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December 1970

Dermatoglyphics in the Holt-Oram Syndrome

Author Affiliations

Brooklyn, NY

From the divisions of hematology (Dr. Rosner) and neurology (Dr. Aberfeld), Department of Medicine, Maimonides Medical Center, Brooklyn, NY. Dr. Rosner is now with the Queens Hospital Center, Jamaica, NY.

Arch Intern Med. 1970;126(6):1010-1013. doi:10.1001/archinte.1970.00310120072010

In 1960, Holt and Oram1 described a syndrome consisting of atrial septic defects, bizarre cardiac arrhythmias, and abnormalities of the upper extremity, particularly the thumb, in numerous members of a family covering at least four generations. One year later, McKusick2 mentioned a mother and daughter with this syndrome and suggested the name atriodigital dysplasia. In 1963, Zetterqvist3 reported a third family, mother and son in whom atrial septal defects, bizarre arrhythmias, and the characteristic thumb anomalies were seen. In the same year, Kuhn et al4 described a fourth family with this syndrome. Three additional pedigrees were reported by Pruzanski,5 Lewis et al,6 and Emerit et al7 in 1964. The following year, Holmes8 described two pedigrees and Emerit et al9 reported five additional cases, four of whom had cardiac anomalies other than interatrial septal defects and no cardiac arrhythmias. In 1966, a very extensive pedigree of the Holt-Oram syndrome with detailed

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