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HOME > Kosin Med J > Volume 30(1); 2015 > Article
Case Report
A Case of Focal Segmental Glomerulosclerosis in Turner Syndrome
A Rum Han1, Young Ki Lee1, Hyun Yon Jung1, Jae Hyun Park1, Jung-Woo Noh1, Eun Suk Nam2
Kosin Medical Journal 2015;30(1):69-72.
DOI: https://doi.org/10.7180/kmj.2015.30.1.69
Published online: January 20, 2015

1Department of Internal Medicine, and Hallym Kidney Research Institute Hallym University College of Medicine, Seoul, Korea

2Department of Pathology, Hallym University College of Medicine, Seoul, Korea

Corresponding Author：Young Ki Lee, Department of Internal Medicine, Kangnam Sacred Heart Hospital, Hallym University, 1, Singil-ro, Yeongdeungpo-gu, Seoul, 150-950, Korea TEL: +82-2-829-5214 FAX: +82-2-846-4669 E-mail: km2071@unitel.co.kr
• Received: February 13, 2014   • Accepted: April 23, 2014

Copyright © 2015 Kosin University School of Medicine Proceedings

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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  • Turner syndrome is usually accompanied with various anomalies. Congenital urological and renal abnormalities are often associated with this syndrome. The occurrence of glomerulonephritis is uncommon. An 18-year-old woman showed fatigue and profound proteinuria. She had been diagnosed with Turner syndrome in her age of 15. The kidney biopsy specimen examined by light microscopy, immunofluorescence and electron microscopic examination revealed focal segmental glomerulosclerosis. This is the first case report of focal segmental glomerulosclerosis in turner syndrome in South Korea.
  • Keywords: Focal Segmental; Glomerulosclerosis; Proteinuria; Turner syndrome
Figure 1.
Jone-silver stain reveals segmental sclerosis in two glomeruli and tubular atrophy and interstitial foam cells(A, x200). Electron microscopy displays diffuse effacement of foot processes. There is no electron dense material deposit(B).
kmj-30-69f1.jpg
Table 1.
Six cases of Turner syndrome with glomerulonephritis
No Year Karyotype Age (y) Presenting symptoms Serum Cr (mg/dL) Serum albumin (g/dL) Proteinuria (g/day) Diagnosis Renal recovery Reference
1 1982 46X, del(X) (p 11) 12 Hematuria, Pyuria 0.5 3.5 1.58 MPGN no 4
2 1989 46XY/ 45, X 18 Amenorrhea, Edema 0.4 2.6 9.6 FSGS no 5
3 1998 45, X 14 Proteinuria, Hematuria 1.1 4.8 1.205 FSGS no 6
4 2004 – 13 Proteinuria, Arthralgia 0.3 3.7 2.3 MN yes 7
5 2013 45, X 11 Proteinuria, Hematuria normal     MN yes 8
6 2014 46, X, der(X) del(X)(p10) del(X)(q25) 18 Proteinuria, Fatigue 0.81 3.7 3.6 FSGS yes  

MPGN, membranoproliferative glomerulonephritis; FSGS, focal segmental glomerulosclerosis; MN, membranous nephropathy.

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  • 9. Deegens JK, Dijkman HB, Borm GF, Steenbergen EJ, van den Berg JG, Weening JJ, et al. Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis. Kidney Int 2008;74:1568–76.ArticlePubMed
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    • Renal Problems in Early Adult Patients with Turner Syndrome
      Dong Uk Yu, Jae Kyun Ku, Woo Yeong Chung
      Childhood Kidney Diseases.2015; 19(2): 154.     CrossRef

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      A Case of Focal Segmental Glomerulosclerosis in Turner Syndrome
      Kosin Med J. 2015;30(1):69-72.   Published online January 20, 2015
      DOI: https://doi.org/10.7180/kmj.2015.30.1.69
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    A Case of Focal Segmental Glomerulosclerosis in Turner Syndrome
    Image
    Figure 1. Jone-silver stain reveals segmental sclerosis in two glomeruli and tubular atrophy and interstitial foam cells(A, x200). Electron microscopy displays diffuse effacement of foot processes. There is no electron dense material deposit(B).

    Figure 1.

    A Case of Focal Segmental Glomerulosclerosis in Turner Syndrome

    Six cases of Turner syndrome with glomerulonephritis

    No Year Karyotype Age (y) Presenting symptoms Serum Cr (mg/dL) Serum albumin (g/dL) Proteinuria (g/day) Diagnosis Renal recovery Reference
    1 1982 46X, del(X) (p 11) 12 Hematuria, Pyuria 0.5 3.5 1.58 MPGN no 4
    2 1989 46XY/ 45, X 18 Amenorrhea, Edema 0.4 2.6 9.6 FSGS no 5
    3 1998 45, X 14 Proteinuria, Hematuria 1.1 4.8 1.205 FSGS no 6
    4 2004 – 13 Proteinuria, Arthralgia 0.3 3.7 2.3 MN yes 7
    5 2013 45, X 11 Proteinuria, Hematuria normal     MN yes 8
    6 2014 46, X, der(X) del(X)(p10) del(X)(q25) 18 Proteinuria, Fatigue 0.81 3.7 3.6 FSGS yes  
    Table 1. Six cases of Turner syndrome with glomerulonephritis

    MPGN, membranoproliferative glomerulonephritis; FSGS, focal segmental glomerulosclerosis; MN, membranous nephropathy.

    Table 1.

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