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이소성 신장을 동반한 신경섬유종 1례

Other Titles
 A Case of Neurofibromatosis Combined with Ectopic Kidney 
Authors
 양유진  ;  김보현  ;  이준수 
Citation
 Korean Journal of Pediatrics, Vol.48(4) : 448-452, 2005 
Journal Title
 Korean Journal of Pediatrics 
ISSN
 1738-1061 
Issue Date
2005
MeSH
Neurofibromatosis ; Ectopic kidney
Keywords
Neurofibromatosis ; Ectopic kidney
Abstract
Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.
Files in This Item:
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DOI
OAK-2005-03821
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Lee, Joon Soo(이준수) ORCID logo https://orcid.org/0000-0001-9036-9343
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/150897
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