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I7 Reproductive behaviour of patients with huntington’s disease
  1. Elena Saifulliina,
  2. Rim Magzhanov
  1. Bashkir State Medical University, Department of Neurology, Neurosurgery, Medical Genetic, Ufa, Bashkortostan, Russia

Abstract

Background/Aim Huntington’s disease (HD) is a severe late-onset autosomal dominant neurodegenerative disorder. The goal of our research was the investigation of the features of the reproductive behaviour in HD patients.

Methods Fertility in 78 patients with HD (mean age 52 years; range = 27–80) and 85 healthy sibs (mean age 51 years; range = 25–80) that were under observation over the period between 1970 and 2000 was analysed.

Results Conjugality among the patients with HD constituted 89.7%, among their healthy sibs – 84.7% (p = 0.5). The early onset of the disease limited marrying and patients’ fertility in marriage. Mean age of the disease manifestation of the patients that were childless (29.5 ± 10.8 years) or the patients that had only one child (31.8 ± 9.8 years) differed from the age of the disease manifestation of the patients that had two or more children (40.4 ± 8.9 years; p < 0.05). The average number of live-birth infants in the families of the HD patients and their healthy sibs was not significantly different from each other (2.9 vs. 2.7; p = 0.2). The infant mortality rate in the families of the HD patients constituted 7.5% (in the families of the healthy sibs the infant mortality was not registered). The rate of childless marriages significantly exceeded among the families of the healthy sibs in comparison with the families of the HD patients (9.7% vs. 2.8%, p < 0.05).

Conclusion The disease did not impact significantly on the reproductive behaviour of patients in case of late-onset manifestation.

  • reproductive behaviour

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