Brackenridge C J
J Med Genet. 1978 Feb;15(1):23-6. doi: 10.1136/jmg.15.1.23.
The time interval between the first appearance of signs in the transmitting parent and the birth of the subsequently affected child has been shown by Brackenridge and Telscher (1975) to influence the age at onset of Huntington's disease. The cirticism by Burke (1976) that the interval factor offers no predictive advantage over parental onset age is refuted. The advantage of small sibship sizes in familial correlation studies is noted and an equation to estimate onset age is derived to control for ascertainment bias. The interval factor is shown to surpass parental onset age as a determinant of offspring onset age. When applied to Queensland material, reasonable agreement is obtained between predicted and reported onset ages. Evidence for the desirability for parents at risk who intend to have families to plan them early is discussed.
布雷肯里奇和特尔舍尔（1975年）指出，在患有亨廷顿舞蹈症的亲代首次出现症状与随后受影响孩子出生之间的时间间隔，会影响亨廷顿舞蹈症的发病年龄。伯克（1976年）批评称，时间间隔因素相比亲代发病年龄并无预测优势，这一批评遭到了驳斥。文中指出了在家族相关性研究中较小同胞规模的优势，并推导出一个估算发病年龄的公式以控制确诊偏倚。研究表明，时间间隔因素作为后代发病年龄的决定因素，其作用超过了亲代发病年龄。将该公式应用于昆士兰的数据时，预测发病年龄与报告发病年龄之间取得了合理的一致性。文中还讨论了有患病风险的父母打算生育时尽早进行规划的必要性。
