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February 1982

FG Syndrome

Author Affiliations

Department of Medical Genetics University of South Alabama College of Medicine 2451 Fillingim St Mobile, AL 36617

Am J Dis Child. 1982;136(2):176-177. doi:10.1001/archpedi.1982.03970380088027

Sir.—I read with great interest the report by Say and Meyer (Journal 1981;135:711-712) describing an X-linked syndrome of trigoncephaly, short stature, and developmental delay. I was struck by the similarity between these cases and those of the FG syndrome.

FG syndrome is an X-linked, recessive, multiple congenital anomaly described originally by Opitz and Kaveggia in 1974.1 Their very comprehensive report describes the typical findings of mental retardation, pleasant personality, seizures, hypotonia, anal anomalies, congenital heart disease, craniosynostosis, and delayed growth. Keller et al2 in 1976 reported cases of similarly affected boys. These were readily accepted as additional cases after this possibility was brought up by Opitz.3 Only one of those three cases had anal abnormalities. The FG syndrome was further characterized by Riccardi et al4 in 1977 by the addition of five new cases from three families.

I think serious consideration should be given to

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