Endocrinol Metab > Volume 20(1); 2005 > Article
Journal of Korean Endocrine Society 2005;20(1):96-102.
DOI: https://doi.org/10.3803/jkes.2005.20.1.96    Published online February 1, 2005.
A Case of Kallmann's Syndrome with Unilateral Renal Aplasia and Diabetes Mellitus.
En Jung Lee, Sung Wook Hong, Yun Ki Hong, Ji Sung Yoon, Ji O Mok, Yeo Joo Kim, Hyeong Kyu Park, Chul Hee Kim, Sang Jin Kim, Dong Won Byun, Won Kyung Bae, Kyo Il Suh, Myung Hi Yoo
1Department of Internal Medicine, College of Medicine, Soonchunhyang University, Chunan, Korea.
2Department of Radiology, College of Medicine, Soonchunhyang University, Chunan, Korea.
Kallmann's syndrome is defined as the combination of hypogonadotropic hypogonadism and anosmia/hyposmia. The syndrome is a result of defect in the embryonic migratory pathway of gonadotropin-releasing hormone, which synthesizes neurons and olfactory axons. The hypogonadotropic hypogonadism results due to absence of or incomplete pubertal development and may be associated with anosmia, hyposmia, midline defect(color blindness, cleft-lip, cleft-palate, unilateral renal agenesis, sensorineural deafness), cryptorchidism and skeletal anomaly. Till date in Korea, few cases of Kallmann's syndrome have been reported but there are no available reports on cases of Kallmann's syndrome with unilateral renal aplasia and diabetes mellitus. We handled a case of Kallmann's syndrome associated with unilateral renal agenesis and diabetes mellitus. In the current work, we present a peculiar case as afore mentioned with the review of related literature.
Key Words: Kallmann's syndrome, Unilateral renal aplasia, Diabetes mellitus

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