A lethal autosomal recessive entero‐colitis of early infancy

A family is presented in which three of four children died with an almost identical syndrome. It started within a week or so of birth and presented as bloody diarrhea with a very swollen abdomen. All died after some weeks: autopsy revealed ulcerative colitis in two and pseudo‐membranous entero‐colitis in the third. The parents were second cousins There may have been a milk intolerance. The disease is apparently due to an autosomal recessive gene.