Targeted strategies in the prevention and management of atypical HUS recurrence after kidney transplantation

Zuber, Julien; Quintrec, Moglie Le; Morris, Heather; Frémeaux‐Bacchi, Véronique; Loirat, Chantal; Legendre, Christophe

doi:10.1016/j.trre.2013.07.003

Cited by 84 publications

(116 citation statements)

References 120 publications

Supporting

Mentioning

107

Contrasting

Unclassified

Order By: Relevance

“…The pathogenic mechanisms are not well understood, but it is likely to be multifactorial, with ischemia-reperfusion injury, antibody-mediated rejection, viral infections such as cytomegalovirus, and immunosuppressant drugs, especially calcineurin inhibitors (CNIs), contributing to an "endothelial damaging milieu" (95). In one series of de novo TMA after kidney transplantation, complement mutations were identified in 29% (96), providing a rationale for complementinhibiting therapy especially where the primary diagnosis was not incompatible with complement-mediated aHUS.…”

Section: Drug-mediated Tmamentioning

confidence: 99%

Thrombotic Microangiopathy and the Kidney

Brocklebank

Wood

Kavanagh

2017

CJASN

288

View full text Add to dashboard Cite

Thrombotic microangiopathy can manifest in a diverse range of diseases and is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury, including AKI. It can be associated with significant morbidity and mortality, but a systematic approach to investigation and prompt initiation of supportive management and, in some cases, effective specific treatment can result in good outcomes. This review considers the classification, pathology, epidemiology, characteristics, and pathogenesis of the thrombotic microangiopathies, and outlines a pragmatic approach to diagnosis and management.

show abstract

Section: Drug-mediated Tmamentioning

confidence: 99%

Thrombotic Microangiopathy and the Kidney

Brocklebank

Wood

Kavanagh

2017

CJASN

288

View full text Add to dashboard Cite

show abstract

“…[19] It has a recurrence of 70%-85% in renal transplantation, being higher if it affects the C-terminal portion (site of patient variation). [20] Patients with Factor H mutation have a high incidence of cardiovascular disease and increased mortality. [1] 8% of cases of aHUS associated with Factor H mutation are triggered by pregnancy or oral contraceptive drugs, like our patient.…”

Section: The Cfh Gene Is Located In the Long Arm Of Chromosomementioning

confidence: 99%

Novel Complement Factor H mutation, a case report of atypical hemolytic uremic syndrome

Sepúlveda

Tagle

Jara

2017

CRIM

View full text Add to dashboard Cite

Atypical hemolytic uremic syndrome (aHUS) is a rare but catastrophic disease. It is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. When the aHUS is primary, the cause is due to mutations in proteins that regulate the alternative pathway of complement, such as Factor H, Factor I, Factor B, C3, Membrane Co-Factor Protein and Thrombomodulin. Usually primary aHUS is associated with other amplifiers complement factors. We present a case of aHUS in a 25-year-old female patient; she presented with malignant hypertension and severe renal failure. After a widespread study, the etiology of the aHUS was a mutation in the complement factor H, not previously described in the literature (p.Tyr1177His). After treatment with Eculizumab (C5 inhibitor monoclonal antibody), she recovered renal function with not hemodialysis requirements.

show abstract

“…Eculizumab inhibits activation of the terminal complement pathway (C5), and its effectiveness in treating aHUS was demonstrated in many studies. [94][95][96][97] Legendre et al 96 studied the outcome of 37 aHUS patients after 26 weeks of eculizumab therapy in two clinical trials. In total, 80% of patients treated with eculizumab had improvements in anemia, thrombocytopenia, and renal function, resulting in eventual cessation of dialysis.…”

Section: Role Of Tbi In the Development Of Post-bmt Tmamentioning

confidence: 99%

Post-bone marrow transplant thrombotic microangiopathy

Obut

Kasinath

Abdi

2016

Bone Marrow Transplant

View full text Add to dashboard Cite

Thrombotic microangiopathy (TMA) is a systemic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ failure. Post-bone marrow transplant TMA (post-BMT TMA) is a life-threatening condition that has been reported to afflict between 0.5 and 63.6% of BMT patients. The incidence of post-BMT TMA is affected by evolving therapies such as conditioning regimens. The etiology of post-BMT TMA is thought to be multifactorial, including the effects of immunosuppressive agents, viral infections, TBI and GvHD. A growing body of evidence highlights the importance of complement system activation and endothelial damage in post-BMT TMA. Although plasmapheresis has commonly been used, its therapeutic rationale for the majority of post-BMT TMA cases is unclear in the absence of circulatory inhibitors. It has become possible to target complement activation with eculizumab, a drug that blocks the terminal complement pathway. Early studies have highlighted the importance of anticomplement therapies in treating post-BMT TMA. Moreover, finding complement gene mutations may identify patients at risk, but whether such patients benefit from prophylactic anti-complement therapies before BMT remains to be studied. This review focuses on diagnostic criteria, pathophysiology, treatment and renal outcomes of post-BMT TMA.

show abstract

Targeted strategies in the prevention and management of atypical HUS recurrence after kidney transplantation

Cited by 84 publications

References 120 publications

Thrombotic Microangiopathy and the Kidney

Thrombotic Microangiopathy and the Kidney

Novel Complement Factor H mutation, a case report of atypical hemolytic uremic syndrome

Post-bone marrow transplant thrombotic microangiopathy

Contact Info

Product

Resources

About