Renal transplantation in HUS patients with disorders of complement regulation

Zimmerhackl, Lothar Bernd; Scheiring, Johanna; Prüfer, Friederike; Taylor, Christopher M.; Loirat, Chantal

doi:10.1007/s00467-006-0210-7

Cited by 34 publications

(25 citation statements)

References 25 publications

(30 reference statements)

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“…However, in the studies published elsewhere, idiopathic TTP-HUS was one of the most common forms of the disease which accounted for 27–40% of the patients [4, 6, 21]. Atypical HUS affected about 5–10% of all patients with TTP-HUS according to literature [22,23,24]; however, no patient was diagnosed as typical TTP-HUS in our study. The etiological differences of the disease might be related to racial differences between the Asian population and Caucasians.…”

Section: Discussioncontrasting

confidence: 42%

Clinicopathological Characteristics and Outcome of Chinese Patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome: A 9-Year Retrospective Study

Zhang

Shi²,

Ren³

et al. 2009

Nephron Clin Pract

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Background: The pathogenesis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is unclear and the prognosis is poor. Few studies have been published focusing on Chinese patients with TTP-HUS. We performed a retrospective study on the clinical characteristics and outcome of Chinese patients with TTP-HUS. Method: Patients with TTP-HUS, admitted to our hospital from 1998 to 2006, were retrospectively analyzed. Results: There were 26 females and 6 males in our study. Fifteen patients had systemic lupus erythematosus (SLE)-associated TTP-HUS; 2 had pregnancy-associated TTP-HUS; 1 had antiphospholipid syndrome-associated TTP-HUS; 2 had drug-associated TTP-HUS; 4 had malignant angionephrosclerosis- associated TTP-HUS; 3 had vasculitis-associated TTP-HUS, and the remaining 5 had idiopathic TTP-HUS. Twenty-six patients had acute kidney injury and 21 had nephrotic syndrome. Hypertension was found in 31 patients. For the treatment, 15 patients had plasmapheresis, 12 had continuous veno-venous hemodiafiltration and 14 had hemodialysis. Eighteen patients were treated with intravenous immunoglobulin. Corticosteroids were used in patients with idiopathic TTP-HUS. For the patients with SLE-associated TTP-HUS, corticosteroids and immunosuppressant were used. Outcome was poor: 6 patients died; 17 recovered from renal insufficiency; 5 progressed to chronic renal failure, and 4 were dependent on hemodialysis. Conclusions: Most of our patients had secondary TTP-HUS. SLE-associated TTP-HUS is the most common form of TTP-HUS. Early diagnosis and treatment can improve prognosis. An immunosuppressant together with corticosteroids could improve prognosis in some patients.

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Section: Discussioncontrasting

confidence: 42%

Clinicopathological Characteristics and Outcome of Chinese Patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome: A 9-Year Retrospective Study

Zhang

Shi²,

Ren³

et al. 2009

Nephron Clin Pract

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“…As this was not our primary endpoint, the study was likely underpowered to detect such a difference. Nevertheless, it is important to consider that although the acute episode of HUS resolves in most cases with supportive therapy, late complications including hypertension, proteinuria, and chronic kidney disease may occur years after the acute episode of HUS has subsided [9][10][11][12][13]. Much of the morbidity associated with HUS involves platelet entrapment and the formation of microthrombi in many organs including the kidney, central nervous system, and intestine among others [14].…”

Section: Discussionmentioning

confidence: 99%

Bleeding risk for surgical dialysis procedures in children with hemolytic uremic syndrome

2010

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Children with hemolytic uremic syndrome (HUS) frequently develop acute kidney injury (AKI) requiring renal replacement therapy (RRT). Peritoneal dialysis (PD) is commonly used. Despite high rates of thrombocytopenia, there is concern that platelet transfusions may worsen HUS by exacerbating microthrombi formation. We evaluated bleeding risk for PD catheter placement with or without central venous catheter (CVC) placement in children with HUS. Records from 1998 to 2007 were searched. Data regarding patient demographics, PD catheter placement, CVC placement, occurrence of procedure-associated bleeding, and time from insertion to removal of PD catheter were collected. Patients were stratified according to those who received and those who did not receive platelet transfusions. Seventy-three patients were identified. Twenty-two (30%) patients received platelet transfusion while 51 (70%) did not. Mean preoperative platelet counts were 37,600+/-21,900/mm(3) in patients receiving transfusions and 64,800 +/- 38,800/mm(3) in patients not receiving transfusions. Sixty-seven children (92%) also underwent CVC placement. There were no bleeding complications related to these procedures in either group. No differences in time to removal of the PD catheter were detected. Although caution and sound clinical judgment must be exercised, our findings suggest that PD catheter and CVC placement can be accomplished safely in most children with HUS, without need for platelet transfusion in spite of the associated thrombocytopenia.

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“…This syndrome is associated with severe clinical manifestations, a tendency to recur, and, until recently, a poor long-term prognosis (Noris & Remuzzi, 2009). With conventional supportive therapy, the expected rate of graft failure after kidney transplantation due to recurrent aHUS among patients with a mutation in a complement gene ranges from 50% to 80% within the first five years post transplant (Le Quintrec et al, 2013;Zimmerhackl, Scheiring, Prufer, Taylor, & Loirat, 2007).…”

Section: Introductionmentioning

confidence: 99%

Preemptive Use of Eculizumab for Living-Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome

Akchurin¹,

Dogra²,

Kaskel³

et al. 2016

EJBM

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Eculizumab is an anti-complement C5 monoclonal antibody that has recently been reported as an effective therapy for atypical hemolytic uremic syndrome. However, few data are available on the preemptive use of this medication in pediatric kidney transplantation. This report describes a successful preemptive use of eculizumab in combination with living unrelated kidney transplanta- tion in a 10-year-old child with end-stage renal disease secondary to atypical hemolytic uremic syndrome who has a complement factor H mutation that has not been previously reported. Further observations and clinical trials are required to address the challenges and areas of uncertainty related to preemptive eculizumab therapy for kidney transplantation in children and adults with atypical hemolytic uremic syndrome.

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Renal transplantation in HUS patients with disorders of complement regulation

Cited by 34 publications

References 25 publications

Clinicopathological Characteristics and Outcome of Chinese Patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome: A 9-Year Retrospective Study

Clinicopathological Characteristics and Outcome of Chinese Patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome: A 9-Year Retrospective Study

Bleeding risk for surgical dialysis procedures in children with hemolytic uremic syndrome

Preemptive Use of Eculizumab for Living-Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome

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