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Items: 1 to 50 of 173

1.

Mitochondrial Haplogroup and the Risk of Acute Kidney Injury Following Cardiac Bypass Surgery.

Kanagasundaram NS, Baudouin SV, Rowling S, Prabhu M, Dark JH, Goodship THJ, Chinnery PF, Hudson G.

Sci Rep. 2019 Feb 19;9(1):2279. doi: 10.1038/s41598-018-37944-2.

2.

Clinical Relapses of Atypical HUS on Eculizumab: Clinical Gap for Monitoring and Individualised Therapy.

Teoh CW, Gorman KM, Lynch B, Goodship THJ, Dolan NM, Waldron M, Riordan M, Awan A.

Case Rep Nephrol. 2018 Feb 6;2018:2781789. doi: 10.1155/2018/2781789. eCollection 2018.

3.

Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy.

Osborne AJ, Breno M, Borsa NG, Bu F, Frémeaux-Bacchi V, Gale DP, van den Heuvel LP, Kavanagh D, Noris M, Pinto S, Rallapalli PM, Remuzzi G, Rodríguez de Cordoba S, Ruiz A, Smith RJH, Vieira-Martins P, Volokhina E, Wilson V, Goodship THJ, Perkins SJ.

J Immunol. 2018 Apr 1;200(7):2464-2478. doi: 10.4049/jimmunol.1701695. Epub 2018 Mar 2.

4.

Publisher Correction: Genome-wide linkage and association study implicates the 10q26 region as a major genetic contributor to primary nonsyndromic vesicoureteric reflux.

Darlow JM, Darlay R, Dobson MG, Stewart A, Charoen P, Southgate J, Baker SC, Xu Y, Hunziker M, Lambert HJ, Green AJ, Santibanez-Koref M, Sayer JA, Goodship THJ, Puri P, Woolf AS, Kenda RB, Barton DE, Cordell HJ.

Sci Rep. 2018 Jan 8;8(1):459. doi: 10.1038/s41598-017-17992-w.

5.

Use of the complement inhibitor Coversin to treat HSCT-associated TMA.

Goodship THJ, Pinto F, Weston-Davies WH, Silva J, Nishimura JI, Nunn MA, Mackie I, Machin SJ, Palm L, Pryce JW, Chiesa R, Amrolia P, Veys P.

Blood Adv. 2017 Jul 3;1(16):1254-1258. doi: 10.1182/bloodadvances.2016002832. eCollection 2017 Jul 11.

6.

Genome-wide linkage and association study implicates the 10q26 region as a major genetic contributor to primary nonsyndromic vesicoureteric reflux.

Darlow JM, Darlay R, Dobson MG, Stewart A, Charoen P, Southgate J, Baker SC, Xu Y, Hunziker M, Lambert HJ, Green AJ, Santibanez-Koref M, Sayer JA, Goodship THJ, Puri P, Woolf AS, Kenda RB, Barton DE, Cordell HJ.

Sci Rep. 2017 Nov 6;7(1):14595. doi: 10.1038/s41598-017-15062-9. Erratum in: Sci Rep. 2018 Jan 8;8(1):459.

7.

Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland.

Brocklebank V, Johnson S, Sheerin TP, Marks SD, Gilbert RD, Tyerman K, Kinoshita M, Awan A, Kaur A, Webb N, Hegde S, Finlay E, Fitzpatrick M, Walsh PR, Wong EKS, Booth C, Kerecuk L, Salama AD, Almond M, Inward C, Goodship TH, Sheerin NS, Marchbank KJ, Kavanagh D.

Kidney Int. 2017 Nov;92(5):1261-1271. doi: 10.1016/j.kint.2017.04.028. Epub 2017 Jul 24.

8.

Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

Goodship TH, Cook HT, Fakhouri F, Fervenza FC, Frémeaux-Bacchi V, Kavanagh D, Nester CM, Noris M, Pickering MC, Rodríguez de Córdoba S, Roumenina LT, Sethi S, Smith RJ; Conference Participants.

Kidney Int. 2017 Mar;91(3):539-551. doi: 10.1016/j.kint.2016.10.005. Epub 2016 Dec 16.

9.

Thrombotic Microangiopathy in Inverted Formin 2-Mediated Renal Disease.

Challis RC, Ring T, Xu Y, Wong EK, Flossmann O, Roberts IS, Ahmed S, Wetherall M, Salkus G, Brocklebank V, Fester J, Strain L, Wilson V, Wood KM, Marchbank KJ, Santibanez-Koref M, Goodship TH, Kavanagh D.

J Am Soc Nephrol. 2017 Apr;28(4):1084-1091. doi: 10.1681/ASN.2015101189. Epub 2016 Dec 14.

10.

Chromosomal rearrangement-A rare cause of complement factor I associated atypical haemolytic uraemic syndrome.

Gleeson PJ, Wilson V, Cox TE, Sharma SD, Smith-Jackson K, Strain L, Lappin D, McHale T, Kavanagh D, Goodship TH.

Immunobiology. 2016 Oct;221(10):1124-30. doi: 10.1016/j.imbio.2016.05.002. Epub 2016 May 10.

PMID:
27268256
11.

The role of ADAMTS-13 activity and complement mutational analysis in differentiating acute thrombotic microangiopathies.

Phillips EH, Westwood JP, Brocklebank V, Wong EK, Tellez JO, Marchbank KJ, McGuckin S, Gale DP, Connolly J, Goodship TH, Kavanagh D, Scully MA.

J Thromb Haemost. 2016 Jan;14(1):175-85. doi: 10.1111/jth.13189. Epub 2016 Jan 11.

12.

A De Novo Deletion in the Regulators of Complement Activation Cluster Producing a Hybrid Complement Factor H/Complement Factor H-Related 3 Gene in Atypical Hemolytic Uremic Syndrome.

Challis RC, Araujo GS, Wong EK, Anderson HE, Awan A, Dorman AM, Waldron M, Wilson V, Brocklebank V, Strain L, Morgan BP, Harris CL, Marchbank KJ, Goodship TH, Kavanagh D.

J Am Soc Nephrol. 2016 Jun;27(6):1617-24. doi: 10.1681/ASN.2015010100. Epub 2015 Oct 21.

13.

Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum?

Ring T, Pedersen BB, Salkus G, Goodship TH.

Clin Kidney J. 2015 Oct;8(5):489-91. doi: 10.1093/ckj/sfv076. Epub 2015 Aug 27.

14.

Thrombotic Microangiopathy as a Cause of Chronic Kidney Transplant Dysfunction: Case Report Demonstrating Successful Treatment with Eculizumab.

Iqbal Z, Wood K, Carter V, Goodship TH, Brown AL, Sheerin NS.

Transplant Proc. 2015 Sep;47(7):2258-61. doi: 10.1016/j.transproceed.2015.08.004.

PMID:
26361694
15.

Mutations in TBX18 Cause Dominant Urinary Tract Malformations via Transcriptional Dysregulation of Ureter Development.

Vivante A, Kleppa MJ, Schulz J, Kohl S, Sharma A, Chen J, Shril S, Hwang DY, Weiss AC, Kaminski MM, Shukrun R, Kemper MJ, Lehnhardt A, Beetz R, Sanna-Cherchi S, Verbitsky M, Gharavi AG, Stuart HM, Feather SA, Goodship JA, Goodship TH, Woolf AS, Westra SJ, Doody DP, Bauer SB, Lee RS, Adam RM, Lu W, Reutter HM, Kehinde EO, Mancini EJ, Lifton RP, Tasic V, Lienkamp SS, Jüppner H, Kispert A, Hildebrandt F.

Am J Hum Genet. 2015 Aug 6;97(2):291-301. doi: 10.1016/j.ajhg.2015.07.001. Epub 2015 Jul 30.

16.

Patient stratification and therapy in atypical haemolytic uraemic syndrome (aHUS).

Wong E, Challis R, Sheerin N, Johnson S, Kavanagh D, Goodship TH.

Immunobiology. 2016 Jun;221(6):715-8. doi: 10.1016/j.imbio.2015.05.002. Epub 2015 May 11.

PMID:
26037115
17.

Case report: Benefits and challenges of long-term eculizumab in atypical hemolytic uremic syndrome.

Cullinan N, Gorman KM, Riordan M, Waldron M, Goodship TH, Awan A.

Pediatrics. 2015 Jun;135(6):e1506-9. doi: 10.1542/peds.2014-3503. Epub 2015 May 4.

18.

Plasma resistant atypical hemolytic uremic syndrome associated with a CFH mutation treated with eculizumab: a case report.

Sevinc M, Basturk T, Sahutoglu T, Sakaci T, Koc Y, Ahbap E, Akgol C, Kara E, Brocklebank V, Goodship TH, Kavanagh D, Unsal A.

J Med Case Rep. 2015 Apr 29;9:92. doi: 10.1186/s13256-015-0575-y.

19.

A national specialized service in England for atypical haemolytic uraemic syndrome-the first year's experience.

Sheerin NS, Kavanagh D, Goodship TH, Johnson S.

QJM. 2016 Jan;109(1):27-33. doi: 10.1093/qjmed/hcv082. Epub 2015 Apr 21.

PMID:
25899302
20.

Atypical aHUS: State of the art.

Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship TH, Kavanagh D, Noris M, Pickering M, Sanchez-Corral P, Skerka C, Zipfel P, Smith RJ.

Mol Immunol. 2015 Sep;67(1):31-42. doi: 10.1016/j.molimm.2015.03.246. Epub 2015 Apr 3. Review.

PMID:
25843230
21.

Factors determining penetrance in familial atypical haemolytic uraemic syndrome.

Sansbury FH, Cordell HJ, Bingham C, Bromilow G, Nicholls A, Powell R, Shields B, Smyth L, Warwicker P, Strain L, Wilson V, Goodship JA, Goodship TH, Turnpenny PD.

J Med Genet. 2014 Nov;51(11):756-64. doi: 10.1136/jmedgenet-2014-102498. Epub 2014 Sep 26.

PMID:
25261570
22.

Autoantibodies to CD59, CD55, CD46 or CD35 are not associated with atypical haemolytic uraemic syndrome (aHUS).

Watson R, Wearmouth E, McLoughlin AC, Jackson A, Ward S, Bertram P, Bennaceur K, Barker CE, Pappworth IY, Kavanagh D, Lea SM, Atkinson JP, Goodship TH, Marchbank KJ.

Mol Immunol. 2015 Feb;63(2):287-96. doi: 10.1016/j.molimm.2014.07.017. Epub 2014 Aug 21.

23.

Urinary tract effects of HPSE2 mutations.

Stuart HM, Roberts NA, Hilton EN, McKenzie EA, Daly SB, Hadfield KD, Rahal JS, Gardiner NJ, Tanley SW, Lewis MA, Sites E, Angle B, Alves C, Lourenço T, Rodrigues M, Calado A, Amado M, Guerreiro N, Serras I, Beetz C, Varga RE, Silay MS, Darlow JM, Dobson MG, Barton DE, Hunziker M, Puri P, Feather SA, Goodship JA, Goodship TH, Lambert HJ, Cordell HJ; UK VUR Study Group, Saggar A, Kinali M; 4C Study Group, Lorenz C, Moeller K, Schaefer F, Bayazit AK, Weber S, Newman WG, Woolf AS.

J Am Soc Nephrol. 2015 Apr;26(4):797-804. doi: 10.1681/ASN.2013090961. Epub 2014 Aug 21.

24.

Atypical haemolytic uraemic syndrome associated with a CD46 mutation triggered by Shigella flexneri.

Brocklebank V, Wong EK, Fielding R, Goodship TH, Kavanagh D.

Clin Kidney J. 2014 Jun;7(3):286-288. Epub 2014 Apr 11.

25.

A novel method for direct measurement of complement convertases activity in human serum.

Blom AM, Volokhina EB, Fransson V, Strömberg P, Berghard L, Viktorelius M, Mollnes TE, López-Trascasa M, van den Heuvel LP, Goodship TH, Marchbank KJ, Okroj M.

Clin Exp Immunol. 2014 Oct;178(1):142-53. doi: 10.1111/cei.12388.

26.

Characterization of a factor H mutation that perturbs the alternative pathway of complement in a family with membranoproliferative GN.

Wong EK, Anderson HE, Herbert AP, Challis RC, Brown P, Reis GS, Tellez JO, Strain L, Fluck N, Humphrey A, Macleod A, Richards A, Ahlert D, Santibanez-Koref M, Barlow PN, Marchbank KJ, Harris CL, Goodship TH, Kavanagh D.

J Am Soc Nephrol. 2014 Nov;25(11):2425-33. doi: 10.1681/ASN.2013070732. Epub 2014 Apr 10.

27.

Cisplatin-induced haemolytic uraemic syndrome associated with a novel intronic mutation of CD46 treated with eculizumab.

Gilbert RD, Stanley LK, Fowler DJ, Angus EM, Hardy SA, Goodship TH.

Clin Kidney J. 2013 Aug;6(4):421-425.

28.

Prevalence in the General Population of a CFH Sequence Variant Associated with Atypical Haemolytic Uraemic Syndrome in an Extensive Family from Southwest England.

Hamilton AJ, Lyons CB, Goodship TH, Bingham C.

Nephron Extra. 2013 Sep 17;3(1):86-90. doi: 10.1159/000354667. eCollection 2013.

29.

Atypical hemolytic uremic syndrome.

Kavanagh D, Goodship TH, Richards A.

Semin Nephrol. 2013 Nov;33(6):508-30. doi: 10.1016/j.semnephrol.2013.08.003. Review.

30.

Genotype/phenotype correlations in complement factor H deficiency arising from uniparental isodisomy.

Wilson V, Darlay R, Wong W, Wood KM, McFarlane J, Schejbel L, Schmidt IM, Harris CL, Tellez J, Hunze EM, Marchbank K, Goodship JA, Goodship TH.

Am J Kidney Dis. 2013 Nov;62(5):978-83. doi: 10.1053/j.ajkd.2013.05.020. Epub 2013 Jul 16.

PMID:
23870792
31.

Complement therapy in atypical haemolytic uraemic syndrome (aHUS).

Wong EK, Goodship TH, Kavanagh D.

Mol Immunol. 2013 Dec 15;56(3):199-212. doi: 10.1016/j.molimm.2013.05.224. Epub 2013 Jun 28. Review.

32.

Eculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B.

Gilbert RD, Fowler DJ, Angus E, Hardy SA, Stanley L, Goodship TH.

Pediatr Nephrol. 2013 Aug;28(8):1315-8. doi: 10.1007/s00467-013-2492-x. Epub 2013 Apr 28.

PMID:
23624872
33.

Determining the population frequency of the CFHR3/CFHR1 deletion at 1q32.

Holmes LV, Strain L, Staniforth SJ, Moore I, Marchbank K, Kavanagh D, Goodship JA, Cordell HJ, Goodship TH.

PLoS One. 2013 Apr 16;8(4):e60352. doi: 10.1371/journal.pone.0060352. Print 2013.

34.

Changing strategies for organ transplantation in atypical haemolytic uraemic syndrome: a tertiary case series.

Forbes TA, Bradbury MG, Goodship TH, McKiernan PJ, Milford DV.

Pediatr Transplant. 2013 May;17(3):E93-9. doi: 10.1111/petr.12066. Epub 2013 Mar 6.

PMID:
23461281
35.

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.

J Am Soc Nephrol. 2013 Feb;24(3):475-86. doi: 10.1681/ASN.2012090884. Epub 2013 Feb 21.

36.

Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation.

Paixão-Cavalcante D, López-Trascasa M, Skattum L, Giclas PC, Goodship TH, de Córdoba SR, Truedsson L, Morgan BP, Harris CL.

Kidney Int. 2012 Nov;82(10):1084-92. doi: 10.1038/ki.2012.250. Epub 2012 Aug 1.

37.

Factor H autoantibodies in membranoproliferative glomerulonephritis.

Goodship TH, Pappworth IY, Toth T, Denton M, Houlberg K, McCormick F, Warland D, Moore I, Hunze EM, Staniforth SJ, Hayes C, Cavalcante DP, Kavanagh D, Strain L, Herbert AP, Schmidt CQ, Barlow PN, Harris CL, Marchbank KJ.

Mol Immunol. 2012 Oct;52(3-4):200-6. doi: 10.1016/j.molimm.2012.05.009. Epub 2012 Jun 20.

PMID:
22721707
38.

Postpartum aHUS secondary to a genetic abnormality in factor H acquired through liver transplantation.

Brown JH, Tellez J, Wilson V, Mackie IJ, Scully M, Tredger MM, Moore I, McDougall NI, Strain L, Marchbank KJ, Sheerin NS, O'Grady J, Harris CL, Goodship TH.

Am J Transplant. 2012 Jun;12(6):1632-6. doi: 10.1111/j.1600-6143.2012.03991.x. Epub 2012 Mar 15.

39.

Factor I autoantibodies in patients with atypical hemolytic uremic syndrome: disease-associated or an epiphenomenon?

Kavanagh D, Pappworth IY, Anderson H, Hayes CM, Moore I, Hunze EM, Bennaceur K, Roversi P, Lea S, Strain L, Ward R, Plant N, Nailescu C, Goodship TH, Marchbank KJ.

Clin J Am Soc Nephrol. 2012 Mar;7(3):417-26. doi: 10.2215/CJN.05750611. Epub 2012 Jan 5.

40.

Atypical hemolytic uremic syndrome, genetic basis, and clinical manifestations.

Kavanagh D, Goodship TH.

Hematology Am Soc Hematol Educ Program. 2011;2011:15-20. doi: 10.1182/asheducation-2011.1.15.

PMID:
22160007
41.

Common genetic variants in complement genes other than CFH, CD46 and the CFHRs are not associated with aHUS.

Ermini L, Goodship TH, Strain L, Weale ME, Sacks SH, Cordell HJ, Fremeaux-Bacchi V, Sheerin NS.

Mol Immunol. 2012 Jan;49(4):640-8. doi: 10.1016/j.molimm.2011.11.003. Epub 2011 Dec 5.

42.

A novel hybrid CFH/CFHR3 gene generated by a microhomology-mediated deletion in familial atypical hemolytic uremic syndrome.

Francis NJ, McNicholas B, Awan A, Waldron M, Reddan D, Sadlier D, Kavanagh D, Strain L, Marchbank KJ, Harris CL, Goodship TH.

Blood. 2012 Jan 12;119(2):591-601. doi: 10.1182/blood-2011-03-339903. Epub 2011 Nov 4.

PMID:
22058112
43.

Successful treatment of de novo posttransplant thrombotic microangiopathy with eculizumab.

Wilson CH, Brown AL, White SA, Goodship TH, Sheerin NS, Manas DM.

Transplantation. 2011 Oct 27;92(8):e42-3. doi: 10.1097/TP.0b013e318230c0bd. No abstract available.

PMID:
21989273
44.

Complement polymorphisms: geographical distribution and relevance to disease.

Ermini L, Wilson IJ, Goodship TH, Sheerin NS.

Immunobiology. 2012 Feb;217(2):265-71. doi: 10.1016/j.imbio.2011.07.020. Epub 2011 Jul 26.

PMID:
21899915
45.

Plasma therapy for atypical haemolytic uraemic syndrome associated with heterozygous factor H mutations.

Kim JJ, Goodship TH, Tizard J, Inward C.

Pediatr Nephrol. 2011 Nov;26(11):2073-6. doi: 10.1007/s00467-011-1944-4. Epub 2011 Jun 30.

PMID:
21717289
46.

Association of genetic variants in complement factor H and factor H-related genes with systemic lupus erythematosus susceptibility.

Zhao J, Wu H, Khosravi M, Cui H, Qian X, Kelly JA, Kaufman KM, Langefeld CD, Williams AH, Comeau ME, Ziegler JT, Marion MC, Adler A, Glenn SB, Alarcón-Riquelme ME; BIOLUPUS Network; GENLES Network, Pons-Estel BA, Harley JB, Bae SC, Bang SY, Cho SK, Jacob CO, Vyse TJ, Niewold TB, Gaffney PM, Moser KL, Kimberly RP, Edberg JC, Brown EE, Alarcon GS, Petri MA, Ramsey-Goldman R, Vilá LM, Reveille JD, James JA, Gilkeson GS, Kamen DL, Freedman BI, Anaya JM, Merrill JT, Criswell LA, Scofield RH, Stevens AM, Guthridge JM, Chang DM, Song YW, Park JA, Lee EY, Boackle SA, Grossman JM, Hahn BH, Goodship TH, Cantor RM, Yu CY, Shen N, Tsao BP.

PLoS Genet. 2011 May;7(5):e1002079. doi: 10.1371/journal.pgen.1002079. Epub 2011 May 26.

47.

Primary, nonsyndromic vesicoureteric reflux and nephropathy in sibling pairs: a United Kingdom cohort for a DNA bank.

Lambert HJ, Stewart A, Gullett AM, Cordell HJ, Malcolm S, Feather SA, Goodship JA, Goodship TH, Woolf AS; UK VUR Study Group.

Clin J Am Soc Nephrol. 2011 Apr;6(4):760-6. doi: 10.2215/CJN.04580510. Epub 2011 Mar 24.

48.

Monogenic diabetes, renal dysplasia and hypopituitarism: a patient with a HNF1A mutation.

Simms RJ, Sayer JA, Quinton R, Walker M, Ellard S, Goodship TH.

QJM. 2011 Oct;104(10):881-3. doi: 10.1093/qjmed/hcq195. Epub 2010 Nov 4. No abstract available.

PMID:
21051477
49.

Successful isolated liver transplantation in a child with atypical hemolytic uremic syndrome and a mutation in complement factor H.

Haller W, Milford DV, Goodship TH, Sharif K, Mirza DF, McKiernan PJ.

Am J Transplant. 2010 Sep;10(9):2142-7. doi: 10.1111/j.1600-6143.2010.03228.x. Epub 2010 Aug 11.

50.

Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation.

Davin JC, Majoie C, Groothoff J, Gracchi V, Bouts A, Goodship TH, Loirat C.

Pediatr Nephrol. 2011 Jan;26(1):155-7. doi: 10.1007/s00467-010-1608-9. Epub 2010 Jul 24. No abstract available.

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