A Case of Turner Syndrome Associated with Idiopathic Central Diabetes Insipidus.

Kang,  Ben; Sung,  Hyeoun U; Kim,  Bok Ki; Park,  Sin Young; Kim,  Soon Ki; Kwon,  Young Se; Lim,  Myung Kwan; Lee,  Ji Eun

doi:2011.16.1.56

Ann Pediatr Endocrinol Metab > Volume 16(1); 2011 > Article

Case Report

Journal of Korean Society of Pediatric Endocrinology 2011;16(1): 56-60.

DOI: https://doi.org/10.6065/jkspe.2011.16.1.56

A Case of Turner Syndrome Associated with Idiopathic Central Diabetes Insipidus.

Ben Kang, Hyeoun U Sung, Bok Ki Kim, Sin Young Park, Soon Ki Kim, Young Se Kwon, Myung Kwan Lim, Ji Eun Lee

¹Department of Pediatrics, Inha University School of Medicine, Incheon, Korea. anicca@inha.ac.kr
²Department of Radiology, Inha University School of Medicine, Incheon, Korea.

Abstract

We report a case of Turner syndrome associated with idiopathic central diabetes insipidus in a 12-year-old girl, who presented with polyuria and polydipsia after a year. The patient was very short and and centrally obese, and was initially diagnosed with Turner syndrome, hyperlipidema, and diabetes mellitus. A water deprivation test revealed central diabetes insipidus, and sellar magnetic resonance imaging (MRI) showed a thickening of the pituitary stalk, with normal high signal intensity in the posterior pituitary gland. Replacement therapy with desmopressin was initiated, and follow-up sellar MRI findings after two years showed spontaneous regression of the thickened pituitary stalk. There are only few reports of concomitant Turner syndrome with central diabetes insipidus worldwide. Further observation is needed in order to disclose the cause of central diabetes insipidus in patients having Turner syndrome.

Keywords: Turner syndrome;Diabetes insipidus, neurogenic