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Pediatric Nephrology

, Volume 33, Issue 9, pp 1531–1538 | Cite as

Proliferative glomerulonephritis with monoclonal IgG deposits in children and young adults

  • Guolan Xing
  • Robert Gillespie
  • Badreldin Bedri
  • Albert Quan
  • Pingchuan Zhang
  • Xin J. Zhou
Original Article

Abstract

Background

Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age.

Methods

The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved. Light microscopy specimens and immunofluorescence and electron microscopy images were revisited. Clinical data and kidney biopsy findings for patients under the age of 20 were recorded.

Results

Five (0.78%) of a total of 637 patients younger than 20 with native renal biopsies had a diagnosis of PGNMID, including three males and two females with an average age of 14 years old (range 10–19). All five patients presented with microscopic hematuria and proteinuria. Three patients were nephrotic and their C3 levels were low. All five cases showed a membranoproliferative pattern with abundant mesangial and subendothelial monoclonal IgG3 deposits (3 κ and 2 λ light chain, respectively). The patients were followed up to 56 months. Two patients had re-biopsies 28 and 18 months after initial diagnosis and both showed similar morphologic changes. Various treatments were attempted including prednisone, mycophenolate mofetil, tacrolimus, rituximab, and eculizmab, with mixed responses.

Conclusions

PGNMID does occur in children and young adults. Membranoproliferative glomerulonephritis pattern with monoclonal IgG3 deposits is a common feature. Despite various immunosuppressive treatments, the disease appears slowly progressive.

Keywords

Kidney biopsy Glomerulonephritis Children Histopathology Membranoproliferative glomerulonephritis 

Notes

Compliance with ethical standards

The study was approved by the Institutional Review Board of Baylor University Medical Center at Dallas and performed in accordance with the Declaration of Helsinki.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© IPNA 2018

Authors and Affiliations

  1. 1.Department of NephrologyThe First Affiliated Hospital of Zhengzhou UniversityZhengzhouChina
  2. 2.Department of NephrologyCook Children’s Medical CenterFort WorthUSA
  3. 3.Children’s Renal CenterMedical City Children’s HospitalDallasUSA
  4. 4.Renal Path Diagnostics at Pathologists BioMedical LaboratoriesLewisvilleUSA
  5. 5.Department of PathologyBaylor University Medical CenterDallasUSA

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