Complement Genes Strongly Predict Recurrence and Graft Outcome in Adult Renal Transplant Recipients with Atypical Hemolytic and Uremic Syndrome

Quintrec, Moglie Le; Zuber, Julien; Moulin, Anne‐Marie; Kamar, Nassim; Jablonski, Mathieu; Lionet, Arnaud; Châtelet, Valérie; Mousson, Christiane; Mourad, Georges; Bridoux, Frank; Cassuto, Élisabeth; Loirat, Chantal; Rondeau, Éric; Delahousse, Michel; Frémeaux‐Bacchi, Véronique

doi:10.1111/ajt.12077

Cited by 237 publications

(259 citation statements)

References 58 publications

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“…Of nine patients who received pre-emptive plasma, four had an event-free successful renal transplantation. TMA occurred in three other patients who were successfully treated with eculizumab in each case [66].…”

Section: Kidney Transplantationmentioning

confidence: 96%

“…Plasma administration on occurrence of TMA in aHUS patients with a kidney transplant is of limited value [65,66]. Prophylactic plasma can decrease graft loss.…”

Section: Kidney Transplantationmentioning

confidence: 99%

“…For this group, eculizumab prophylactic therapy has been proposed as preferable to PE for several reasons, including failure of PE to prevent TMA, the unpredictable risk of TMA when PE is tapered and subclinical progression of disease [46,48,66,68].…”

Section: Kidney Transplantationmentioning

confidence: 99%

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Atypical hemolytic uremic syndrome: from diagnosis to treatment

Franchini¹

2015

Clinical Chemistry and Laboratory Medicine (CCLM)

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Thrombotic microangiopathy (TMA) is a relatively rare condition but a medical urgency requiring immediate intervention to avoid irreversible organ damage or death. Symptoms on presentation include microangiopathic haemolytic anaemia, thrombocytopenia and organ damage. The most frequent direct causes of TMA are thrombotic thrombocytopenic purpura (TTP) and haemolytic uremic syndrome (HUS). The most common form of HUS is related to Shiga toxin producing Escherichia coli (STEC) infection while approximately 10% of cases are due to dysregulation of the complement pathway (atypical haemolytic uremic syndrome, aHUS). Optimal treatment regimens differ depending on the underlying cause; however, differential diagnosis may be difficult. The most accurate method of diagnosis is based on exclusion and should consider, beyond the symptoms common to TMA, ADAMTS13 activity levels and STEC infection status. For the management of TTP, plasma exchange (PE) is the most important acute intervention and is associated with lower mortality and better outcomes than plasma infusion. In most patients with STEC-HUS, the course of disease is selflimiting although management of acute kidney injury is often required. Until recently, the management of aHUS consisted of early and intensive PE, although this was mostly ineffective in protecting from subsequent organ damage. Eculizumab, an inhibitor of the alternative complement pathway, produces a rapid and sustained inhibition of the TMA process, with significant improvements in long-term clinical outcomes. Due to the significant improvement achieved, eculizumab has subsequently been approved as first-line therapy when an unequivocal diagnosis of aHUS has been made.

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Section: Kidney Transplantationmentioning

confidence: 96%

“…Plasma administration on occurrence of TMA in aHUS patients with a kidney transplant is of limited value [65,66]. Prophylactic plasma can decrease graft loss.…”

Section: Kidney Transplantationmentioning

confidence: 99%

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Atypical hemolytic uremic syndrome: from diagnosis to treatment

Franchini¹

2015

Clinical Chemistry and Laboratory Medicine (CCLM)

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“…Végállapotú veseelégtelenség esetén az atípusos HUS alapdiagnózis hosszú ideig a vesetranszplantáció ellenjavallatát képezte, döntően a kifejezetten rossz grafttúlélé-si adatok miatt. Atípusos HUS-ban végzett vesetranszplantáció esetén, történeti adatok szerint, a graftvesztés legfőbb oka az alapbetegség visszatérése volt [93]. Ez kialakulhat közvetlenül az átültetést követően is, az idé-zett tanulmányban jellemzően 6 hónappal a transzplantációt követően lépett fel, és a betegek ~60-90%-át érin-tette a különböző esetsorozat-leírások adatai szerint [94,95].…”

Section: Rápia (1b) éLetveszélyes Extrarenális Tünetek Ese Tén Az Iunclassified

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

…¹

2017

Orvosi Hetilap

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“…[55][56][57][58] Complement system mutations can be implicated in up to 52% of patients who have aHUS. 59 Many different variants in complement genes have been reported to predispose to aHUS, 60-63 such as complement factor H (CFH), 64,65 factor I (CFI), 66,67 C3, 68 thrombomodulin (THBD), 60 membrane cofactor protein (MCP or CD46) 69,70 and factor B (CFB).…”

Section: Role Of Genetic Mutations In Post-bmt Tmamentioning

confidence: 99%

Post-bone marrow transplant thrombotic microangiopathy

Obut

Kasinath

Abdi

2016

Bone Marrow Transplant

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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.

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Complement Genes Strongly Predict Recurrence and Graft Outcome in Adult Renal Transplant Recipients with Atypical Hemolytic and Uremic Syndrome

Cited by 237 publications

References 58 publications

Atypical hemolytic uremic syndrome: from diagnosis to treatment

Atypical hemolytic uremic syndrome: from diagnosis to treatment

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

Post-bone marrow transplant thrombotic microangiopathy

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