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Quoted phrase not found in phrase index: "Atypical hemolytic uremic syndrome with complement gene abnormality"
Page 1
Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype.
Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G. Noris M, et al. Clin J Am Soc Nephrol. 2010 Oct;5(10):1844-59. doi: 10.2215/CJN.02210310. Epub 2010 Jul 1. Clin J Am Soc Nephrol. 2010. PMID: 20595690 Free PMC article.
BACKGROUND AND OBJECTIVES: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. ...Genetic complement abnormalities have been found in aHUS. DESIGN, SETTING, PARTIC …
BACKGROUND AND OBJECTIVES: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia …
Hemolysis is associated with altered heparan sulfate of the endothelial glycocalyx and with local complement activation in thrombotic microangiopathies.
Laboux T, Maanaoui M, Allain F, Boulanger E, Denys A, Gibier JB, Glowacki F, Grolaux G, Grunenwald A, Howsam M, Lancel S, Lebas C, Lopez B, Roumenina L, Provôt F, Gnemmi V, Frimat M. Laboux T, et al. Kidney Int. 2023 Aug;104(2):353-366. doi: 10.1016/j.kint.2023.03.039. Epub 2023 May 9. Kidney Int. 2023. PMID: 37164260 Free article.
The complement system plays a key role in the pathophysiology of kidney thrombotic microangiopathies (TMA), as illustrated by atypical hemolytic uremic syndrome. But complement abnormalities are not the only drivers of TMA l …
The complement system plays a key role in the pathophysiology of kidney thrombotic microangiopathies (TMA), as illustrated by …
Atypical haemolytic uraemic syndrome and mutations in complement regulator genes.
Dragon-Durey MA, Frémeaux-Bacchi V. Dragon-Durey MA, et al. Springer Semin Immunopathol. 2005 Nov;27(3):359-74. doi: 10.1007/s00281-005-0003-2. Epub 2005 Nov 11. Springer Semin Immunopathol. 2005. PMID: 16189652 Review.
These three proteins are involved in the regulation of the alternative pathway of the complement system. Several series have reported mutations in the FH gene (called HF1) in between 10 and 22% of atypical HUS patients. ...
These three proteins are involved in the regulation of the alternative pathway of the complement system. Several series …
Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype.
Bresin E, Rurali E, Caprioli J, Sanchez-Corral P, Fremeaux-Bacchi V, Rodriguez de Cordoba S, Pinto S, Goodship TH, Alberti M, Ribes D, Valoti E, Remuzzi G, Noris M; European Working Party on Complement Genetics in Renal Diseases. Bresin E, et al. J Am Soc Nephrol. 2013 Feb;24(3):475-86. doi: 10.1681/ASN.2012090884. Epub 2013 Feb 21. J Am Soc Nephrol. 2013. PMID: 23431077 Free PMC article.
Several abnormalities in complement genes reportedly contribute to atypical hemolytic uremic syndrome (aHUS), but incomplete penetrance suggests that additional factors are necessary for the disease to manifest. Here, we sought to …
Several abnormalities in complement genes reportedly contribute to atypical hemolytic uremic sy …
Clinical characteristics and genetic backgrounds of Japanese patients with atypical hemolytic uremic syndrome.
Fujisawa M, Kato H, Yoshida Y, Usui T, Takata M, Fujimoto M, Wada H, Uchida Y, Kokame K, Matsumoto M, Fujimura Y, Miyata T, Nangaku M. Fujisawa M, et al. Clin Exp Nephrol. 2018 Oct;22(5):1088-1099. doi: 10.1007/s10157-018-1549-3. Epub 2018 Mar 6. Clin Exp Nephrol. 2018. PMID: 29511899 Free PMC article.
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is caused by complement overactivation, and its presentation and prognosis differ according to the underlying molecular defects. ...In addition, the favorable prognosis of patients with the u …
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is caused by complement overactivation, and its pre …
An ex vivo test to investigate genetic factors conferring susceptibility to atypical haemolytic uremic syndrome.
Gastoldi S, Aiello S, Galbusera M, Breno M, Alberti M, Bresin E, Mele C, Piras R, Liguori L, Santarsiero D, Benigni A, Remuzzi G, Noris M. Gastoldi S, et al. Front Immunol. 2023 Feb 9;14:1112257. doi: 10.3389/fimmu.2023.1112257. eCollection 2023. Front Immunol. 2023. PMID: 36845135 Free PMC article.
INTRODUCTION: Comprehensive genetic analysis is essential to clinical care of patients with atypical haemolytic uremic syndrome (aHUS) to reinforce diagnosis, and to guide treatment. However, the characterization of complement gene variants rema …
INTRODUCTION: Comprehensive genetic analysis is essential to clinical care of patients with atypical haemolytic uremic synd …
Efficacy and safety of eculizumab in childhood atypical hemolytic uremic syndrome in Japan.
Ito N, Hataya H, Saida K, Amano Y, Hidaka Y, Motoyoshi Y, Ohta T, Yoshida Y, Terano C, Iwasa T, Kubota W, Takada H, Hara T, Fujimura Y, Ito S. Ito N, et al. Clin Exp Nephrol. 2016 Apr;20(2):265-72. doi: 10.1007/s10157-015-1142-y. Epub 2015 Jul 9. Clin Exp Nephrol. 2016. PMID: 26156042
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a severe life-threatening disease with frequent progression to end-stage renal disease (ESRD). ...RESULTS: Seven patients were resistant to plasma therapy and three were dependent on it. Causativ …
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a severe life-threatening disease with frequent progres …
Thrombomodulin and Endothelial Dysfunction: A Disease-Modifier Shared between Malignant Hypertension and Atypical Hemolytic Uremic Syndrome.
Demeulenaere M, Devreese K, Vanbelleghem H, De Zaeytijd J, Vande Walle J, Van Biesen W, Van Laecke S. Demeulenaere M, et al. Nephron. 2018;140(1):63-73. doi: 10.1159/000490201. Epub 2018 Jun 25. Nephron. 2018. PMID: 29940557 Free article. Review.
Thrombomodulin (TM) is an endothelial glycoprotein that is present in all blood vessels. Five percent of all patients with atypical hemolytic uremic syndrome (aHUS) have mutations in the gene coding for TM, with a peak presentation in young chil …
Thrombomodulin (TM) is an endothelial glycoprotein that is present in all blood vessels. Five percent of all patients with atypical …
Case report: Novel FHR2 variants in atypical Hemolytic Uremic Syndrome: A case study of a translational medicine approach in renal transplantation.
Stea ED, Skerka C, Accetturo M, Pesce F, Wiech T, Hartman A, Pontrelli P, Conserva F, Castellano G, Zipfel PF, Gesualdo L. Stea ED, et al. Front Immunol. 2022 Nov 14;13:1008294. doi: 10.3389/fimmu.2022.1008294. eCollection 2022. Front Immunol. 2022. PMID: 36451836 Free PMC article.
Atypical hemolytic-uremic syndrome (aHUS) is a severe thrombotic microangiopathy in which kidney involvement is common. aHUS can be due to either genetic or acquired abnormalities, with most abnormalities affecting the alternative comp …
Atypical hemolytic-uremic syndrome (aHUS) is a severe thrombotic microangiopathy in which kidney involvement is …
An Ex Vivo Test of Complement Activation on Endothelium for Individualized Eculizumab Therapy in Hemolytic Uremic Syndrome.
Galbusera M, Noris M, Gastoldi S, Bresin E, Mele C, Breno M, Cuccarolo P, Alberti M, Valoti E, Piras R, Donadelli R, Vivarelli M, Murer L, Pecoraro C, Ferrari E, Perna A, Benigni A, Portalupi V, Remuzzi G. Galbusera M, et al. Am J Kidney Dis. 2019 Jul;74(1):56-72. doi: 10.1053/j.ajkd.2018.11.012. Epub 2019 Mar 7. Am J Kidney Dis. 2019. PMID: 30851964
RATIONALE & OBJECTIVE: Although primary atypical hemolytic uremic syndrome (aHUS) is associated with abnormalities in complement genes and antibodies to complement factor H, the role of complement in secondary aHU …
RATIONALE & OBJECTIVE: Although primary atypical hemolytic uremic syndrome (aHUS) is associated with abno …
20 results