Remission of aHUS neurological damage with eculizumab

被引：8

作者：

Avila, Ana ^{[1
]}

Vizcaino, Belen ^{[1
]}

Molina, Pablo ^{[1
]}

Gavela, Eva ^{[1
]}

Perez-Ebri, Maria ^{[2
]}

Pallardo, Luis ^{[1
]}

机构：

[1] Hosp Univ Dr Peset, Dept Nephrol, Valencia, Spain

[2] Univ Dr Peset Hosp, Dept Pathol, Valencia, Spain

来源：

CLINICAL KIDNEY JOURNAL | 2015年 / 8卷 / 02期

关键词：

atypical haemolytic uraemic syndrome; eculizumab; neurologic involvement; plasma exchange; thrombotic microangiopathy;

D O I：

10.1093/ckj/sfu144

中图分类号：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

摘要：

Atypical haemolytic uraemic syndrome (aHUS) is a rare disease characterized by haemolytic microangiopathic anaemia, thrombocytopaenia and acute onset of renal failure, in the absence of Escherichia coli infection. Renal damage usually progresses to end-stage renal disease (ESRD), sometimes being accompanied by signs of extrarenal thrombotic microangiopathy (TMA). We report a case of full neurological and haematological recovery after eculizumab treatment in a patient with ESRD secondary to chronic aHUS refractory to plasmatherapy while she was under dialysis. It highlights the use of eculizumab for controlling extrarenal manifestations of aHUS in this population.

引用

页码：232 / 236

页数：5

共 25 条

[1] Eculizumab in an anephric patient with atypical haemolytic uraemic syndrome and advanced vascular lesions [J].

Bekassy, Zivile D. ;

Kristoffersson, Ann-Charlotte ;

Cronqvist, Mats ;

Roumenina, Lubka T. ;

Rybkine, Tania ;

Vergoz, Laura ;

Hue, Christophe ;

Fremeaux-Bacchi, Veronique ;

Karpman, Diana .

NEPHROLOGY DIALYSIS TRANSPLANTATION, 2013, 28 (11) :2899-2907

[2] Genetics of HUS:: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome [J].

Caprioli, Jessica ;

Noris, Marina ;

Brioschi, Simona ;

Pianetti, Gaia ;

Castelletti, Federica ;

Bettinaglio, Paola ;

Mele, Caterina ;

Bresin, Elena ;

Cassis, Linda ;

Gamba, Sara ;

Porrati, Francesca ;

Bucchioni, Sara ;

Monteferrante, Giuseppe ;

Fang, Celia J. ;

Liszewski, M. K. ;

Kavanagh, David ;

Atkinson, John P. ;

Remuzzi, Giuseppe .

BLOOD, 2006, 108 (04) :1267-1279

[3] Eculizumab as rescue therapy for atypical hemolytic uremic syndrome with normal platelet count [J].

Dorresteijn, Eiske M. ;

van de Kar, Nicole C. A. J. ;

Cransberg, Karlien .

PEDIATRIC NEPHROLOGY, 2012, 27 (07) :1193-1195

[4] VEGF inhibition and renal thrombotic microangiopathy [J].

Eremina, Vera ;

Jefferson, J. Ashley ;

Kowalewska, Jolanta ;

Hochster, Howard ;

Haas, Mark ;

Weisstuch, Joseph ;

Richardson, Catherine ;

Kopp, Jeffrey B. ;

Kabir, M. Golam ;

Backx, Peter H. ;

Gerber, Hans-Peter ;

Ferrara, Napoleone ;

Barisoni, Laura ;

Alpers, Charles E. ;

Quaggin, Susan E. .

NEW ENGLAND JOURNAL OF MEDICINE, 2008, 358 (11) :1129-1136

[5]

Fakhouri F, 2013, AM SOC NEPHR KIDN WE

[6] Genetics and Outcome of Atypical Hemolytic Uremic Syndrome: A Nationwide French Series Comparing Children and Adults [J].

Fremeaux-Bacchi, Veronique ;

Fakhouri, Fadi ;

Gamier, Arnaud ;

Bienaime, Frank ;

Dragon-Durey, Marie-Agnes ;

Ngo, Stephanie ;

Moulin, Bruno ;

Servais, Aude ;

Provot, Francois ;

Rostaing, Lionel ;

Burtey, Stephane ;

Niaudet, Patrick ;

Deschenes, Georges ;

Lebranchu, Yvon ;

Zuber, Julien ;

Loirat, Chantal .

CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2013, 8 (04) :554-562

[7]

Greenbaum LA, 2013, 23 ASN KIDN WEEK SA, p821A

[8] Eculizumab in atypical haemolytic uraemic syndrome with severe cardiac and neurological involvement [J].

Hu, Hushi ;

Nagra, Arvind ;

Haq, Mushfequr R. ;

Gilbert, Rodney D. .

PEDIATRIC NEPHROLOGY, 2014, 29 (06) :1103-1106

[9] Terminal Complement Inhibitor Eculizumab in Atypical Hemolytic-Uremic Syndrome [J].

Legendre, C. M. ;

Licht, C. ;

Muus, P. ;

Greenbaum, L. A. ;

Babu, S. ;

Bedrosian, C. ;

Bingham, C. ;

Cohen, D. J. ;

Delmas, Y. ;

Douglas, K. ;

Eitner, F. ;

Feldkamp, T. ;

Fouque, D. ;

Furman, R. R. ;

Gaber, O. ;

Herthelius, M. ;

Hourmant, M. ;

Karpman, D. ;

Lebranchu, Y. ;

Mariat, C. ;

Menne, J. ;

Moulin, B. ;

Nuernberger, J. ;

Ogawa, M. ;

Remuzzi, G. ;

Richard, T. ;

Sberro-Soussan, R. ;

Severino, B. ;

Sheerin, N. S. ;

Trivelli, A. ;

Zimmerhackl, L. B. ;

Goodship, T. ;

Loirat, C. .

NEW ENGLAND JOURNAL OF MEDICINE, 2013, 368 (23) :2169-2181

[10] Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome [J].

Lemaire, Mathieu ;

Fremeaux-Bacchi, Veronique ;

Schaefer, Franz ;

Choi, Murim ;

Tang, Wai Ho ;

Le Quintrec, Moglie ;

Fakhouri, Fadi ;

Taque, Sophie ;

Nobili, Franois ;

Martinez, Frank ;

Ji, Weizhen ;

Overton, John D. ;

Mane, Shrikant M. ;

Nuernberg, Gudrun ;

Altmueller, Janine ;

Thiele, Holger ;

Morin, Denis ;

Deschenes, Georges ;

Baudouin, Veronique ;

Llanas, Brigitte ;

Collard, Laure ;

Majid, Mohammed A. ;

Simkova, Eva ;

Nuernberg, Peter ;

Rioux-Leclercq, Nathalie ;

Moeckel, Gilbert W. ;

Gubler, Marie Claire ;

Hwa, John ;

Loirat, Chantal ;

Lifton, Richard P. .

NATURE GENETICS, 2013, 45 (05) :531-+

← 1 2 3 →