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KMID : 0384119950150030353
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Korean Journal of Clinical Pathology
1995 Volume.15 No. 3 p.353 ~ p.357
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Chediak - Higashi syndrome -Report of a case-
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김수영
한경자/정대철/감학기/굼한모/심상인
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Abstract
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We report 23-month-old boy with Chediak -Higashi syndrome (CHS) characterized by recurrent respiratory tract infection, focal hyper- and hypopigmentation of skin, hepatosplenomegaly, lymphadenopathy, pancytopenia. The posterior pole of retina
showed
marked hypopigmentation on fundus examination. Light microscopy of the hyperpigmented skin from the thigh showed many large sized melanin pigments throughout the epidermis and several melanophages in the upper dermis. Pathognomonic giant
peroxidase
-
positive granules in most granule containing cells were found. The abnormal granules were very large and have variable election density on electron microscopy. It appeared to be arised from fusion of primary and secondary granules. The child
responded
to the treatment with antibioties and ascorbic acid(Korean J Clin Pathol 1995;15(3) : 353~7).
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KEYWORD
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