Limits...
Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome.

Durán CE, Blasco M, Maduell F, Campistol JM - Clin Kidney J (2012)

Bottom Line: The latter is called atypical haemolytic-uraemic syndrome (aHUS).Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation.The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis.

View Article: PubMed Central - PubMed

Affiliation: Nephrology and Urology Clinical Institute (ICNU), Hospital Clinic, University of Barcelona, Barcelona, Spain and Hospital Clinic, Institut d'investigacions Biomèdiques August Pi i Suñer (IDIBAPS), University of Barcelona, Barcelona, Spain.

ABSTRACT
Haemolytic-uraemic syndrome is a clinical syndrome characterized by thrombocytopaenia, non-autoimmune haemolytic anaemia and renal impairment. Pathological alterations in kidney samples show thrombotic microangiopathy. The underlying pathogenesis is endothelial cell injury with thrombotic occlusion of the arterioles and capillaries. A variety of causes have been identified, associated with infection of Escherichia coli O157:H7, environmental factors as immunosuppressive drugs and genetic deficiencies in complement regulatory factors. The latter is called atypical haemolytic-uraemic syndrome (aHUS). Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation. The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis.

No MeSH data available.


Related in: MedlinePlus

Ten months evolution of renal function after eculizumab initiation.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4400444&req=5

fig1: Ten months evolution of renal function after eculizumab initiation.

Mentions: One month later, the patient was re-admitted to hospital because of uraemic syndrome, with a serum creatinine concentration of 6.4 mg/dL, LDH 1100 UI/dL, haemoglobin 7.6 g/dL and platelet count 105 000/mm3. He admitted sporadic cocaine consumption. A new renal biopsy confirmed the severity of the thrombotic microangiopathy with glomerular capillary thrombosis and Grade I chronic allograft nephropathy. The patient started chronic dialysis (5 × weeks), and treatment with eculizumab (900 mg weekly for 4 weeks followed by 1200 mg every other week thereafter until now) was initiated. Anti-meningococcal vaccination was administered before the first dose of eculizumab. Renal function was followed up with an isotopic renogram. Two months after starting dialysis and eculizumab treatment, the patient reported a progressive increase in diuresis. Isotope studies confirmed a marked and progressive improvement in renal parameters. Dialysis was gradually reduced, and 3.5 months after restarting dialysis, the patient ceased replacement therapy with a serum creatinine concentration of 5 mg/dL [creatinine clearance (CrCl) 12 mL/min]. Renal function progressively improved, with a current serum creatinine concentration of 2.6 mg/dL (CrCl 35 mL/min), the patient being in good health (Figure 1). Eculizumab treatment (1200 mg per intravenous/2 weeks) has been maintained with good tolerance. No haematological parameters of aHUS have been detected in the last 10 months. Strict control of blood/urine cocaine has been negative.


Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome.

Durán CE, Blasco M, Maduell F, Campistol JM - Clin Kidney J (2012)

Ten months evolution of renal function after eculizumab initiation.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400444&req=5

fig1: Ten months evolution of renal function after eculizumab initiation.
Mentions: One month later, the patient was re-admitted to hospital because of uraemic syndrome, with a serum creatinine concentration of 6.4 mg/dL, LDH 1100 UI/dL, haemoglobin 7.6 g/dL and platelet count 105 000/mm3. He admitted sporadic cocaine consumption. A new renal biopsy confirmed the severity of the thrombotic microangiopathy with glomerular capillary thrombosis and Grade I chronic allograft nephropathy. The patient started chronic dialysis (5 × weeks), and treatment with eculizumab (900 mg weekly for 4 weeks followed by 1200 mg every other week thereafter until now) was initiated. Anti-meningococcal vaccination was administered before the first dose of eculizumab. Renal function was followed up with an isotopic renogram. Two months after starting dialysis and eculizumab treatment, the patient reported a progressive increase in diuresis. Isotope studies confirmed a marked and progressive improvement in renal parameters. Dialysis was gradually reduced, and 3.5 months after restarting dialysis, the patient ceased replacement therapy with a serum creatinine concentration of 5 mg/dL [creatinine clearance (CrCl) 12 mL/min]. Renal function progressively improved, with a current serum creatinine concentration of 2.6 mg/dL (CrCl 35 mL/min), the patient being in good health (Figure 1). Eculizumab treatment (1200 mg per intravenous/2 weeks) has been maintained with good tolerance. No haematological parameters of aHUS have been detected in the last 10 months. Strict control of blood/urine cocaine has been negative.

Bottom Line: The latter is called atypical haemolytic-uraemic syndrome (aHUS).Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation.The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis.

View Article: PubMed Central - PubMed

Affiliation: Nephrology and Urology Clinical Institute (ICNU), Hospital Clinic, University of Barcelona, Barcelona, Spain and Hospital Clinic, Institut d'investigacions Biomèdiques August Pi i Suñer (IDIBAPS), University of Barcelona, Barcelona, Spain.

ABSTRACT
Haemolytic-uraemic syndrome is a clinical syndrome characterized by thrombocytopaenia, non-autoimmune haemolytic anaemia and renal impairment. Pathological alterations in kidney samples show thrombotic microangiopathy. The underlying pathogenesis is endothelial cell injury with thrombotic occlusion of the arterioles and capillaries. A variety of causes have been identified, associated with infection of Escherichia coli O157:H7, environmental factors as immunosuppressive drugs and genetic deficiencies in complement regulatory factors. The latter is called atypical haemolytic-uraemic syndrome (aHUS). Here, we present a patient with severe aHUS with complement factor H deficiency triggered by cocaine use and recurrence after kidney transplantation. The patient restarted haemodialysis for severe renal insufficiency and anti-C5 antibody eculizumab was used as salvage treatment with progressive recovery of graft function and suppression of dialysis.

No MeSH data available.


Related in: MedlinePlus