| Autor: |
Carbonell, C. Cavero, Páramo-Rodríguez, L., Moreno-Marro, S., Barrachina-Bonet, L., De la Natividad, M., Guardiola-Vilarroig, S., Zurriaga, O. |
| Předmět: |
|
| Zdroj: |
European Journal of Public Health; 2021 Supplement, Vol. 31, piii428-iii428, 1/3p |
| Abstrakt: |
Background: Huntington Disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by involuntary choreic movements and behavioural/psychiatric disorders, which compromises the quality of life. Objective: To identify the distribution of HD by sex, age and province in the Valencian Region (VR) and to determine its prevalence and mortality rate. Methods: A cross-sectional epidemiological study was performed. HD’s cases between 2010-2018 were identified from the Rare Disease Information System of the VR by selecting codes 333.4 (ICD9) and G10 (ICD10). A descriptive analysis was performed by country of birth, province of residence and sex. Median ages at diagnosis and at death, with interquartile range (IQR), and prevalence and mortality rate, with 95% confidence intervals (95%CI), were obtained. Results: 225 cases were identified: 50.2% women, 52.0% residents in the southernmost province, 92.9% born in Spain and 68.9% confirmed by clinical manifestations. Median age at diagnosis was 54.1 years (IQR:41.1-66.1): 54.7 (IQR:41.2-66.1) in men and 53.0 (IQR:40.5-65.8) in women. Prevalence in 2018 was 2.0/100000 inhabitants (95%CI:0.4-2.4), being higher in women [2.2 (95%CI:0.3-2.8)] than in men [1.8 (95%CI:0.3- 2.3)]. Even an increasing trend was observed, significant differences weren’t found. 49.8% of cases died during the study, being 51.8% men. The median age at death was 62.7 years (IQR:51.1-74.2), 61.1 (IQR:48.7-72.5) in men and 66.6 (IQR:53.3-76.8) in women. The mortality rate in 2018 in both sexes was 0.3/100000 inhabitants (95%CI:0.3-2.3), significant differences weren’t found. Conclusions: Most of confirmations at registry level were based on clinical manifestations, reinforcing that HD has a characteristic pattern of symptoms although most are unspecific. The slight difference between sexes in the median age of diagnosis was significantly increased in the age of death, with men being the group with the highest mortality and an earlier death. Key messages: In the Valencian Region of Spain, men with Huntington Disease have a higher mortality rate and a lower median age at death (5.5 years earlier) than women. There is a pattern of symptoms which is characteristic of Huntington Disease, although most of them are unspecific. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
|
