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KMID : 0648420110160010056
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Journal of Korean Society of Pediatric Endocrinology
2011 Volume.16 No. 1 p.56 ~ p.60
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A Case of Turner Syndrome Associated with Idiopathic Central Diabetes Insipidus
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Kang Ben
Sung Hyeon-U
Kim Bok-Ki
Park Sin-Young
Kim Soon-Ki
Kwon Young-Se
Lim Myung-Kwan
Lee Ji-Eun
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Abstract
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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.
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KEYWORD
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Turner syndrome, Diabetes insipidus, neurogenic
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