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Neurofibromatosis-Noonan syndrome

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Description

A variant of neurofibromatosis type 1 characterized by the combination of features of neurofibromatosis type 1, such as café-au-lait spots, iris Lisch nodules, axillary and inguinal freckling, optic nerve glioma and multiple neurofibromas; and Noonan syndrome, with features such as short stature, typical facial features, congenital heart defects and unusual pectus deformity.

SNOMEDCT_US

  • Mode of Inheritance

  • Autosomal dominant inheritance

VARIANTS

200

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Genes

External Links

  • OMIM

    601321

  • Orphanet

    638

  • HPO
  • Medgen

    C2931482

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