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The Upper Limb Cardiovascular Syndrome

Andrew K. Poznanski, MD; Aaron Stern, MD; John Gall Jr., MD
Am J Dis Child. 1973;125(4):622. doi:10.1001/archpedi.1973.04160040116026.
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To the Editor.  —The recent paper of Brans and Lintermans on "The Upper Limb Cardiovascular Syndrome" in the November issue (124:779-783, 1972) leaves certain misconceptions about the limb manifestations of the condition and about the definition of this syndrome which we felt should be corrected.The authors stress the usefulness of the sign of absence of the carpal bones in this condition. This sign is actually of little value, as it represents simply a delay of skeletal maturation. Some delay in maturation is expected in these children, particularly in those that have severe cardiac problems. In an attempt to prove that this occurs in a large majority of cases, the authors use some unfortunate examples: They mention one of Holmes's cases—a four-year-old boy who had absence of the lunate, triquetrum, trapezium, and trapezoid. According to Garn's tables1 for white American children, the 5th through 95th percentile range for


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