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Han, Lee, Jung, Park, Noh, and Nam: A Case of Focal Segmental Glomerulosclerosis in Turner Syndrome
Case Report

Kosin Medical Journal 2015;30(1):69-72.

Published online: 20 January 2015

DOI: https://doi.org/10.7180/kmj.2015.30.1.69

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

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 13 February 201430 March 2014       Accepted 23 April 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.

Abstract

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

References

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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.tif
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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