A case of focal segmental glomerulosclerosis in an adult patient with hypogammaglobulinemia superimposed on membranoproliferative glomerulonephritis in childhood

Kenji Tsuji, Haruhito Adam Uchida, Tetsuichirou Ono, Tatsuyuki Inoue, Katsuji Shinagawa, Shinji Kitamura, Yohei Maeshima, Hitoshi Sugiyama, Hirofumi Makino

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Abstract

Background: Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia without a known predisposing cause. Case presentation: We report a 36-year-old man who had suffered membranoproliferative glomerulonephritis (MPGN) in his childhood, later diagnosed with CVID at 35 years of age. He presented at our hospital with signs of proteinuria. A renal biopsy revealed he suffered from focal segmental glomerulosclerosis (FSGS), possibly due to obesity and hypertension, not CVID - associated MPGN. Conclusion: This is the first case report of FSGS in a CVID patient. In this case, we have to pay attention not only to the treatment of obesity and hypertension for FSGS but also to the recurrence of immune-complex glomerulonephritis such as MPGN, in case of the restoration of hypogammaglobulinemia.

Original languageEnglish
Article number46
JournalBMC Nephrology
Volume13
Issue number1
DOIs
Publication statusPublished - 2012

Fingerprint

Common Variable Immunodeficiency
Membranoproliferative Glomerulonephritis
Focal Segmental Glomerulosclerosis
Agammaglobulinemia
Obesity
Hypertension
Glomerulonephritis
Antigen-Antibody Complex
Proteinuria
Kidney
Biopsy
Recurrence

Keywords

  • Focal segmental glomerulosclerosis
  • Hypogammaglobulinemia
  • Proteinuria

ASJC Scopus subject areas

  • Nephrology

Cite this

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title = "A case of focal segmental glomerulosclerosis in an adult patient with hypogammaglobulinemia superimposed on membranoproliferative glomerulonephritis in childhood",
abstract = "Background: Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia without a known predisposing cause. Case presentation: We report a 36-year-old man who had suffered membranoproliferative glomerulonephritis (MPGN) in his childhood, later diagnosed with CVID at 35 years of age. He presented at our hospital with signs of proteinuria. A renal biopsy revealed he suffered from focal segmental glomerulosclerosis (FSGS), possibly due to obesity and hypertension, not CVID - associated MPGN. Conclusion: This is the first case report of FSGS in a CVID patient. In this case, we have to pay attention not only to the treatment of obesity and hypertension for FSGS but also to the recurrence of immune-complex glomerulonephritis such as MPGN, in case of the restoration of hypogammaglobulinemia.",
keywords = "Focal segmental glomerulosclerosis, Hypogammaglobulinemia, Proteinuria",
author = "Kenji Tsuji and Uchida, {Haruhito Adam} and Tetsuichirou Ono and Tatsuyuki Inoue and Katsuji Shinagawa and Shinji Kitamura and Yohei Maeshima and Hitoshi Sugiyama and Hirofumi Makino",
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AU - Tsuji, Kenji

AU - Uchida, Haruhito Adam

AU - Ono, Tetsuichirou

AU - Inoue, Tatsuyuki

AU - Shinagawa, Katsuji

AU - Kitamura, Shinji

AU - Maeshima, Yohei

AU - Sugiyama, Hitoshi

AU - Makino, Hirofumi

PY - 2012

Y1 - 2012

N2 - Background: Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia without a known predisposing cause. Case presentation: We report a 36-year-old man who had suffered membranoproliferative glomerulonephritis (MPGN) in his childhood, later diagnosed with CVID at 35 years of age. He presented at our hospital with signs of proteinuria. A renal biopsy revealed he suffered from focal segmental glomerulosclerosis (FSGS), possibly due to obesity and hypertension, not CVID - associated MPGN. Conclusion: This is the first case report of FSGS in a CVID patient. In this case, we have to pay attention not only to the treatment of obesity and hypertension for FSGS but also to the recurrence of immune-complex glomerulonephritis such as MPGN, in case of the restoration of hypogammaglobulinemia.

AB - Background: Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia without a known predisposing cause. Case presentation: We report a 36-year-old man who had suffered membranoproliferative glomerulonephritis (MPGN) in his childhood, later diagnosed with CVID at 35 years of age. He presented at our hospital with signs of proteinuria. A renal biopsy revealed he suffered from focal segmental glomerulosclerosis (FSGS), possibly due to obesity and hypertension, not CVID - associated MPGN. Conclusion: This is the first case report of FSGS in a CVID patient. In this case, we have to pay attention not only to the treatment of obesity and hypertension for FSGS but also to the recurrence of immune-complex glomerulonephritis such as MPGN, in case of the restoration of hypogammaglobulinemia.

KW - Focal segmental glomerulosclerosis

KW - Hypogammaglobulinemia

KW - Proteinuria

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