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Literature record

Novel FGD1 mutation underlying Aarskog-Scott syndrome with myopathy and distal arthropathy.

PMID 23211637 | DOI 10.1097/MCD.0b013e32835b6dc4 · Clinical dysmorphology · 2013

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In this report, we describe a kindred consisting of five affected males presenting with many of the well-recognized features of Aarskog-Scott syndrome. The diagnosis, which was confirmed by the identification of a novel nonsense mutation of FGD1, was associated with the presence of a symmetric distal arthropathy with electromyographic signs of myopathy. These features should be considered in the evaluation of future patients.

Validated evidence

TypeEntitySource evidenceConfidenceExtractor
geneFGD1“Novel FGD1 mutation underlying Aarskog-Scott syndrome with myopathy and distal arthropathy.”0.98hgnc_dict_v1
phenotypecongenital myopathy“Novel FGD1 mutation underlying Aarskog-Scott syndrome with myopathy and distal arthropathy.”0.93phenotype_alias_lexicon_v2