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Noonans syndrom kan diagnosticeres klinisk og molekylaergenetisk
Engelsk titel: Noonan syndrome can be diagnosed clinically and through molecular genetic analyses Läs online Författare: Krab Henningsen, Marie ; Jelsig, Anne Marie ; Andersen, Helle ; Brusgaard, Klaus ; Ousager, Lilian Bomme ; Hertz, Jens Michael Språk: Dan Antal referenser: 30 Dokumenttyp: Översikt UI-nummer: 16087726

Tidskrift

Ugeskrift for Laeger 2016;178(10)940-4 ISSN 0041-5782 E-ISSN 1603-6824 KIBs bestånd av denna tidskrift Denna tidskrift är expertgranskad (Peer-Reviewed)

Sammanfattning

Noonan syndrome is part of the group of RASopathies caused by germline mutations in genes involved in the RAS/MAPK pathway. There is substantial phenotypic overlap among the RASopathies. Diagnosis of Noonan syndrome is often based on clinical features including dysmorphic facial features, short stature and congenital heart disease. Rapid advances in sequencing technology have made molecular genetic analyses a helpful tool in diagnosing and distinguishing Noonan syndrome from other RASopathies.