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Eculizumab in atypical haemolytic-uraemic syndrome allows cessation of plasma exchange and dialysis.

Kim JJ, Waller SC, Reid CJ - Clin Kidney J (2012)

Bottom Line: Eculizumab is emerging as an effective treatment for post-transplant aHUS recurrence and may have a role in treating de novo aHUS, halting the haemolytic process.In this case report, we describe the fourth case of aHUS treated with eculizumab.In our patient, with a known complement Factor H mutation, not only has the disease process become quiescent but also this therapy has led to significantly improved renal function so that dialysis is no longer necessary.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatric Nephrology, Evelina Children's Hospital, London, UK.

ABSTRACT
Disorders in complement regulation are a major cause of atypical haemolytic-uraemic syndrome (aHUS). Eculizumab, a monoclonal antibody targeting complement C5 and blocking the terminal complement cascade, should theoretically be useful in this disease, particularly when associated with specific complement pathway anomalies such as Factor H deficiency. Eculizumab is emerging as an effective treatment for post-transplant aHUS recurrence and may have a role in treating de novo aHUS, halting the haemolytic process. In this case report, we describe the fourth case of aHUS treated with eculizumab. In our patient, with a known complement Factor H mutation, not only has the disease process become quiescent but also this therapy has led to significantly improved renal function so that dialysis is no longer necessary.

No MeSH data available.


Related in: MedlinePlus

Clinical progress. Each thin arrow denotes a plasma exchange session (1 volume exchange with 5% albumin and 10 ml/kg virion inactivated plasma). Thick arrows denote eculizumab infusions. Vertical line represents the first eculizumab infusion. Horizontal dotted lines represent normal ranges for haemoglobin (bottom) and platelets (top) respectively. Time on haemodialysis (HD) and peritoneal dialysis (PD) represented by double arrowed lines.
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fig1: Clinical progress. Each thin arrow denotes a plasma exchange session (1 volume exchange with 5% albumin and 10 ml/kg virion inactivated plasma). Thick arrows denote eculizumab infusions. Vertical line represents the first eculizumab infusion. Horizontal dotted lines represent normal ranges for haemoglobin (bottom) and platelets (top) respectively. Time on haemodialysis (HD) and peritoneal dialysis (PD) represented by double arrowed lines.

Mentions: Peritoneal dialysis was commenced to manage her renal failure. Attempts at weaning PEX were unsuccessful due to haematological relapses (Figure 1). She required nine packed red blood cell transfusions in 4 months and blood priming for PEX sessions. She had three exacerbations of HUS following infectious illnesses despite regular PEX.


Eculizumab in atypical haemolytic-uraemic syndrome allows cessation of plasma exchange and dialysis.

Kim JJ, Waller SC, Reid CJ - Clin Kidney J (2012)

Clinical progress. Each thin arrow denotes a plasma exchange session (1 volume exchange with 5% albumin and 10 ml/kg virion inactivated plasma). Thick arrows denote eculizumab infusions. Vertical line represents the first eculizumab infusion. Horizontal dotted lines represent normal ranges for haemoglobin (bottom) and platelets (top) respectively. Time on haemodialysis (HD) and peritoneal dialysis (PD) represented by double arrowed lines.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400463&req=5

fig1: Clinical progress. Each thin arrow denotes a plasma exchange session (1 volume exchange with 5% albumin and 10 ml/kg virion inactivated plasma). Thick arrows denote eculizumab infusions. Vertical line represents the first eculizumab infusion. Horizontal dotted lines represent normal ranges for haemoglobin (bottom) and platelets (top) respectively. Time on haemodialysis (HD) and peritoneal dialysis (PD) represented by double arrowed lines.
Mentions: Peritoneal dialysis was commenced to manage her renal failure. Attempts at weaning PEX were unsuccessful due to haematological relapses (Figure 1). She required nine packed red blood cell transfusions in 4 months and blood priming for PEX sessions. She had three exacerbations of HUS following infectious illnesses despite regular PEX.

Bottom Line: Eculizumab is emerging as an effective treatment for post-transplant aHUS recurrence and may have a role in treating de novo aHUS, halting the haemolytic process.In this case report, we describe the fourth case of aHUS treated with eculizumab.In our patient, with a known complement Factor H mutation, not only has the disease process become quiescent but also this therapy has led to significantly improved renal function so that dialysis is no longer necessary.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatric Nephrology, Evelina Children's Hospital, London, UK.

ABSTRACT
Disorders in complement regulation are a major cause of atypical haemolytic-uraemic syndrome (aHUS). Eculizumab, a monoclonal antibody targeting complement C5 and blocking the terminal complement cascade, should theoretically be useful in this disease, particularly when associated with specific complement pathway anomalies such as Factor H deficiency. Eculizumab is emerging as an effective treatment for post-transplant aHUS recurrence and may have a role in treating de novo aHUS, halting the haemolytic process. In this case report, we describe the fourth case of aHUS treated with eculizumab. In our patient, with a known complement Factor H mutation, not only has the disease process become quiescent but also this therapy has led to significantly improved renal function so that dialysis is no longer necessary.

No MeSH data available.


Related in: MedlinePlus