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A Case of Neurofibromatosis Combined with Ectopic Kidney / 소아과
Korean Journal of Pediatrics ; : 448-452, 2005.
Artigo em Coreano | WPRIM | ID: wpr-148986
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.
Assuntos

Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Exame Físico / Manifestações Cutâneas / Neurofibromatose 1 / Neurofibromatoses / Síndromes Neurocutâneas / Diagnóstico / Placa Neural / Hipertensão Renovascular / Rim / Crista Neural Tipo de estudo: Estudo diagnóstico Limite: Criança / Feminino / Humanos Idioma: Coreano Revista: Korean Journal of Pediatrics Ano de publicação: 2005 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Exame Físico / Manifestações Cutâneas / Neurofibromatose 1 / Neurofibromatoses / Síndromes Neurocutâneas / Diagnóstico / Placa Neural / Hipertensão Renovascular / Rim / Crista Neural Tipo de estudo: Estudo diagnóstico Limite: Criança / Feminino / Humanos Idioma: Coreano Revista: Korean Journal of Pediatrics Ano de publicação: 2005 Tipo de documento: Artigo