytic uraemic syndrome and acute post-infectious glomerulonephritis.
Eur J Pediatr. 2001 Mar;160(3):173-6. doi: 10.1007/s004310000684.
Zotero 插件