Becoming a nonagenarian: factors associated with survival up to 90 years old in 70+ men and women. Results from the PAQUID longitudinal cohort.

OBJECTIVES: To identify factors associated with survival to the age of 90 years old in 70+ elderly people. DESIGN: The PAQUID prospective cohort on brain and functional ageing. SETTING: 75 randomly selected administrative communities in Gironde and Dordogne (France). PARTICIPANTS: A sub-sample of 2,578 community dwellers aged 70 years and over at baseline in 1988 and followed-up over 20 years, all participants of the PAQUID study. MEASUREMENTS: Data on socio-material environments, lifestyle, health, perceived health, and family background were collected at home every 2-3 years over 20 years, with a prospective update of vital status. Participants were compared according to their survival status (subjects who reached 90 compared to those who did not). The factors associated with survival were investigated separately for men and women by Cox regression with, as much as possible, time-dependent variables. RESULTS: Some factors associated with survival were common to both genders, whereas some others appeared gender specific. For men, tenant status (HR=1.46), former or current smoking (HR=1.17), disability (respective HR of 1.50, 1.78 and 2.81 for mild, moderate and severe level), dementia (HR=1.51), a recent hospitalisation (HR=1.32), dyspnoea (HR=1.32), and cardiovascular symptoms (HR=1.15) were associated with lower chance of becoming nonagenarian. Conversely, regular physical activity (HR=0.74) was associated with higher chance of survival. For women, the presence of a professional help (HR=1.19), living arrangements (HR=1.29 and HR=1.33), disability (respective HR of 1.55, 1.95 and 2.70 for mild, moderate and severe disability), dementia (HR=1.54), a recent hospitalisation (HR=1.19), diabetes (HR=1.49), and dyspnoea (HR=1.20) were associated with lower chance of becoming nonagenarian. Conversely, satisfaction of level income (HR=0.87), comfortable housing (HR=0.81), length of living in the dwelling (HR=0.80 upper to 6 years), regular physical activity (HR=0.89) and a medium (HR=0.79) or good (HR=0.68) subjective health, were associated with higher chance of becoming nonagenarian. CONCLUSION: Our findings confirm that survival up to 90 is a multifactorial phenomenon with similarities and specificities by gender. Consequently, primary prevention and global consideration of ageing (social, material, financial, psychological) are necessary to promote not only longevity but also successful ageing in order to face the future societal challenges due to demographic ageing.

[Genetics of retinitis pigmentosa: metabolic classification and phenotype/genotype correlations]. / Génétique des rétinites pigmentaires: classification métabolique et corrélations phénotype/génotype.

Retinitis pigmentosa (RP, prevalence 1/4000) is a set of hereditary retinal dystrophies characterized by pigment deposits in fundus and progressive death of photoreceptors, always associated with the alteration of retinal pigment epithelium. Genetic heterogeneity of the typical nonsyndromic form (rod cone dystrophy) is extensive: 11 genes and one locus were reported for autosomal dominant RP, 17 genes and five loci for autosomal recessive RP, and two genes and two loci for X-linked RP. A survey of mutation screening reports in large series of patients indicates that the frequency of mutations for all cloned genes varies from 40% to 54% of cases in autosomal dominant RP, from 17% to 24% in autosomal recessive RP (excluding the USH2A gene for which the values remain uncertain) and from 61% to 89% in X-linked RP. Very few studies report on sporadic cases except for the two X-linked genes, RP2 and RPGR, which account for 29% of sporadic cases in males. Altogether, the two most frequently involved genes are RPGR (13% of all RP cases) and RHO (4%), an important consideration for molecular diagnosis. Finally, we roughly estimate that currently known genes do not represent more than 50% of RP cases, suggesting that many genes remain to be discovered. The known genes can be classified into metabolic groups according to the encoded protein: visual transduction, visual cycle, transcription factors, structural proteins, spliceosome complex and cellular traffic, indicating the high level of specialization of photoreceptors and of the retinal pigment epithelium. In parallel with this classification, genotype/phenotype correlations have been established that will help ophthalmologists to suspect particular genes, and thereby mechanisms. This approach will provide better informations to patients and will orient the choice of future therapies.