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A life table for onset of Huntington's chorea
Author(s) -
NEWCOMBE R. G.
Publication year - 1981
Publication title -
annals of human genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.537
H-Index - 77
eISSN - 1469-1809
pISSN - 0003-4800
DOI - 10.1111/j.1469-1809.1981.tb00351.x
Subject(s) - chorea , huntington's disease , heterozygote advantage , age of onset , medicine , pediatrics , genetics , biology , disease , allele , gene
SUMMARY Accurate quantitative assessment of risk in Huntington's chorea necessitates unbiased estimation of the distribution of age at onset. Previous studies have quoted the mean observed age at onset but this is biased, containing no contribution from heterozygotes who have not manifested the disorder by the study date. An iterative weighted life‐table approach is developed, which takes due account of such censored heterozygotes; it demonstrates a considerably older median age at onset than has hitherto been reported.