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KMID : 0391520100180010117
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Journal of the Korean Child Neurology Society
2010 Volume.18 No. 1 p.117 ~ p.122
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A Case of Silver-Russell Syndrome Diagnosed in the Patient Admitted with Hypoglycemic Seizure
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Lee Jung-Yong
Kim Young-A
Lee Kyung-Yeon
Oh Ki-Won
Kim Ja-Hyeong
Kim Joon-Sung
Jeong Jin-Young
Park Sang-Kyu
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Abstract
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The Silver-Russell syndrome(SRS) is a clinically heterogeneous syndrome characterized by intrauterine and postnatal growth retardation with spared cranial growth, characteristic facial features, and body asymmetry. Although mild to moderate hypoglycemic symptoms occasionally appear in children with SRS especially those who are not fed frequently and regularly, hypoglycemic seizures rarely occur. We report a rare case of SRS which was diagnosed in a 4-year-old female who admitted with hypoglycemic seizure. The patient showed the characteristic features of SRS. Endocrinologic studies were normal except for partial growth hormone insufficiency. To prevent seizures and chronic neurologic deficits in children with SRS, the early recognition and appropriate management of hypoglycemia is critical.
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KEYWORD
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Silver-Russell syndrome, Hypoglycemia, Seizures
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