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Early treatment with eculizumab in atypical haemolytic uraemic syndrome.

Garjau M, Azancot M, Ramos R, Sánchez-Corral P, Montero MA, Serón D - Clin Kidney J (2012)

Bottom Line: Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis.Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation.These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.

View Article: PubMed Central - PubMed

Affiliation: Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.

ABSTRACT
Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.

No MeSH data available.


Related in: MedlinePlus

Renal arteriolar wall thickening, mild interstitial fibrosis and tubular atrophy areas.
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fig1: Renal arteriolar wall thickening, mild interstitial fibrosis and tubular atrophy areas.

Mentions: Renal biopsy (Figure 1) conducted the second week after admission revealed occlusive thrombosis in several small- and medium-sized arteries with secondary ischaemic glomerular changes. There was extensive acute tubular necrosis in the renal tubules with focal areas of tubular atrophy. The interstitium showed moderate fibrosis and mild chronic inflammatory infiltrate. Direct immunofluorescence was negative. A second renal biopsy (Figure 2) performed 2.5 months after admission, and 3 weeks before starting eculizumab, showed changes similar to those observed at the first biopsy: thrombotic microangiopathic lesions persisted and there was deterioration of interstitial fibrosis and tubular atrophy.


Early treatment with eculizumab in atypical haemolytic uraemic syndrome.

Garjau M, Azancot M, Ramos R, Sánchez-Corral P, Montero MA, Serón D - Clin Kidney J (2012)

Renal arteriolar wall thickening, mild interstitial fibrosis and tubular atrophy areas.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400458&req=5

fig1: Renal arteriolar wall thickening, mild interstitial fibrosis and tubular atrophy areas.
Mentions: Renal biopsy (Figure 1) conducted the second week after admission revealed occlusive thrombosis in several small- and medium-sized arteries with secondary ischaemic glomerular changes. There was extensive acute tubular necrosis in the renal tubules with focal areas of tubular atrophy. The interstitium showed moderate fibrosis and mild chronic inflammatory infiltrate. Direct immunofluorescence was negative. A second renal biopsy (Figure 2) performed 2.5 months after admission, and 3 weeks before starting eculizumab, showed changes similar to those observed at the first biopsy: thrombotic microangiopathic lesions persisted and there was deterioration of interstitial fibrosis and tubular atrophy.

Bottom Line: Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis.Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation.These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.

View Article: PubMed Central - PubMed

Affiliation: Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.

ABSTRACT
Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.

No MeSH data available.


Related in: MedlinePlus