The high rate of youth suicide in some First Nations villages of Northern Quebec is an important public health problem. Based on a six-year field study in three villages belonging to the Atikamekw and Anishinabe groups, this paper proposes changes in three areas of social policy that could contribute to prevention of youth suicide. These three areas are: youth protection, administration of justice, and housing. An argument is made first to adapt the youth protection law of Quebec and to give greater responsibility to communities in individual cases in order to prevent child placement outside the villages. Regarding the administration of justice, we suggest initiatives to encourage rapid prosecution of crimes on reserves and the adoption of an approach based on reconciliation between perpetrator and victim. Finally, we indicate how housing measures could help safeguard children's wellbeing given that overcrowding can contribute to suicide. The discussion also proposes that these three key changes in social policy could be relevant in other Aboriginal communities both within and outside of Quebec.
X-linked adrenoleukodystrophy is a peroxisomial disorder caused by mutations in the ABCD1 gene. Adrenomyeloneuropathy is the second most frequent phenotype (25-46%) of this disease and classically presents in adulthood with spastic paraparesis. Female heterozygotes can be symptomatic, but they are frequently misdiagnosed as having multiple sclerosis.
We report a novel missense mutation in the ABCD1 gene in a 47-year-old French-Canadian female with spastic paraparesis and no confirmed family history of X-linked adrenoleukodystrophy. The mutation is located on exon 1 and causes the amino acid substitution of a valine for an alanine in a region of the protein highly conserved between mouse and man.
Adrenomyeloneuropathy must be considered in the differential diagnosis of spastic paraparesis in men or women. This is an initial report of an ABCD1 gene mutation in the French-Canadian population, which should lead to the recognition of other cases in the future.
The WHO Child Growth Standards (CGS) which were recently adopted by the Canadian Pediatric Society were used to assess the relative size of Cree newborns.
Birth weight, length, and head circumference, and growth indices of 2,127 Cree newborns were compared with the CGS. Maternal characteristics of pregnancy and infant birth outcomes were recorded and stratified by birth weight category.
Among Cree newborns, 2.4% were low birth weight (LBW) (+2SD from the WHO CGS median for weight-for-length-for-age, BMI-for-age and head circumference-for-age, respectively. The majority (53.4%) of pregnancies was complicated by obesity and 10.3% were complicated by gestational diabetes mellitus (GDM). Infants weighing 4,000-4,499 g had a comparable prevalence of operative delivery (15.4%) as infants weighing 2,500-3,999 g (13.7%). Infants weighing =4,500 g had the highest prevalence of birth injuries (14.0%) and being born to women whose pregnancies were complicated by GDM (20%).
Cree newborns were larger than newborns of the CGS. The appropriateness for Cree infants of defining low and high birth weight from the WHO CGS is uncertain and may lead to inaccurate prognosis of postnatal health.
Mutations in senataxin have been described recently in 24 cases of French-Canadian descent with ataxia-oculomotor apraxia 2. This recessive ataxia is associated with an elevation in alpha-fetoprotein as in ataxia-telangiectasia. Because ataxia-telangiectasia cells are highly radiosensitive, we used a colony survival assay to measure the radiosensitivity of lymphoblastoid cell lines derived from five French-Canadian patients with ataxia-oculomotor apraxia 2. Two were homozygous for the common French-Canadian L1976R SETX missense mutation; the three others were compound heterozygotes for the common mutation and three different missense mutations. Overall, lymphoblastoid cell lines derived from these cases did not show significant variation from a normal response to 1 Gray of ionizing radiation but the two patients who were homozygous for the common L1976R mutation fell in the intermediate or non-diagnostic range.
Hereditary sensory and autonomic neuropathy type 2 (HSAN2) is a rare recessive genetic disorder characterized by severe sensory loss affecting the tactile, thermal and nociceptive modalities. Although heterozygous carriers of nonsense mutations in the HSN2 gene, called with-no-lysine(K)-1 (WNK1), do not develop the disease, historical and experimental evidence suggests that these individuals might perceive somatosensory stimuli differently from others. Using the method-of-limits, we assessed the thresholds for warmth detection, cool detection, heat pain and cold pain in 25 mutation carriers and 35 controls. In group analyses, carriers displayed significantly lower warmth (p
Thirty-three French-Canadian families with non-dystrophic myotonia were identified. Fifty subjects were recruited and submitted to a complete clinical, electrophysiologic and genetic evaluation. Thirteen mutations were identified in CLCN1 and five mutations were identified in SCN4A. Onset in the lower extremities, presence of tongue myotonia and transient weakness suggested recessive CLCN1 mutations. Lid myotonia, absence of hypertrophy and exacerbation with cold temperature suggested SCN4A mutations. Pain was not a feature of dominant CLCN1 mutations while it could be seen in the others, more frequently in SCN4A mutations. Warm up phenomenon, hand grip myotonia, percussion myotonia, lid lag and hormonal effects were not distinguishing features. Repetitive nerve stimulation and short exercise test showed either a large (>50%) or mild-moderate (10-50%) decrement with recessive CLCN1 mutations while they showed only mild or no decrement with dominant CLCN1 and SCN4A mutations. The French-Canadian population shows wide phenotypic and genotypic heterogeneity in non-dystrophic myotonias.
Inuit communities of Canada experience many disparities in health and psychosocial context. Research in community psychology has shown associations between such socio-ecological factors and individual well-being. The objective of the study was to explore how community-level determinants of well-being influence family well-being in a northern community of Nunavik, Quebec. A total of 14 participants were interviewed. A thematic inductive analysis was conducted to extract community determinants of family well-being from the data. A system science approach was used to explore the associations between determinants and larger psychosocial dynamics. A community workshop was held to discuss the results and their meaning. A total of 25 determinants were coded, 16 of which were community-level. Community-level stressors were highly interrelated, whereas community supports were generally disconnected and superimposed on narratives of stressors. Participants spoke of desired supports. In their narratives, these supports were connected to a variety of determinants of well-being, suggesting the need to connect, redefine and support existing resources rather than simply add on new ones. We discuss intricate links between family and community well-being in small and geographically isolated communities.
This case study of the use of health-care services in northern Quebec is the first of a series of reports dealing with the health status, risk factors and access to care of the two major aboriginal populations in this region, the Cree and the Inuit. The focus of the broader study is on the health status and risk factors in these native populations. This research, based on a survey conducted in 1983-84, describes the present health status and health-care system of the Cree and Inuit, then presents a path analysis model of their use of health services. The purpose of this approach is to provide health planners and care providers with useful information and to encourage the development of well-organized and reasonable health-care service delivery to these populations.
From: Fortuine, Robert et al. 1993. The Health of the Inuit of North America: A Bibliography from the Earliest Times through 1990. University of Alaska Anchorage. Citation number 1476.
Previous studies suggest that nonelective coronary artery bypass graft surgery (CABG) is more costly than elective CABG. The goal of this study was to examine why cost differences exist between patients undergoing nonelective and elective CABG.
We compared the outcomes and costs of treating 1613 consecutive patients undergoing nonelective (N = 1071) and elective (N = 542) CABG at three U.S. hospitals. Participating centers each used the same cost accounting system to provide patient-level clinical and cost data. Total, direct, and overhead costs were examined as were department-level costs.
Compared to elective patients, nonelective patients were of similar age (66.4 years vs 67.0 years, respectively, p = NS), but were more likely to be female (32.7% vs 24.0%, p = 0.0003). Nonelective patients had longer lengths of stay (LOS) than elective patients (9.7 +/- 0.2 days vs 6.6 +/- 0.3 days, p
To explore the impact of cross-cultural differences on University of California, Los Angeles, Prostate Cancer Index (PCI) reliability and validity, which is unknown. The PCI represents the most widely used prostate cancer-specific health-related quality-of-life assessment tool.
The PCI sexual and urinary scales, the RAND SF-36 survey, and the Prostate Outcomes Research Team (PORT) prostate cancer treatment complication profile were self-administered. The principal sample consisted of 2415 men (anglophone 256, francophone 2159) treated with radical prostatectomy in Quebec between 1988 and 1996. An additional 35 men (anglophone 17, francophone 18) formed the retest sample.
The PCI demonstrated excellent internal consistency and test-retest reliability in tests based on the entire cohort and in tests addressing the two linguistically different groups. The instrument showed a lack of convergence with the SF-36 scales, confirming the distinctness of the generic and prostate cancer-specific constructs. The PCI sexual scales converged with the aggregate PORT sexual items (r = 0.8), and the PCI urinary scales were strongly related to the aggregate PORT urinary items (r = 0.7). Convergence between PCI urinary bother and function was strong (r = 0.8), but only moderate convergence was noted between PCI sexual bother and function (r = 0.4). The relation between bother and function in both urinary and sexual domains was weaker in anglophone participants relative to their francophone counterparts.
The PCI is reliable and, at best, only modestly affected by cultural differences when administered to culturally distinct English-speaking men or when translated into French.