Bone density and bone area in Canadian Aboriginal women: the First Nations Bone Health Study.

INTRODUCTION: Canadian Aboriginal women are at increased risk of fracture compared with the general population. HYPOTHESIS: There is disproportionately reduced bone density in Aboriginal women as compared to white females of similar age. METHODS: A random age-stratified (25-39, 40-59 and 60-75) sample of Aboriginal women (n=258) and white women (n=181) was recruited. All subjects had calcaneus and distal forearm bone density measurements, and urban participants (n=397 [90.4%]) also had measurements of the lumbar spine, hip and total body. RESULTS: Unadjusted measurements were similar in the two groups apart from the distal forearm which showed a significantly lower mean Z-score in the Aboriginal women (p=0.03). Aboriginal women were heavier than white women (81.0+/-18.0 kg vs. 76.0+/-18.0 kg, p=0.02). Weight was directly associated with BMD at all measurement sites (p<0.00001) and potentially confounded the assessment of ethnicity on bone mass measurements. Weight-adjusted ANCOVA models demonstrated significantly lower bone density in Aboriginal than white women for the calcaneus, distal forearm, and total body (all p<0.05), but not at the other sites. ANCOVA models (adjusted for age, height and weight) were used to explore differences in bone area and bone mineral content (BMC). There was a significant effect of ethnicity on bone area with Aboriginal women having larger adjusted mean values than white women (lumbar spine p=0.038, total hip p=0.0004, total body p=0.020). In contrast, there was no detectable effect of ethnicity on BMC (all p>0.2). CONCLUSIONS: We identified significant site-specific differences in age-and weight-adjusted bone density for Aboriginal and white women. Larger bone area, rather than a reduction in BMC, appeared to be primarily responsible. Further work is needed to define how these differences in bone density and geometry affect indices of bone strength.

Outcome of the first 3-years of a DNA-based neonatal screening program for glutaric acidemia type 1 in Manitoba and northwestern Ontario, Canada.

Glutaric acidemia type 1 (GA1) is overrepresented in the aboriginal population of Island Lake, Manitoba, and northwestern Ontario who speak the Ojibway-Cree (Oji-Cree) dialect. The carrier frequency in these communities has been predicted to be as high as 1 in 10 individuals. Prior to beginning newborn screening for GA1 in May 1998, 18 of 20 affected patients diagnosed at this center have been from these high-risk communities. Most have followed an acute encephalopathic course with permanent neurologic sequelae and high mortality. They excrete small amounts of glutaric acid and 3-hydroxyglutaric acid and have significant residual enzyme activity. A single homozygous mutation in glutaryl-CoA-dehydrogenase (GCDH IVS-1 + 5g right arrow t) has been identified in this population. DNA-based newborn screening targeted to our high-risk communities was begun in order to provide presymptomatic detection and treatment of affected patients. Of the first 1176 newborns screened, 4 affected infants were identified and treated with a low-protein diet, carnitine, and riboflavin. All 4 infants have required numerous hospitalizations for treatment of intercurrent illnesses. Eventually, 3 infants presented with acute dystonic encephalopathy and seizures along with permanent neurological sequelae. One of these infants died unexpectedly at home at 18 months of age. The fourth, now 9 months old, has had a gastrostomy tube placed to facilitate fluid replacement in addition to a standard treatment protocol and is doing well. The reasons for our initial disappointing outcomes in the first 3 of 4 affected babies are likely multiple. Based on our early experience and that of other centers screening newborns for GA1, current therapeutic strategies may be insufficient in preventing the occurrence of neurologic sequelae in some children. An incomplete understanding of the neurotoxic mechanisms underlying this devastating disorder hampers effective management.

Childhood obesity in a population at high risk for type 2 diabetes.

OBJECTIVES: To determine the prevalence of obesity and investigate its association with fasting glucose and insulin among children and adolescents in a population at high risk for type 2 diabetes. DESIGN: A cross-sectional screening survey involving anthropometry and fasting serum levels of glucose and insulin. SETTING: A remote aboriginal (Ojibwa-Cree) community in northern Manitoba, Canada. PARTICIPANTS: All children aged 4 to 19 years in the community were invited to participate, with a response rate of 82% (n = 719). MAIN OUTCOME MEASURES: Obesity is defined as body mass index exceeding the 85th percentile of the National Center for Health Statistics reference data. The diagnosis of diabetes and impaired fasting glucose is based on the new criteria of the American Diabetes Association. RESULTS: There is a high prevalence of obesity, with 64% (female) and 60% (male) exceeding the 85th percentile and 40% (female) and 34% (male) exceeding the 95th percentile. Body mass index is a significant predictor of both glucose and insulin in both sexes, independent of age. Obese children are at increased risk of being classified as having diabetes or impaired fasting glucose (odds ratio 5.1, 95% CI 1.51, 17.0). CONCLUSIONS: The early onset of type 2 diabetes in childhood is increasingly observed in many populations. Childhood obesity is a strong risk factor. Early detection and intervention directed at obesity are potential strategies to avert the long-term consequences of type 2 diabetes.