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Neurofibromatosis-Noonan syndrome

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Disease definition

Neurofibromatosis-Noonan syndrome (NFNS) is a RASopathy and a variant of neurofibromatosis type 1 (NF1) characterized by the combination of features of NF1, such as café-au-lait spots, iris Lisch nodules, axillary and inguinal freckling, optic nerve glioma and multiple neurofibromas, and Noonan syndrome (NS), such as short stature, typical facial features (hypertelorism, ptosis, downslanting palpebral fissures, low-set posteriorly rotated ears with a thickened helix, and a broad forehead), congenital heart defects and unusual pectus deformity. As these three entities have significant phenotypic overlap, molecular genetic testing is often necessary for a correct diagnosis (such as when café-au-lait spots are present in patients diagnosed with NS).

ORPHA:638

Classification level: Disorder

Synonym(s):
  • NFNS
  • Neurofibromatosis type 1-Noonan syndrome

Source: PubMed ID 2411134 40289159

Prevalence: Unknown

Inheritance: Autosomal dominant

Age of onset: Infancy, Neonatal

ICD-10: Q87.1

ICD-11: LD2F.1Y

OMIM: 601321

UMLS: C2931482

MeSH: C537393

GARD: 372

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2019.pdf) - Orphanet Urgences
Clinical practice guidelines
Disease review articles
Clinical genetics review
English (2018) - GeneReviews
Genetic testing
Guidance for genetic testing
Deutsch (2015.pdf) - Kardiologe
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.