Recurrence of haemolytic-uraemic syndrome in renal transplants: a single-centre report

Miller, Robert B.; Burke, Barbara A.; Schmidt, Walter; Gillingham, Kristen J.; Matas, Arthur J.; Mauer, Michael; Kashtan, Clifford E.

doi:10.1093/ndt/12.7.1425

Cited by 51 publications

(52 citation statements)

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“…Renal transplantation is not necessarily an option for non-Stx-HUS, at variance with Stx-HUS. Actually, approximately 50% of the patients who had a renal transplant had a recurrence of the disease in the grafted organ (86,155). Recurrences occur at a median time of 30 d after transplant (range, 0 d to 16 yr).…”

Section: Which Treatment For Non-stx-hus?mentioning

confidence: 99%

Hemolytic Uremic Syndrome

Noris¹,

Remuzzi²

2005

Journal of the American Society of Nephrology

499

428

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Section: Which Treatment For Non-stx-hus?mentioning

confidence: 99%

Hemolytic Uremic Syndrome

Noris¹,

Remuzzi²

2005

Journal of the American Society of Nephrology

499

428

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“…ndividuals who suffer ESRD due to atypical hemolytic uremic syndrome (aHUS) have poor outcomes after kidney transplant because of high rates of disease recurrence (1)(2)(3). In the case of carriers of a CFH [complement factor H (CFH)] mutation, the recurrence risk is over 80%, usually accompanied by graft loss (2,4,5).…”

mentioning

confidence: 99%

Successful Split Liver-Kidney Transplant for Factor H Associated Hemolytic Uremic Syndrome

Saland¹,

Shneider²,

Bromberg³

et al. 2009

Clinical Journal of the American Society of Nephrology

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Background and objectives: A male infant with a family history of thrombotic microangiopathy developed atypical hemolytic uremic syndrome (aHUS).Design, setting, participants, & measurements: Case report. Results: Genetic analysis demonstrated a heterozygous mutation (S1191L) of CFH, the gene coding complement factor H (CFH). The child suffered many episodes of HUS, each treated with plasma exchange. In time, despite initiation of a prophylactic regimen of plasma exchange, his renal function declined significantly. At the age of 4 yr he received a (split liver) combined liver-kidney transplant (LKT) with preoperative plasma exchange and enoxaparin anticoagulation. Initial function of both grafts was excellent and is maintained for nearly 2 yr.Conclusions: This report adds to the small but growing number of individuals in whom LKT has provided a favorable outcome for aHUS associated with CFH mutation, expands the technique of using a split liver graft, and describes the unique histologic features of subclinical liver disease in HUS.

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“…Some authors have also emphasized that the risk of recurrence is higher in patients with recurrent and familial forms of HUS, whereas it would be negligible in children with the typical form of HUS. Miller et al (138) reported a recurrence rate of 67% among 18 patients with atypical HUS and of 17% among 6 patients with typical, diarrheaassociated HUS. The outcome of post-transplant HUS is poor, with a 5-yr graft failure rate of 63% in one series (139).…”

Section: Transplant-associated Thrombotic Microangiopathymentioning

confidence: 99%