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

[1] Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome [J].

Ariceta, Gema ;

Besbas, Nesrin ;

Johnson, Sally ;

Karpman, Diana ;

Landau, Daniel ;

Licht, Christoph ;

Loirat, Chantal ;

Pecoraro, Carmine ;

Taylor, C. Mark ;

Van de Kar, Nicole ;

VandeWalle, Johan ;

Zimmerhackl, Lothar B. .

PEDIATRIC NEPHROLOGY, 2009, 24 (04) :687-696

[2] A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders [J].

Besbas, N. ;

Karpman, D. ;

Landau, D. ;

Loirat, C. ;

Proesmans, W. ;

Remuzzi, G. ;

Rizzoni, G. ;

Taylor, C. M. ;

Van de Kar, N. ;

Zimmerhackl, L. B. .

KIDNEY INTERNATIONAL, 2006, 70 (03) :423-431

[3] Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: Prognostic significance-of genetic background [J].

Bresin, Elena ;

Daina, Erica ;

Noris, Marina ;

Castelletti, Federica ;

Stefanov, Rumen ;

Hill, Prudence ;

Goodship, Timothy H. J. ;

Remuzzi, Giuseppe .

CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2006, 1 (01) :88-99

[4] Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria [J].

Brodsky, Robert A. ;

Young, Neal S. ;

Antonioli, Elisabetta ;

Risitano, Antonio M. ;

Schrezenmeier, Hubert ;

Schubert, Jorg ;

Gaya, Anna ;

Coyle, Luke ;

De Castro, Carlos ;

Fu, Chieh-Lin ;

Maciejewski, Jaroslaw P. ;

Bessler, Monica ;

Kroon, Henk-Andre ;

Rother, Russell P. ;

Hillmen, Peter .

BLOOD, 2008, 111 (04) :1840-1847

[5] 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

[6] Recurrent Atypical Hemolytic Uremic Syndrome Associated With Factor I Mutation in a Living Related Renal Transplant Recipient [J].

Chan, Micah R. ;

Thomas, Christie P. ;

Torrealba, Jose R. ;

Djamali, Ariang ;

Fernandez, Luis A. ;

Nishimura, Carla J. ;

Smith, Richard J. H. ;

Samaniego, Millie D. .

AMERICAN JOURNAL OF KIDNEY DISEASES, 2009, 53 (02) :321-326

[7] Safety and Long-Term Efficacy of Eculizumab in a Renal Transplant Patient with Recurrent Atypical Hemolytic-Uremic Syndrome [J].

Chatelet, V. ;

Fremeaux-Bacchi, V. ;

Lobbedez, T. ;

Ficheux, M. ;

de Ligny, B. Hurault .

AMERICAN JOURNAL OF TRANSPLANTATION, 2009, 9 (11) :2644-2645

[8] Maintenance of Kidney Function Following Treatment With Eculizumab and Discontinuation of Plasma Exchange After a Third Kidney Transplant for Atypical Hemolytic Uremic Syndrome Associated With a CFH Mutation [J].

Davin, Jean-Claude ;

Gracchi, Valentina ;

Bouts, Antonia ;

Groothoff, Jaap ;

Strain, Lisa ;

Goodship, Tim .

AMERICAN JOURNAL OF KIDNEY DISEASES, 2010, 55 (04) :708-711

[9] Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome [J].

de Jorge, Elena Goicoechea ;

Harris, Claire L. ;

Esparza-Gordillo, Jorge ;

Carreras, Luis ;

Arranz, Elena Aller ;

Garrido, Cynthia Abarrategui ;

Lopez-Trascasa, Margarita ;

Sanchez-Corral, Pilar ;

Morgan, B. Paul ;

Rodriguez de Cordoba, Santiago .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2007, 104 (01) :240-245

[10] Thrombomodulin Mutations in Atypical Hemolytic-Uremic Syndrome [J].

Delvaeye, Mieke ;

Noris, Marina ;

De Vriese, Astrid ;

Esmon, Charles T. ;

Esmon, Naomi L. ;

Ferrell, Gary ;

Del-Favero, Jurgen ;

Plaisance, Stephane ;

Claes, Bart ;

Lambrechts, Diether ;

Zoja, Carla ;

Remuzzi, Giuseppe ;

Conway, Edward M. .

NEW ENGLAND JOURNAL OF MEDICINE, 2009, 361 (04) :345-357

← 1 2 3 4 →