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Dermatol. pediatr. latinoam. (Impr.) ; 7(1): 7-13, ene.-abr. 2009. ilus
Article in Spanish | LILACS | ID: lil-598141

ABSTRACT

La neurofibromatosis tipo 1 es la genodermatosis más común, heredada de manera autosómica dominante. Presenta una distribución mundial y no tiene predominio racial. Afecta fundamentalmente la piel, los ojos y los sistemas nervioso y esquelético. El presente trabajo consiste en una revisión sobre su patogénesis, manifestaciones clínicas, criterios diagnósticos, tratamiento y pronóstico.


Neurofibromatosis type 1 is the most common genodermatosis, inherited in an autosomal dominant way. It has a worldwide distribution and has no racial predilection. Skin, eyes, nervous system and bones are usually affected. The present work reviews its pathogenesis, clinical manifestations, diagnostic criteria, treatment and prognoses.


Subject(s)
Humans , Infant , Child, Preschool , Neurofibromatosis 1/diagnosis , Neurofibromatosis 1/etiology , Neurofibromatosis 1/physiopathology , Neurofibromatosis 1/pathology , Neurofibromatosis 1/therapy , Cafe-au-Lait Spots , Neurofibroma , Neurofibromin 1
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