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Dyskeratosis congenita. Report of a large kindred

This kindred includes six males with dyskeratosis congenita. It is the largest British pedigree so far reported and brings the total number of reported cases to fifty‐nine. Our pedigree supports X‐linked recessive inheritance and close linkage with the Xg2 locus was excluded. Three previously unreported complications are noted; Hodgkin's disease, adenocarcinoma of the pancreas and deafness. Normal chromosomal stability was found in three patients and immunological studies precluded an early universal defect in cell‐mediated immunity.