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KMID : 0367419940370091305
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Journal of Korean Pediatric Society
1994 Volume.37 No. 9 p.1305 ~ p.1311
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A Case of Severe Pituitary Dwarfism due to Agenesis of Anterior Pituitary Gland with Pituitary Stalk Transection
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유명주
한동기/신재훈/설인준/이승로
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Abstract
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We experienced one case of severe pituitary dwarfism in a 10 years old female girl.
Magnetic resonance image (MRI) revealed transection of the pituitary stalk stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland.
The serum growth Hormone (GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient had responses to TRH, normal TSH and partial prolactin response, respectively. There was not response LH and FSH to GnRH.
The morning cortisol concentration and serum T4 concentration were decreased below the normal range.
These findings and no hyperprolactinemia suggested the presence of a vascular connection between the pituitary gland and hypothalamus, which is not visible on MRI.
Sofar, the primary cause of idiopathic pituitary dwarfism in many patients is injury to hypothalamus by perinatal insults. In this patient, there was no history of perinatal insults and postnatal head trauma but transection of the pituitary
stalk.
We report a case of severe pituitary dwarfism due to agenesis with brief review of related litereature.
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