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December 1964

Infantile Hypertrophic Pyloric Stenosis In Four Siblings

Author Affiliations


Am J Dis Child. 1964;108(6):617-624. doi:10.1001/archpedi.1964.02090010619006

Infantile (or congenital) hypertrophic pyloric stenosis is a fairly common pediatric disease in the United States and Northern Europe, with an estimated frequency between one to three cases per 1,000 live births and a sex ratio of four or five to one favoring males.1-4 Up to a generation ago, the risk among siblings of affected children could be estimated, but not among their offspring, because of high infant mortality from the disease. With the introduction, in 1912, of pyloromyotomy as definitive treatment,5 increased numbers of affected individuals survive and produce families, permitting study of parents and children with the disease. Even so, familial occurrence is low, as shown by one author who, adding his own cases to those found in a search of the world literature, was able to list in some detail less than 120 families in which there were two or more verified instances of pyloric