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Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy

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Abstract

Background

Dense deposit disease (DDD) (also known as membranoproliferative glomerulonephritis type II) in childhood is a rare glomerulonephritis with frequent progression to end-stage renal disease (ESRD) and a high recurrence after kidney transplantation. The pathophysiologic basis of DDD is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade.

Case-diagnosis/treatment

A 14-year-old girl presented with edema and nephrotic range proteinuria. Blood tests showed hypoalbuminemia, nephrotic range proteinuria, normal renal function, and a low C3 level. Renal biopsy confirmed the diagnosis of crescentic DDD. Complement analysis revealed strong AP activation (low C3), positive C3 nephritic factor (C3NeF), and a decreased complement factor H (CFH) levels with CFH polymorphisms. Therapy with eculizumab was considered after the failure of corticosteroid and plasmapheresis to modulate the ongoing massive proteinuria and persistence of low serum C3 levels. There was a marked clinical and biochemical response following the administration of eculizumab.

Conclusions

Our case emphasizes the efficacy of eculizumab in the management of crescentic DDD in a patient with a normal renal function, in a short follow-up period. Considering previously reported cases, it appears that eculizumab represents a promising new approach which may prevent progression to ESRD in a subset of patients with DDD.

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Authors and Affiliations

  1. Pediatric Nephrology Department, Ondokuz Mayis University Faculty of Medicine, Kurupelit, Samsun, Turkey

    Ozan Ozkaya, Hulya Nalcacioglu, Demet Tekcan & Gurkan Genc

  2. Pathology Department, Ondokuz Mayis University Faculty of Medicine, Kurupelit, Samsun, Turkey

    Bilge Can Meydan

  3. Pathology Department, Baskent University Faculty of Medicine, Bahçelievler, Ankara, Turkey

    B. Handan Ozdemir

  4. Pediatric Cardiology Department, Ondokuz Mayis University Faculty of Medicine, Kurupelit, Samsun, Turkey

    M. Kemal Baysal

  5. Cardiovascular Surgery Department, Ondokuz Mayis University Faculty of Medicine, Kurupelit, Samsun, Turkey

    Hasan Tahsin Keceligil

Authors
  1. Ozan Ozkaya
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  2. Hulya Nalcacioglu
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  3. Demet Tekcan
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  4. Gurkan Genc
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  5. Bilge Can Meydan
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  6. B. Handan Ozdemir
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  7. M. Kemal Baysal
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  8. Hasan Tahsin Keceligil
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Corresponding author

Correspondence to Hulya Nalcacioglu.

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Ozkaya, O., Nalcacioglu, H., Tekcan, D. et al. Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy. Pediatr Nephrol 29, 1283–1287 (2014). https://doi.org/10.1007/s00467-013-2748-5

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  • DOI: https://doi.org/10.1007/s00467-013-2748-5

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