To the Editor
In a recent evaluation of several individuals with Sotos syndrome we discovered a typographical error in our article entitled “Metacarpophalangeal Pattern Profile Analysis in Sotos Syndrome” in Volume 20:625–629, 1985 of the American Journal of Medical Genetics. The legend of Figure 2 (p. 628) presently reads “D = −2.24 + 0.92(X8) + 0.41(X11) + 0.24(age in years)”; it should read “−2.24 − 0.92(X8) + 0.41(X11) + 0.24(age in years).” Therefore only the corrected discriminant equation should be used in the evaluation of possible Sotos syndrome individuals.
It has been brought to our attention that the discriminant equation if applied to an adult individual with Sotos syndrome (> 18 years of age) may produce an inaccurate classification of the individual. Subjects in our control and Sotos syndrome samples were below the age of 18 years; thus the equation was generated with subadults. If adults are analyzed, then one should assign a maximum age of 18 years in the calculation with the discriminant equation. This minor modification should allow for the use of the discriminant equation with adults as well as subadults.
In order to test the power of the discriminant equation to identify Sotos syndrome individuals, we have analyzed hand x-rays from 17 additional patients (kindly provided by Dr. P.F. Dijkstra). Fifteen of 17 Sotos syndrome individuals (88%) in our test sample were classified correctly based on the original discriminant equation from the 16 Sotos syndrome individuals. Research is in progress in order to develop a more accurate discriminant equation for diagnostic purposes. We would encourage the use of this equation by others to determine its usefulness for diagnosis of Sotos syndrome individuals.
Footnotes
Edited by John M. Opitz
Contributor Information
Merlin G. Butler, Division of Generics, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee
F. John Meaney, Genetic Diseases Section, Indiana State Board of Health, Indianapolis; Department of Medical Genetics, Indiana University School of Medicine.