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KMID : 0371219930150010075
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Medical Bulletin of the Presbyterian Medical Center
1993 Volume.15 No. 1 p.75 ~ p.82
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Pendred's Syndrome -A report of 5 cases-
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박윤규
이삼열/오성수/정을삼
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Abstract
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The historical cornerstone of Pendred's syndrome is derived from Vaughan Pendred's description of two sisters with goiter and congenital deafness in 1986. Patient with this syndrome has variable degrees of congenital sensoryneural deafness,
goiter,
hypothyroidism and positive perchlorate discharge test
Iodine organification defect in the synthesis of thyroid hormone has been thought to be the major cause of hypothyroidism and goiter in Pendred's syndrome. The real biochemical abnormality responsible for the iodine organification is still
unknown
and
the etiology of sensoryneural hearing loss is unclear, too.
For the first time in the Korean Surgical Society, authors presented 5 cases of Pendred's syndrome experienced at the department of Surgery, Presbyterian Medical Center, Korea form January 1990 to Feburary, 1992.
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KEYWORD
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