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A Case of Neurofibromatosis Combined with Ectopic Kidney

Korean Journal of Pediatrics 2005;48(4):448-452.
Published online April 15, 2005.
A Case of Neurofibromatosis Combined with Ectopic Kidney
Eugene Yang, Bo Hyun Kim, Joon Soo Lee
Department of Pediatrics, Handicapped Children's Institute, Yonsei University, College of Medicine, Seoul, Korea
이소성 신장을 동반한 신경섬유종 1례
양유진, 김보현, 이준수
연세대학교 의과대학 소아과학교실, 연세대학교 장애아동 연구소
Joon Soo Lee, Email: joonsl96@yumc.yonsei.ac.kr
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.
Key Words: Neurofibromatosis , Ectopic kidney

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