Ectrodactyly and Germany's eugenics law of 14 July 1933

TY - JOUR

T1 - Ectrodactyly and Germany's eugenics law of 14 July 1933

AU - Busch, Roland

AU - Kjær, Klaus Wilbrandt

N1 - Copyright 2002 Wiley-Liss, Inc.

PY - 2002

Y1 - 2002

N2 - The family reported herein serves as a genetically and historically important vignette on the issues of nonpenetrance (versus germinal mosaicism) in nonsyndromic autosomal dominant ectrodactyly and the Eugenics Law of Germany of 14 July 1933, which was used to coerce the sterilization of the propositus despite infertility in his first marriage. In a sibship of seven children (with normal parents), three boys were affected. The propositus (adoptive grandfather of the author) was the patient of Paul Leopold Friedrich and Georg Perthes, who published their observations on the propositus. Except for an adopted daughter, the propositus was childless. His two affected brothers each had an affected child, and the father- to son transmission confirmed the hypothesis of autosomal dominant inheritance. The issue of nonpenetrance versus germinal mosaicism in ectrodactyly was debated by Auerbach [1956:Ann Hum Genet 20:266-269] and Vogel [1958:Ann Hum Genet 22:132-137], and remains unresolved.

AB - The family reported herein serves as a genetically and historically important vignette on the issues of nonpenetrance (versus germinal mosaicism) in nonsyndromic autosomal dominant ectrodactyly and the Eugenics Law of Germany of 14 July 1933, which was used to coerce the sterilization of the propositus despite infertility in his first marriage. In a sibship of seven children (with normal parents), three boys were affected. The propositus (adoptive grandfather of the author) was the patient of Paul Leopold Friedrich and Georg Perthes, who published their observations on the propositus. Except for an adopted daughter, the propositus was childless. His two affected brothers each had an affected child, and the father- to son transmission confirmed the hypothesis of autosomal dominant inheritance. The issue of nonpenetrance versus germinal mosaicism in ectrodactyly was debated by Auerbach [1956:Ann Hum Genet 20:266-269] and Vogel [1958:Ann Hum Genet 22:132-137], and remains unresolved.

KW - Eugenics

KW - Family Health

KW - Female

KW - Fingers

KW - Germany

KW - Hand Deformities, Congenital

KW - History, 20th Century

KW - Humans

KW - Male

KW - Pedigree

U2 - 10.1002/ajmg.10313

DO - 10.1002/ajmg.10313

M3 - Journal article

C2 - 12116260

SN - 1552-4868

VL - 110

SP - 184

EP - 190

JO - American Journal of Medical Genetics. Part C: Seminars in Medical Genetics

JF - American Journal of Medical Genetics. Part C: Seminars in Medical Genetics

IS - 2

ER -