Pre-emptive Eculizumab and Plasmapheresis for Renal Transplant in Atypical Hemolytic Uremic Syndrome

被引：86

作者：

Nester, Carla ^{[1
,2
]}

Stewart, Zoe ^{[3
]}

Myers, David ^{[1
]}

Jetton, Jennifer ^{[1
]}

Nair, Ramesh ^{[4
]}

Reed, Alan ^{[3
]}

Thomas, Christie ^{[2
]}

Smith, Richard ^{[4
,5
]}

Brophy, Patrick ^{[1
]}

机构：

[1] Univ Iowa, Dept Pediat, Iowa City, IA 52242 USA

[2] Univ Iowa, Dept Internal Med, Iowa City, IA 52242 USA

[3] Univ Iowa, Dept Surg, Iowa City, IA 52242 USA

[4] Univ Iowa, Dept Pathol, Iowa City, IA 52242 USA

[5] Univ Iowa, Dept Otolaryngol, Iowa City, IA USA

来源：

CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY | 2011年 / 6卷 / 06期

关键词：

COMPLEMENT INHIBITOR ECULIZUMAB; PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA; LIVER-KIDNEY TRANSPLANTATION; FACTOR-H; CLINICAL PRESENTATION; MUTATIONS; AHUS; HUS; CFH; C3;

D O I：

10.2215/CJN.10181110

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：

The case of a 12-year-old with a hybrid CFH/CFHL1 gene and atypical hemolytic uremic syndrome (aHUS) that had previously developed native kidney and then renal allograft loss is reported. This case illustrates the relatively common occurrence of renal loss from the late presentation of aHUS. Also presented is a protocol for the pre-emptive use of eculizumab and plasmapheresis as part of a renal transplant plan for the treatment of aHUS in patients deemed at high risk for recurrent disease. This protocol was a result of a multidisciplinary approach including adult and pediatric nephrology, transplant surgery, transfusion medicine, and infectious disease specialists. This protocol and the justifications and components of it can function as a guideline for the treatment of a group of children that have waited in limbo for the first U.S. transplant to open the door to this type of definitive care for this devastating disease. Clin J Am Soc Nephrol 6: 1488-1494, 2011. doi: 10.2215/CJN.10181110

引用

页码：1488 / 1494

页数：7

共 38 条

[11] Recurrence of hemolytic uremic syndrome after live related renal transplantation associated with subsequent de novo disease in the donor [J].

Donne, RL ;

Abbs, I ;

Barany, P ;

Elinder, CG ;

Little, M ;

Conlon, P ;

Goodship, THJ .

AMERICAN JOURNAL OF KIDNEY DISEASES, 2002, 40 (06)

[12] Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome [J].

Fremeaux-Bacchi, Veronique ;

Miller, Elizabeth C. ;

Liszewski, M. Kathryn ;

Strain, Lisa ;

Blouin, Jacques ;

Brown, Alison L. ;

Moghal, Nadeem ;

Kaplan, Bernard S. ;

Weiss, Robert A. ;

Lhotta, Karl ;

Kapur, Gaurav ;

Mattoo, Tej ;

Nivet, Hubert ;

Wong, William ;

Gie, Sophie ;

de Ligny, Bruno Hurault ;

Fischbach, Michel ;

Gupta, Ritu ;

Hauhart, Richard ;

Meunier, Vincent ;

Loirat, Chantal ;

Dragon-Durey, Marie-Agnes ;

Fridman, Wolf H. ;

Janssen, Bert J. C. ;

Goodship, Timothy H. J. ;

Atkinson, John P. .

BLOOD, 2008, 112 (13) :4948-4952

[13] Eculizumab for Congenital Atypical Hemolytic-Uremic Syndrome. [J].

Gruppo, Ralph A. ;

Rother, Russell P. .

NEW ENGLAND JOURNAL OF MEDICINE, 2009, 360 (05) :544-546

[14] The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria [J].

Hillmen, Peter ;

Young, Neal S. ;

Schubert, Joerg ;

Brodsky, Robert A. ;

Socie, Gerard ;

Muus, Petra ;

Roeth, Alexander ;

Szer, Jeffrey ;

Elebute, Modupe O. ;

Nakamura, Ryotaro ;

Browne, Paul ;

Risitano, Antonio M. ;

Hill, Anita ;

Schrezenmeier, Hubert ;

Fu, Chieh-Lin ;

Maciejewski, Jaroslaw ;

Rollins, Scott A. ;

Mojcik, Christopher F. ;

Rother, Russell P. ;

Luzzatto, Lucio .

NEW ENGLAND JOURNAL OF MEDICINE, 2006, 355 (12) :1233-1243

[15] Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H [J].

Jalanko, H. ;

Peltonen, S. ;

Koskinen, A. ;

Puntila, J. ;

Isoniemi, H. ;

Holmberg, C. ;

Pinomaki, A. ;

Armstrong, E. ;

Koivusalo, A. ;

Tukiainen, E. ;

Makisalo, H. ;

Saland, J. ;

Remuzzi, G. ;

de Cordoba, S. ;

Lassila, R. ;

Meri, S. ;

Jokiranta, T. S. .

AMERICAN JOURNAL OF TRANSPLANTATION, 2008, 8 (01) :216-221

[16] Genetics and complement in atypical HUS [J].

Kavanagh, David ;

Goodship, Tim .

PEDIATRIC NEPHROLOGY, 2010, 25 (12) :2431-2442

[17]

LARREA CF, 2010, TRANSPLANTATION, V15, P903

[18] A Large Family with a Gain-of-Function Mutation of Complement C3 Predisposing to Atypical Hemolytic Uremic Syndrome, Microhematuria, Hypertension and Chronic Renal Failure [J].

Lhotta, Karl ;

Janecke, Andreas R. ;

Scheiring, Johanna ;

Petzlberger, Barbara ;

Giner, Thomas ;

Fally, Verena ;

Wuerzner, Reinhard ;

Zimmerhackl, Lothar B. ;

Mayer, Gert ;

Fremeaux-Bacchi, Veronique .

CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2009, 4 (08) :1356-1362

[19] Complement and the atypical hemolytic uremic syndrome in children [J].

Loirat, Chantal ;

Noris, Marina ;

Fremeaux-Bacchi, Veronique .

PEDIATRIC NEPHROLOGY, 2008, 23 (11) :1957-1972

[20] Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome [J].

Mache, Christoph J. ;

Acham-Roschitz, Birgit ;

Fremeaux-Bacchi, Veronique ;

Kirschfink, Michael ;

Zipfel, Peter F. ;

Roedl, Siegfried ;

Vester, Udo ;

Ring, Ekkehard .

CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2009, 4 (08) :1312-1316

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