Neonatal onset atypical hemolytic uremic syndrome successfully treated with eculizumab

Pediatric Nephrology

Volumn 28, Issue 1, 2013, Pages 155-158

  Besbas, Nesrin ^a   Gulhan, Bora ^a   Karpman, Diana ^b   Topaloglu, Rezan ^a   Duzova, Ali ^a   Korkmaz, Emine ^a   Ozaltin, Fatih ^a  

^a HACETTEPE UNIVERSITY   (Turkey)

^b LUND UNIVERSITY   (Sweden)

Author keywords

Atypical hemolytic uremic syndrome; Complement factor H mutation; Eculizumab; Newborn

Indexed keywords

COMPLEMENT COMPONENT C3; COMPLEMENT FACTOR H; ECULIZUMAB; ENALAPRIL; FRESH FROZEN PLASMA; FUROSEMIDE; NIFEDIPINE; NITROPRUSSIDE SODIUM; PROPRANOLOL;

ARTICLE; CASE REPORT; DRUG EFFICACY; GENETIC SCREENING; HEMATOLOGICAL PARAMETERS; HEMOLYTIC ANEMIA; HEMOLYTIC UREMIC SYNDROME; HUMAN; HYPERTENSION; KIDNEY DYSFUNCTION; KIDNEY FUNCTION; MALE; NEWBORN; ONSET AGE; PERITONEAL DIALYSIS; PHYSICAL EXAMINATION; PRIORITY JOURNAL; RECURRENT DISEASE; REMISSION; THROMBOCYTOPENIA; TREATMENT OUTCOME;

ANTIBODIES, MONOCLONAL, HUMANIZED; COMPLEMENT ACTIVATION; COMPLEMENT C5; COMPLEMENT FACTOR H; HEMOLYTIC-UREMIC SYNDROME; HUMANS; INFANT, NEWBORN; KIDNEY DISEASES; MALE;

EID: 84870411202     PISSN: 0931041X     EISSN: 1432198X     Source Type: Journal    
DOI: 10.1007/s00467-012-2296-4     Document Type: Article

References (15)

1. Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, Rizzoni G, Taylor CM, Van de Kar N, Zimmerhackl LB, European Paediatric Research Group for HUS (2006) A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int 70:423-431
2. Scheiring J, Rosales A, Zimmerhackl LB (2010) Clinical practice. Today's understanding of the haemolytic uraemic syndrome. Eur J Pediatr 169:7-13
3. Kavanagh D, Goodship T (2010) Genetics and complement in atypical HUS. Pediatr Nephrol 25:2431-2442
4. Moore I, Strain L, Pappworth I, Kavanagh D, Barlow PN, Herbert AP, Schmidt CQ, Staniforth SJ, Holmes LV, Ward R, Morgan L, Goodship TH, Marchbank KJ (2010) Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome. Blood 115:379-387
5. Delvaeye M, Noris M, De Vriese A, Esmon CT, Esmon NL, Ferrell G, Del-Favero J, Plaisance S, Claes B, Lambrechts D, Zoja C, Remuzzi G, Conway EM (2009) Thrombomodulin mutations in atypical hemolytic-uremic syndrome. N Engl J Med 361:345-357
6. Menni F, Testa S, Guez S, Chiarelli G, Alberti L, Esposito S (2012) Neonatal atypical hemolytic uremic syndrome due to methylmalonic aciduria and homocystinuria. Pediatr Nephrol 27:1401-1405
7. Fremeaux-Bacchi V (2012) Treatment of atypical uraemic syndrome in the era of eculizumab. Clin Kidney J 5:4-6
8. Loirat C, Frèmeaux-Bacchi V (2011) Atypical hemolytic uremic syndrome. Orphanet J Rare Dis 6:60
9. Waters AM, Licht C (2011) aHUS caused by complement dysregulation: new therapies on the horizon. Pediatr Nephrol 26:41-57
10. Vaziri-Sani F, Holmberg L, Sjöholm AG, Kristoffersson AC, Manea M, Frémeaux-Bacchi V, Fehrman-Ekholm I, Raafat R, Karpman D (2006) Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome. Kidney Int 69:981-988
11. Gruppo RA, Rother RP (2009) Eculizumab for congenital atypical hemolytic uremic syndrome. N Eng J Med 360:544-546
12. Mache CJ, Acham-Roschitz B, Frèmeaux-Bacchi V, Kirschfink M, Zipfel PF, Roedl S, Vester U, Ring E (2009) Complement inhibitor eculizumab in atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 4:1312-1316
13. Köse A, Zimmerhackl LB, Jungraithmayr T, Mache C, Nürnberger J (2010) New treatment options for atypical hemolytic uremic syndrome with the complement inhibitor eculizumab. Semin Thromb Hemost 36:669-672
14. Lapeyraque AL, Frèmeaux-Bacchi V, Robitaille P (2011) Efficacy of eculizumab in patient with factor-H-associated atypical hemolytic uremic syndrome. Pediatr Nephrol 26:621-624
15. Ariceta G, Arrizabalaga B, Aguirre M, Morteruel E, Lopez-Trascasa M (2012) Eculizumab in the treatment of atypical hemolytic uremic syndrome in infants. Am J Kidney Dis 59:707-710