TY - JOUR
T1 - Inheritance of the mitral valve prolapse syndrome. Discussion of a three-dimensional penetrance model
AU - Strahan, Nancy V.
AU - Murphy, Edmond A.
AU - Fortuin, Nicholas J.
AU - Come, Patricia C.
AU - Humphries, J. O.Neal
PY - 1983/6
Y1 - 1983/6
N2 - The families of 12 probands with classic mural valve prolapse were studied for evidence of mitral valve prolapse. Seventy parents, sibs, and progeny were included in the analysis. Forty-seven percent (16 of 34) of progeny were affected compared with 30 percent (3 of 10) of parents. Thirty-eight percent (10 of 26) of sibs were affected. A three-compartmental penetrance model was devised to account for the variation in expression with age. This includes a latent stage (time before onset of signs), an affected stage, and a stage in which the subjects are withdrawn (because of treatment, regression, or death). The implications of this model are discussed.
AB - The families of 12 probands with classic mural valve prolapse were studied for evidence of mitral valve prolapse. Seventy parents, sibs, and progeny were included in the analysis. Forty-seven percent (16 of 34) of progeny were affected compared with 30 percent (3 of 10) of parents. Thirty-eight percent (10 of 26) of sibs were affected. A three-compartmental penetrance model was devised to account for the variation in expression with age. This includes a latent stage (time before onset of signs), an affected stage, and a stage in which the subjects are withdrawn (because of treatment, regression, or death). The implications of this model are discussed.
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U2 - 10.1016/0002-9343(83)90791-X
DO - 10.1016/0002-9343(83)90791-X
M3 - Article
C2 - 6859065
AN - SCOPUS:0020631801
VL - 74
SP - 967
EP - 972
JO - American Journal of Medicine
JF - American Journal of Medicine
SN - 0002-9343
IS - 6
ER -