Journal List > J Korean Endocr Soc > v.22(4) > 1003374

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Ahn, Bae, Kim, Lee, Shon, and Jung: A Case of Idiopathic Central Diabetes Insipidus together with Primary Empty Sella and Combined Pituitary Hormone Deficiency
Case Report
Journal of Korean Endocrine Society

Journal of Korean Endocrine Society 2007; 22(4): 272-276.

Published online: 20 August 2007

DOI: https://doi.org/10.3803/jkes.2007.22.4.272

A Case of Idiopathic Central Diabetes Insipidus together with Primary Empty Sella and Combined Pituitary Hormone Deficiency

Sun Young Ahn, Kyu Hwan Bae, Myung Hwan Kim, Ji Hyun Lee, Ho Sang Shon, Eui Dal Jung

Department of Internal Medicine, Catholic University of Daegu School of Medicine, Korea.

Received 28 February 2007       Accepted 25 May 2007

Copyright © 2007 Korean Endocrine Society

Abstract

Central diabetes insipidus is a heterogeneous condition that is characterized by polyuria and polydipsia, and this is due to a deficiency of arginine vasopressin. Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. Genetic abnormalities in the homeobox genes have recently been shown, on sellar magnetic resonance imaging, to be associated with combined pituitary hormone deficiency with pituitary defect. We report here on a 44-year-old female who suffered from polydipsia, polyuria and primary amenorrhea since childhood. She was diagnosed with idiopathic central diabetes insipidus together with primary empty sella and combined pituitary hormone deficiency. On the genetic analysis, she was proven to have a point mutation of the PROP-1 gene, which is known as a cause of combined pituitary hormone deficiency.

Keywords: Central diabetes insipidus, Combined pituitary hormone deficiency, Primary empty sella

Figures and Tables

Fig. 1
Simple X-ray of skull lateral view (A) shows no enlarged sella and hand (B) shows epiphyseal plate fusion.
jkes-22-272-g001
Fig. 2
Sella MRI T-1 weighted coronal (A) and sagittal (B) images of the case show pituitary fossa filled with cerebrospinal fluid without definite focal pituitary mass lesion and show atrophied pituitary stalk.
jkes-22-272-g002
Table 1
Combined pituitary function test
jkes-22-272-i001
Table 2
Changes of serum and urine osmolarity using desmopressin spray
jkes-22-272-i002

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