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Page 1
Molecules Great and Small: The Complement System.
Mathern DR, Heeger PS. Mathern DR, et al. Clin J Am Soc Nephrol. 2015 Sep 4;10(9):1636-50. doi: 10.2215/CJN.06230614. Epub 2015 Jan 7. Clin J Am Soc Nephrol. 2015. PMID: 25568220 Free PMC article. Review.
Complement components produced by the liver and circulating in the plasma undergo activation through the classical and/or mannose-binding lectin pathways to mediate anti-HLA antibody-initiated kidney transplant rejection and autoantibody-initiated GN, the latter including membran …
Complement components produced by the liver and circulating in the plasma undergo activation through the classical and/or mannose-binding le …
C3 glomerulopathy and atypical hemolytic uremic syndrome: an updated review of the literature on alternative complement pathway disorders.
Turkmen K, Baloglu I, Ozer H. Turkmen K, et al. Int Urol Nephrol. 2021 Oct;53(10):2067-2080. doi: 10.1007/s11255-020-02729-y. Epub 2021 Jan 3. Int Urol Nephrol. 2021. PMID: 33389509 Review.
The complement system plays a significant role within the pathological process of C3 glomerulopathy (C3GP) and atypical hemolytic uremic syndrome (aHUS). In daily practice, clinicians should differentiate the subgroups of C3GP because of they sh …
The complement system plays a significant role within the pathological process of C3 glomerulopathy (C3GP) and atypical hem
Inherited Kidney Complement Diseases.
Lemaire M, Noone D, Lapeyraque AL, Licht C, Frémeaux-Bacchi V. Lemaire M, et al. Clin J Am Soc Nephrol. 2021 Jun;16(6):942-956. doi: 10.2215/CJN.11830720. Epub 2021 Feb 3. Clin J Am Soc Nephrol. 2021. PMID: 33536243 Free PMC article. Review.
Staggering progress was realized toward a better understanding of the genetic underpinnings and pathophysiology of many forms of atypical hemolytic uremic syndrome (aHUS) and C3-dominant glomerulopathies that are driven by complement system a
Staggering progress was realized toward a better understanding of the genetic underpinnings and pathophysiology of many forms of atypical
Atypical hemolytic uremic syndrome.
Loirat C, Frémeaux-Bacchi V. Loirat C, et al. Orphanet J Rare Dis. 2011 Sep 8;6:60. doi: 10.1186/1750-1172-6-60. Orphanet J Rare Dis. 2011. PMID: 21902819 Free PMC article. Review.
Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. ...However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from t
Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and ren
Evolving complexity of complement-related diseases: C3 glomerulopathy and atypical haemolytic uremic syndrome.
Cook HT. Cook HT. Curr Opin Nephrol Hypertens. 2018 May;27(3):165-170. doi: 10.1097/MNH.0000000000000412. Curr Opin Nephrol Hypertens. 2018. PMID: 29517501 Free article. Review.
PURPOSE OF REVIEW: The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS). RECENT FINDINGS: C3 glomerulopathy and aHUS are associated with abno
PURPOSE OF REVIEW: The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and …
Retinal disease in the C3 glomerulopathies and the risk of impaired vision.
Savige J, Amos L, Ierino F, Mack HG, Symons RC, Hughes P, Nicholls K, Colville D. Savige J, et al. Ophthalmic Genet. 2016 Dec;37(4):369-376. doi: 10.3109/13816810.2015.1101777. Epub 2016 Feb 25. Ophthalmic Genet. 2016. PMID: 26915021 Review.
BACKGROUND: Dense deposit disease and atypical hemolytic uremic syndrome are often caused by Complement Factor H (CFH) mutations. This study describes the retinal abnormalities in dense deposit disease and, for the first time, atypical ha …
BACKGROUND: Dense deposit disease and atypical hemolytic uremic syndrome are often caused by Complement Factor H …
Diagnostic Challenges and Emerging Pathogeneses of Selected Glomerulopathies.
Andeen NK, Hou J. Andeen NK, et al. Pediatr Dev Pathol. 2024 Sep-Oct;27(5):387-410. doi: 10.1177/10935266241237656. Epub 2024 Apr 5. Pediatr Dev Pathol. 2024. PMID: 38576387 Review.
Finally, progress in understanding the roles of complement in pediatric glomerular disease is reviewed, with specific attention to overlapping clinical, histologic, and genetic or functional alternative complement pathway (AP) abnormalities among C3 glomerulopathy ( …
Finally, progress in understanding the roles of complement in pediatric glomerular disease is reviewed, with specific attention to overlappi …
Treatment of C3 glomerulopathy with complement blockers.
Vivarelli M, Emma F. Vivarelli M, et al. Semin Thromb Hemost. 2014 Jun;40(4):472-7. doi: 10.1055/s-0034-1375299. Epub 2014 May 5. Semin Thromb Hemost. 2014. PMID: 24799307 Review.
C3 glomerulopathy (C3G) is a newly defined clinical entity comprising glomerular lesions with predominant C3 staining. ...Recent advances have expanded our understanding of the underlying mechanisms, leading to the hypothesis that blocking the alternative complement
C3 glomerulopathy (C3G) is a newly defined clinical entity comprising glomerular lesions with predominant C3 staining. ...Rece
Complement factor H related proteins (CFHRs).
Skerka C, Chen Q, Fremeaux-Bacchi V, Roumenina LT. Skerka C, et al. Mol Immunol. 2013 Dec 15;56(3):170-80. doi: 10.1016/j.molimm.2013.06.001. Epub 2013 Jul 3. Mol Immunol. 2013. PMID: 23830046 Free article. Review.
Mutations, genetic deletions, duplications or rearrangements in the individual CFHR genes are associated with a number of diseases including atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathies (C3 glomerulonephritis (C3GN), den …
Mutations, genetic deletions, duplications or rearrangements in the individual CFHR genes are associated with a number of diseases including …
[Atypical HUS caused by complement-related abnormalities].
Yoshida Y, Matsumoto M. Yoshida Y, et al. Rinsho Ketsueki. 2015 Feb;56(2):185-93. doi: 10.11406/rinketsu.56.185. Rinsho Ketsueki. 2015. PMID: 25765799 Review. Japanese.
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. ...Many aHUS cases (approximately 70%) are reportedly caused by uncontrolled
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by the triad of microangiopathic he
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