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Hepatorenal tyrosinemia1 is a severe inborn error of metabolism that can affect numerous organs, particularly the liver, kidneys, and peripheral nerves. In the first accounts of patients with features typical of tyrosinemia in the 1950s, almost all died of liver disease in infancy and childhood [1]. However, tyrosinemia is highly variable and rare case reports described surviving affected adults. Since the identification of tyrosinemia, its clinical course has been improved successively by the introduction of diet therapy, neonatal screening, liver transplantation and treatment with nitisinone (NTBC, 2-(2-nitro-4-trifluoromethyl benzoyl)-1,3-cyclohexanedione) [2]. Tyrosinemia raises questions in liver biology, biochemical and population genetics, cell biology, oncology, and public health.
Whereas Dr Burney's writings are often mentioned in studies on eighteenth-century music, not much interest seems to have been given specifically to his relation to the organ, which played an important part in his professional career as a practising musician. No better introduction to the aesthetic ethos of the eighteenth-century English organ can be found than in Burney's remarks disseminated in his various writings. Taken together, they construct a coherent discourse on taste and constitute an aesthetic. Burney's view of the organ is indicative of a broader ethos of moderation that permeates his whole work, and is at one with the dominant moral philosophy of Georgian England. This conception is ripe with patriotic undertones, while it also articulates a constant plea for politeness as a condition for harmonious social interaction. He believed that moderation, simplicity, and fancy were the constituents of good taste as well as good manners.
The present study aims at measuring the association between household food insecurity and psychological distress in adolescents in Inuit communities, concurrently and overtime from childhood to adolescence.
Design:
The study used measures of internalising behaviours (anxiety, withdrawn attitude, somatic complaints and depression) as indicators of psychological distress during adolescence, a concurrent measure of household food insecurity in adolescence and an assessment of longitudinal patterns of household food insecurity from childhood to adolescence. We collected descriptive information at birth, childhood and adolescence on potential confounders.
Setting:
Inuit communities of Nunavik in northern Quebec, Canada
Participants:
The study consisted of 212 participants from the Nunavik Child Development Study, who have been assessed at birth, childhood (mean age = 11 years, range = 9–13 years) and adolescence (mean age = 18 years, range = 16–21 years).
Results:
Concurrent severe household food insecurity in adolescence was associated with higher measures of psychological distress: depression (βstd = 0·26, P < 0·01) and withdrawn attitude (βstd = 0·20, P = 0·04). Persistent household food insecurity (both at childhood and adolescence) was associated with higher levels of adolescent depression (βstd = 0·18, P = 0·02) and anxiety (βstd = 0·17, P = 0·03).
Conclusions:
Adolescents from Nunavik living with higher food insecurity and those having experienced food insecurity in both childhood and adolescence were more likely to report symptoms of psychological distress. Considering the high level of distress experienced by young Inuit, existing initiatives to reduce food insecurity in Nunavik communities should be targeted to include children and adolescents.
The age-at-onset (AAO) of Parkinson’s disease (PD) is thought to be influenced by environmental factors and polygenic predispositions. Professional exposures to pesticides and toxic metals were shown to be associated with an earlier onset in small sample studies.
Aim of Study:
The aim of this study was to confirm the association between professional exposures to pesticides and toxic metals and the AAO of PD, on a larger cohort of patients, defined with a clinic-based ascertainment scheme.
Methods:
We used an incident cohort of 290 patients recruited through three designated movement disorder clinics in the province of Quebec, Canada. Patients completed a detailed questionnaire regarding professional exposures to pesticides and toxic metals. We compared the AAO in patients without prior professional exposure (N = 170) and those with exposure to pesticides (N = 53) or toxic metals through welding (N = 30). We further subdivided patients exposed to pesticides according to the frequency and proximity of their contacts.
Results:
Patients with prior exposure to pesticides (AAO = 54.74 years) or toxic metals (54.27 years) had a significantly earlier AAO compared to the control group (59.26 years) (p = 0.003). In those exposed to pesticides, closer (p = 0.03) and more frequent (p = 0.02) contacts were negatively correlated with AAO.
Conclusion:
Exposure to pesticides and toxic metals were both associated with an earlier onset of PD, an effect that was greater with higher levels of exposure, both in terms of frequency and proximity.