A Case of Glomerulonephritis Associated with Klinefelter' Syndrome

Jin Hyuk Cho; Yeoung Sin Sin; Eun Hee Sim; Min Gyu Park; Dong Heo; Hak Rim

doi:10.7180/kmj.2012.27.2.177

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Cho, Sin, Sim, Park, Heo, and Rim: A Case of Glomerulonephritis Associated with Klinefelter' Syndrome

Case Report

Kosin Medical Journal

Kosin Medical Journal 2012; 27(2): 177-180.

Published online: 27 December 2012

DOI: https://doi.org/10.7180/kmj.2012.27.2.177

A Case of Glomerulonephritis Associated with Klinefelter' Syndrome

Jin Hyuk Cho¹, Yeoung Sin Sin¹, Eun Hee Sim¹, Min Gyu Park¹, Dong Heo¹, Hak Rim²

¹Department of Internal Medecine, Dae Dong Hospital, Busan, Korea.

²Department of Nephrology, College of Medicine, Kosin University, Gospel Hospital, Busan, Korea.

Corresponding Author: Jin Hyuck Cho, Department of internal medecine, Dae Dong Hospital, Myeongnyun-dong, Dongnae-gu, Busan 607-711, Korea. TEL: +82-10-5334-7508, FAX: +82-51-553-7575, happy2639@hanmail.net

Received 14 June 2012 Revised 25 July 2012 Accepted 5 August 2012

Abstract

Klinefelter' syndrome is a disorder of sexual differentiation in males, characterized by the presence of two or more X-chromosomes, hypogonadism, and lack of secondary sexual characteristics. The association between Klinefelter' syndrome and glomerulonephritis has been reported, while cases of glomerulonephritis associated with Klinefelter' syndrome are rare. We report the Korean case: a 31-year-old man with Klinefelter' syndrome who developed glomerulonephritis. The patient's urine analysis shows microscopic hematuria and the result of kidney biopsy was minimal change disease. The onset and course of his disease might have been influenced by the sex hormone imbalance.

Keywords: Glomerulonephritis, Klinefelter' syndrome, Minimal change disease

Figures and Tables

Fig. 1

The patient's karyotype is compatible with Klinefelter' syndrome.

Fig. 2

Light microsccopic finding of kidney biopsy shows no definite abnormal finding except mild increase of mesangeal matrix (× 200, H & E staining).

Fig. 3

Electron microscopic finding of kidney biospy shows focal effacement of foot process.

References

1. Smyth CM, Bremner WJ. Klinefelter syndrome. Arch Intern Med. 1998. 158:1309–1314.

2. Geschwind DH, Boone KB, Miller BL, Swerdloff RS. Neurobehavioral phenotype of Klinfelter syndrome. Ment Retard Dev Disabil Res Rev. 2000. 6:107–116.

3. Takeuchi Y, Murata Y, Sintani J, Masukawa H, Nakamura R, Oi K, et al. Klinefelter's syndrome accompanied by mixed connective tissue disease and diabetes mellitus. Intern Med. 1999. 38:875–881.

4. Aoki N. Klinefelter' syndrome, autoimmunity associated endocrinopathies. Intern Med. 1999. 38:838–839.

5. Carlotti AP, Franco PB, Elias LL, Facincani I, Costa EL, Foss N, et al. Glucocorticoid receptors, in vitro steroid sensitivity, and cytokine secretion in idiopathic nephrotic syndrome. Kidney Int. 2004. 65:403–408.

6. Friedman A, Waksman Y. Sex hormones and autoimmunity. Isr J Med Sci. 1997. 33:254–257.

7. Cutolo M, Balleari E, Giusti M, Intra E, Accardo S. Androgen replacement theraphy in male patients with rheumatoid arthritis. Arthritis Rheum. 1991. 34:1–5.

8. Zinn AR, Ramos P, Elder FF, Kowal K, Samango-Sprouse C, Ross JL. Androgen receptor CAGn repeat length influences phenotype of 47,XXY (klinefelter) syndrome. J Clin Endocrinol metab. 2005. 90:5041–5046.

9. Ichii O, Konno A, Sasaki N, Endoh D, Hashimoto Y, Kon Y. Onset of autoimmune glomerulonephritis derived from the telomeric region of MRL-chromosome 1 is associated with the male sex hormone in mice. Lupus. 2009. 18:491–500.

10. Xu Q, Wells CC, Garman JH, Asico L, Escano CS, Maric C. Imbalance in sex hormone levels exacerbates diabetic renal disease. Hypertension. 2008. 51:1218–1224.

11. Swerdlow AJ, Higgins CD, Schoemaker MJ, Wright AF, Jacobs PA. United Kingdom Clinical Cytogenetics Group. Mortality in patients with klinefelter syndrome in britain: a cohort study. J Clin Endocrinol Metab. 2005. 90:6516–6522.

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