Autosomal Dominant Inheritance in Cantu ´ Syndrome (Congenital Hypertrichosis, Osteochondrodysplasia, and Cardiomegaly) Brissia Lazalde, 1,2 Rocı ´o Sa ´ nchez-Urbina, 1,2 Ismael Nun ˜ o-Arana, 1,2 Wadih E. Bitar, 3 and Marı ´a de Lourdes Ramı ´rez-Duen ˜ as 1,2 * 1 Divisio ´n de Gene ´tica, Centro de Investigacio ´n Biome ´dica de Occidente, Jalisco, Me ´xico 2 Doctorado en Gene ´tica Humana, Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Jalisco, Me ´xico 3 Servicio de Traumatologı ´a y Ortopedia, Hospital de Especialidades, Centro Me ´dico Nacional de Occidente, Instituto Mexicano del Seguro Social, Jalisco, Me ´xico Cantu ´ syndrome (CS) is characterized by congenital hypertrichosis, osteochondro- dysplasia, cardiomegaly, and coarse facial appearance; autosomal recessive inheri- tance has been postulated. We report on a Mexican family with CS; the affected mem- bers are the 44-year-old father and his two children (a male and female), aged 14 and 4 years, respectively; each shows the classic characteristics, but the father and the brother also have a previously unreported feature, namely, a thick calvarium. This is the first reported instance of male-to-male transmission of CS. With the paternal age effect found in the reported sporadic cases and the segregation analysis [Robertson et al., 1999: Am J Med Genet 85:395–402], auto- somal dominant inheritance is more likely than autosomal recessive inheritance. The cases of affected sibs reported by Cantu ´ et al. [1982: Hum Genet 60:36–41] could be ex- plained by parental gonadal mosaicism. Am. J. Med. Genet. 94:421–427, 2000. © 2000 Wiley-Liss, Inc. KEY WORDS: hypertrichosis; osteochon- drodysplasia; cardiomegaly; autosomal dominant; pater- nal age effect; gonadal mosa- icism *Correspondence to: Ramı ´rez Duen ˜ as, Divisio ´n de Gene ´tica, Centro de Investigacio ´n Biome ´dica de Occidente, IMSS, apartado postal 5–35 Guadalajara Jalisco, Me ´xico. E-mail: ramirezl@udgserv.cencar.udg.mx Received 17 February 2000; Accepted 25 May 2000 Fig. 1. Family pedigree. American Journal of Medical Genetics 94:421–427 (2000) © 2000 Wiley-Liss, Inc.