J Korean Soc Pediatr Endocrinol.  2011 Apr;16(1):56-60. 10.6065/jkspe.2011.16.1.56.

A Case of Turner Syndrome Associated with Idiopathic Central Diabetes Insipidus

• Kang B
• Sung HU
• Kim BK
• Park SY
• Kim SK
• Kwon YS
• Lim MK
• Lee JE

• KMID: 1450625
• DOI: http://doi.org/10.6065/jkspe.2011.16.1.56

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.

Keyword

Turner syndrome; Diabetes insipidus, neurogenic

MeSH Terms

Child
Deamino Arginine Vasopressin
Diabetes Insipidus, Neurogenic
Diabetes Mellitus
Follow-Up Studies
Humans
Magnetic Resonance Imaging
Pituitary Gland
Pituitary Gland, Posterior
Polydipsia
Polyuria
Turner Syndrome
Water Deprivation
Deamino Arginine Vasopressin