Health, Social, Education, and Justice Outcomes of Manitoba First Nations Children Diagnosed with Fetal Alcohol Spectrum Disorder: A Population-Based Cohort Study of Linked Administrative Data.

OBJECTIVE: To examine health services, social services, education, and justice system outcomes among First Nations children and youth with fetal alcohol spectrum disorder (FASD). METHODS: In this retrospective cohort study, health and social services, education, and justice data were linked with clinical records on First Nations (FN) individuals aged 1 to 25 and diagnosed with FASD between 1999 and 2010 (n = 743). We compared the FN FASD group to non-FN individuals with FASD (non-FN FASD; n = 315) and to First Nations individuals (matched on age, sex, and income) not diagnosed with FASD (FN non-FASD; n = 2229). Rates and relative risks (RRs) were calculated using generalized linear models. RESULTS: FN FASD individuals had similar health services use to non-FN FASD individuals but had greater involvement with child welfare (RR, 1.20; 95% confidence interval [CI], 1.02 to 1.41) and the justice system (RR, 1.37; 95% CI, 1.07 to 1.74) and were more likely to be charged with a crime (RR, 1.40; 95% CI, 1.05 to 1.86). There were no suicides/suicide attempts among the non-FN FASD individuals during the study, but the crude rate/100 person-years of suicides among FN FASD individuals (0.22 for females; 1.06 for males) was substantially higher than for FN non-FASD individuals (0.08 for females; 0.32 for males). There were no significant differences between groups in the education outcomes we measured. CONCLUSIONS: Young people with FASD are at risk for poor health, education, and social outcomes, but First Nations young people with FASD face comparably higher risks, particularly with child welfare and justice system involvement. The study emphasizes a critical need for appropriate resources for First Nations children with FASD.

Alcohol abuse recovery through the lens of Manitoban First Nations and Aboriginal women: A qualitative study.

We describe a phenomenological study that explored the experiences of First Nations and Aboriginal women during their journey of recovery from alcohol abuse. Seven women participated in individual, tape-recorded interviews, during which they described their experiences, yielding written protocols that were thematically analyzed. Results from this study revealed seven predominant themes that included the following: (a) guilt and shame, (b) unresolved core issues, (c) resilience, (d) tenacity, (e) recovery process, (f) family and friends, and (g) tradition and spirituality. Findings and implications for clinicians, administrators, educators, and future research are included.

"People try and label me as someone I'm not": The social ecology of Indigenous people living with HIV, stigma, and discrimination in Manitoba, Canada.

Indigenous peoples (First Nations, Inuit, and Métis) are currently overrepresented in the HIV epidemic in Canada and are infected at a younger age than those who are not Indigenous. This article presents our findings on the stigma and discrimination (as well as related themes such as disclosure) experienced by Indigenous people who contracted HIV in their youth and live in urban and non-urban settings in Manitoba, Canada. The findings were derived from a qualitative study that sought to understand the experiences and needs of Indigenous people living with HIV (including AIDS). We situate such experiences within a social ecological framework towards developing a better structural understanding of the impacts of stigma and discrimination on the lives of Indigenous people who are HIV positive. Stigma and discrimination caused barriers for Indigenous people living with HIV through inhibiting their ease of access to supports including family, peers, community, and long- and short-term health services. Creative forms of outreach and education that are culturally appropriate and/or rooted in culture were considered to be possibly impactful ways of reducing stigma and discrimination at the community level. Learning from communities who are successfully managing stigma also showed promise for developing new programming.

Managing Matajoosh: determinants of first Nations' cancer care decisions.

BACKGROUND: Accessing cancer treatment requires First Nation peoples living in rural and remote communities to either commute to care, or to relocate to an urban centre for the length or part of the treatment. While Canadians living in rural and remote communities must often make difficult decisions following a cancer diagnosis, such decisions are further complicated by the unique policy and socio-historical contexts affecting many First Nation peoples in Canada. These contexts often intersect with negative healthcare experiences which can be related to jurisdictional confusion encountered when seeking care. Given the rising incidence of cancer within First Nation populations, there is a growing potential for negative health outcomes. METHODS: The analysis presented in this paper focuses on the experience of First Nation peoples' access to cancer care in the province of Manitoba. We analyzed policy documents and government websites; interviewed individuals who have experienced relocation (N = 5), family members (N = 8), healthcare providers and administrators (N = 15). RESULTS: Although the healthcare providers (social workers, physicians, nurses, patient navigators, and administrators) we interviewed wanted to assist patients and their families, the focus of care remained informed by patients' clinical reality, without recognition of the context which impacts and constrains access to cancer care services. Contrasting and converging narratives identify barriers to early diagnosis, poor coordination of care across jurisdictions and logistic complexities that result in fatigue and undermine adherence. Providers and decision-makers who were aware of this broader context were not empowered to address system's limitations. CONCLUSIONS: We argue that a whole system's approach is required in order to address these limitations.

Gambling and Problem Gambling among Canadian Urban Aboriginals.

OBJECTIVE: To assess the prevalence of gambling and problem gambling in urban Aboriginals in the Canadian Prairie provinces and to determine the predictors of problem gambling. METHOD: In total, 1114 Aboriginals living in 15 cities in Alberta, Saskatchewan, and Manitoba were recruited via posters and direct solicitation at Native Friendship Centres, shopping malls, and other locations where Aboriginals congregated. They each completed a self-administered 5- to 10-minute survey. RESULTS: Urban Aboriginals in the present sample were found to have a much higher level of gambling participation than the general Canadian public, especially for electronic gambling machines, instant lotteries, and bingo. Their intensity of participation in terms of number of formats, frequency of play, and gambling expenditure was also very high. This, in turn, is an important contributing factor to their very high rate of problem gambling, which was found to be 27.2%. Problem gambling was higher in males, unemployed people, and cities having the highest proportion of their population consisting of urban Aboriginals. CONCLUSIONS: Urban Aboriginal people appear to have some of the highest known rates of problem gambling of any group in Canada. This is attributable to having many more risk factors for problem gambling, such as a greater level of participation in gambling, greater participation in continuous forms of gambling (e.g., electronic gambling machines), younger average age, higher rates of substance abuse and mental health problems, and a range of disadvantageous social conditions (e.g., poverty, unemployment, poor education, cultural stress) that are conducive to the development of addictive behaviour.

Mammography rates for breast cancer screening: a comparison of First Nations women and all other women living in Manitoba, Canada, 1999-2008.

INTRODUCTION: First Nations (FN) women historically have low rates of preventive care, including breast cancer screening. We describe the frequency of breast cancer screening among FN women living in Manitoba and all other Manitoba (AOM) women after the introduction of a provincial, organized breast screening program and explore how age, area of residence, and time period influenced breast cancer screening participation. METHODS: The federal Indian Registry was linked to 2 population-based, provincial data sources. A negative binomial model was used to compare breast cancer screening for FN women with screening for AOM women. RESULTS: From 1999 through 2008, 37% of FN and 59% of AOM women had a mammogram in the previous 2 years. Regardless of area of residence, FN women were less likely to have had a mammogram than AOM women (relative rate [RR] = 0.69 in the north, RR = 0.55 in the rural south, and RR = 0.53 in urban areas). CONCLUSIONS: FN women living in Manitoba had lower mammography rates than AOM women. To ensure equity for all Manitoba women, strategies that encourage FN women to participate in breast cancer screening should be promoted.

Breast feeding practices as cultural interventions for early childhood caries in Cree communities.

BACKGROUND: Breastfeeding is a gift from mother to child and has a wide range of positive health, social and cultural impacts on infants. The link between bottle feeding and the prevalence of early childhood caries (ECC) is well documented. In Aboriginal communities, the higher rates of ECC are linked with low rates of breast feeding and inappropriate infant feeding of high sugar content liquids. METHODS: The Baby Teeth Talk Study (BTT) is one project that is exploring the use of four interventions (motivational interviewing, anticipatory guidance, fluoride varnish and dental care to expectant mothers) for reducing the prevalence of ECC in infants within Aboriginal communities. This research explored cultural based practices through individual interviews and focus groups with older First Nations women in the community. RESULTS: Participants in a First Nations community identified cultural based practices that have also been used to promote healthy infant feeding and good oral health. A wide range of themes related to oral health and infant feeding emerged. However, this paper focuses on three themes including: breastfeeding attitudes, social support for mothers and birthing and supporting healthy infant feeding through community programs. CONCLUSIONS: The importance of understanding cultural health traditions is essential for those working in oral public health capacities to ensure there is community acceptance of the interventions.

Rapid human immunodeficiency virus disease progression is associated with human leukocyte antigen-B homozygocity and human leukocyte antigen-B51 in a cohort from Manitoba, Canada.

BACKGROUND: Human immunodeficiency virus type 1 (HIV-1) infection is associated with variable rates of disease progression, influenced by the quality of CD8 T-lymphocyte response, which is determined by human leukocyte antigen (HLA) I alleles. Some individuals progress slowly and maintain viral control, while at the opposite end of the spectrum some individuals endure a faster progression with rapid CD4 decline. We sought to determine the role of HLA-B allele frequency on rapid HIV disease progression. It was hypothesized that rapid progression is associated with the presence of high allele frequency of HLA-B35 and HLA-B homozygocity. METHODS: This retrospective cohort study was conducted in the Manitoba HIV Program, Health Sciences Centre, a tertiary care facility in Winnipeg, Manitoba, Canada. We defined a set of new criteria to describe a subset of individuals with the most rapid HIV disease progression, and collected demographic, clinical, laboratory (CD4 count, viral load) and HLA data on a subset of 20 individuals meeting these criteria. RESULTS: Among those individuals who display extreme rapid progression, an overrepresentation of Aboriginal ethnicities, high frequencies of HLA-B35 and significantly higher rates of HLA-B51, as well as a very high rate of homozygocity for HLA-B alleles, were observed. CONCLUSIONS: Individuals with the most rapid disease progression have higher rates of HLA-B homozygocity, HLA-B51 alleles and higher viral loads than those with normal progression rates. This group, at the extreme end of the spectrum of progression, should be targeted for early treatment.

Ethnicity and geographic distribution of pediatric chronic ataxia in Manitoba.

BACKGROUND: Genetic and environmental factors are important determinants of disease distribution. Several disorders associated with ataxia are known to occur more commonly in certain ethnic groups; for example, the disequilibrium syndrome in the Hutterites. The aim of this study was to determine the ethnic and geographic distribution of pediatric patients with chronic ataxia in Manitoba, Canada. METHODS: We identified 184 patients less than 17 years-of-age with chronic ataxia during 1991-2008 from multiple sources. Their diagnosis, ethnicity and place of residence were determined following a chart review. RESULTS: Most patients resided in Manitoba (N=177) and the majority in Winnipeg, the provincial capital. Thirty five Aboriginal, 29 Mennonite and 11 Hutterite patients resided in Manitoba. The latter two groups were significantly overrepresented in our cohort. Ataxia telangiectasia, mitochondrial disorders, and non-progressive ataxia of unknown etiology associated with pyramidal tracts signs and developmental delay were significantly more common in Mennonite patients. Four of five patients with neuronal migration disorders associated with chronic ataxia were Aboriginal. Few isolated disorders with chronic ataxia occurred in the 11 Hutterite patients including a Joubert syndrome related disorder. CONCLUSIONS: Three disorders associated with chronic ataxia were more prevalent than expected in Mennonites in Manitoba. Few rare disorders were more prevalent in the Hutterite and Aboriginal population. Further research is needed to determine the risk factors underlying these variations in prevalence within different ethnic groups. The unique risk factor profiles of each ethnic group need to be considered in health promotion endeavors.Ethnie et distribution géographique de l'ataxie chronique chez des patients d'âge pédiatrique au Manitoba.

Parent child coalitions: innovative public-sector management and early childhood development in Manitoba.

This article considers a coalition model of governance as an innovative approach to public management. In general, the coalition governance model adopts key principles of new public management and inherits criticisms similar to those levelled against the new managerialism. Looking at a case study of parent child coalitions in Manitoba, this article explores some benefits and consequences of implementing and utilizing coalition governance as a model for social policy. It finds that the attempt to increase child-centred programming across the province required innovative adjustments to the management of this social policy issue, as well as a restructuring of the overarching policy structure. Innovative public management and the implementation of a coalition governance approach helped transform early childhood development in Manitoba from a private and personal family concern to a public policy issue. It has increased citizen engagement and has also increased government access to a previously inaccessible segment of society. Although these innovations resolved some key concerns, additional criticisms remain as yet unaddressed.

Obesity and obesity-related comorbidities in a Canadian First Nation population.

INTRODUCTION: Rates of obesity are higher among Canada's Aboriginal First Nations populations than among non-First Nations populations. We studied obesity and obesity-related illness in a Manitoba First Nation community. METHODS: We conducted a screening study of diabetes and diabetes complications in 2003, from which we drew a representative sample of Manitoba First Nations adults (N = 483). We assessed chronic disease and chronic disease risk factors. RESULTS: Prevalence of obesity and associated comorbidities was higher among women than men. By using multivariate analysis, we found that factors significantly associated with obesity among women were diastolic blood pressure, insulin resistance, and employment status. Among men, factors were age, apolipoprotein A1 level, apolipoprotein B level, and insulin resistance. Seventy-five percent of study participants had at least 1 of the following conditions: obesity, dyslipidemia, hypertension, or diabetes. Comorbidity was high even among the youngest age groups; 22% of men and 43% of women aged 18 to 29 had 2 or more chronic conditions. Twenty-two percent of participants had undiagnosed hypertension. Participants with undiagnosed hypertension had significantly more chronic conditions and were more likely to have microalbuminuria than were those without hypertension. The number of chronic conditions was not significantly different for participants with newly diagnosed hypertension than for those with previously diagnosed hypertension. CONCLUSION: The prevalence of obesity and other chronic conditions in the study community is high, especially considering the number of young people. Community-based interventions are being undertaken to reduce the excessive rate of illness.

A Decade Later: assessing successes and challenges in Manitoba's Provincial Immigrant Nominee Program.

During the past decade, Manitoba's Provincial Nominee Program (MPNP) has increased immigration to the province and dispersed immigrants more widely within Manitoba. At the same time, the rapid growth of the program and the decentralized way in which it has been implemented have contributed to some challenges. This ten-year analysis of the MPNP finds that many places in Manitoba are experiencing settlement service gaps, and that immigrants and communities are taking on much of the burden for MPNP application and settlement. In addition, the analysis demonstrates that the fragmented way in which the MPNP has been marketed and implemented (i.e., by relying on particular employers, consultants, and ethnocultural organizations) has resulted in a sort of ethnocultural inequality where certain groups are ushered into the province-often to perform particular occupations-while others are bypassed.

Correlates of severe disease in patients with 2009 pandemic influenza (H1N1) virus infection.

BACKGROUND: In the context of 2009 pandemic influenza (H1N1) virus infection (pandemic H1N1 influenza), identifying correlates of the severity of disease is critical to guiding the implementation of antiviral strategies, prioritization of vaccination efforts and planning of health infrastructure. The objective of this study was to identify factors correlated with severity of disease in confirmed cases of pandemic H1N1 influenza. METHODS: This cumulative case-control study included all laboratory-confirmed cases of pandemic H1N1 influenza among residents of the province of Manitoba, Canada, for whom the final location of treatment was known. Severe cases were defined by admission to a provincial intensive care unit (ICU). Factors associated with severe disease necessitating admission to the ICU were determined by comparing ICU cases with two control groups: patients who were admitted to hospital but not to an ICU and those who remained in the community. RESULTS: As of Sept. 5, 2009, there had been 795 confirmed cases of pandemic H1N1 influenza in Manitoba for which the final treatment location could be determined. The mean age of individuals with laboratory-confirmed infection was 25.3 (standard deviation 18.8) years. More than half of the patients (417 or 52%) were female, and 215 (37%) of 588 confirmed infections for which ethnicity was known occurred in First Nations residents. The proportion of First Nations residents increased with increasing severity of disease (116 [28%] of 410 community cases, 74 [54%] of 136 admitted to hospital and 25 [60%] of 42 admitted to an ICU; p<0.001), as did the presence of an underlying comorbidity (201 [35%] of 569 community cases, 103 [57%] of 181 admitted to hospital and 34 [76%] of 45 admitted to an ICU; p<0.001). The median interval from onset of symptoms to initiation of antiviral therapy was 2 days (interquartile range, IQR 1-3) for community cases, 4 days (IQR 2-6) for patients admitted to hospital and 6 days (IQR 4-9) for those admitted to an ICU (p<0.001). In a multivariable logistic model, the interval from onset of symptoms to initiation of antiviral therapy (odds ratio [OR] 8.24, 95% confidence interval [CI] 2.82-24.1), First Nations ethnicity (OR 6.52, 95% CI 2.04-20.8) and presence of an underlying comorbidity (OR 3.19, 95% CI 1.07-9.52) were associated with increased odds of admission to the ICU (i.e., severe disease) relative to community cases. In an analysis of ICU cases compared with patients admitted to hospital, First Nations ethnicity (OR 3.23, 95% CI 1.04-10.1) was associated with increased severity of disease. INTERPRETATION: Severe pandemic H1N1 influenza necessitating admission to the ICU was associated with a longer interval from onset of symptoms to treatment with antiviral therapy and with the presence of an underlying comorbidity. First Nations ethnicity appeared to be an independent determinant of severe infection. Despite these associations, the cause and outcomes of pandemic HINI influenza may involve many complex and interrelated factors, all of which require further research and analysis.

Unique disease heritage of the Dutch-German Mennonite population.

The Dutch-German Mennonites are a religious isolate with foundational roots in the 16th century. A tradition of endogamy, large families, detailed genealogical records, and a unique disease history all contribute to making this a valuable population for genetic studies. Such studies in the Dutch-German Mennonite population have already contributed to the identification of the causative genes in several conditions such as the incomplete form of X-linked congenital stationary night blindness (CSNB2; previously iCSNB) and hypophosphatasia (HOPS), as well as the discovery of founder mutations within established disease genes (MYBPC1, CYP17alpha). The Dutch-German Mennonite population provides a strong resource for gene discovery and could lead to the identification of additional disease genes with relevance to the general population. In addition, further research developments should enhance delivery of clinical genetic services to this unique community. In the current review we discuss 31 genetic conditions, including 17 with identified gene mutations, within the Dutch-German Mennonite population.

Type 2 diabetes mellitus in children: prenatal and early infancy risk factors among native canadians.

BACKGROUND: Type 2 diabetes mellitus is increasingly being observed among children and youth, including the Native population of Canada. Only one study has investigated prenatal and early infancy risk factors for the disease. METHODS: A case-control study was conducted; 46 patients younger than 18 years were recruited from the only clinical center for the treatment of diabetes serving the province of Manitoba, and 92 age- and sex-matched controls were recruited from a pediatric ambulatory clinic serving a large Native population in Winnipeg, Manitoba. Information on exposure to prenatal and early infancy risk factors was obtained through questionnaires administered by a Native nurse-interviewer. RESULTS: Multiple logistic regression modeling identified preexisting diabetes (odds ratio [OR], 14.4; 95% confidence interval [CI], 2.86-72.5), gestational diabetes (OR, 4.40; 95% CI, 1.38-14.1), and breastfeeding longer than 12 months (OR, 0.24; 95% CI, 0.13-0.99) as significant independent predictors of diabetic status. Other factors, such as low (<2500 g) and high (>4000 g) birth weight and maternal obesity, were also associated with diabetes in our population, but the elevated risks were not statistically significant. CONCLUSION: Breastfeeding reduces the risk of type 2 diabetes among Native Canadian children and should be promoted as a potential intervention to control the disease.

Diabetes-associated autoantibodies in aboriginal children.

Type-2 diabetes is increasing in aboriginal children and adolescents and must be distinguished from type-1 diabetes in this population. The absence of diabetes-associated autoantibodies supports the clinical impression of type-2 diabetes in the affected members of this population.

Knowledge of fetal alcohol syndrome (FAS) among natives in Northern Manitoba.

OBJECTIVE: To investigate knowledge about fetal alcohol syndrome (FAS) among Natives in northern Manitoba. A second objective was to determine if there are age or gender differences in level of knowledge. A third objective was to examine the relationship between knowledge about FAS and reported frequency of drug and alcohol use during pregnancy. METHOD: A nonrandom sample of 466 Natives from northern Manitoba was interviewed about drug and alcohol use during pregnancy and knowledge of FAS. The sample was proportionately representative of the 26 northern reserves, with an approximately equal number of male and female subjects ranging in age from 13 to 71 years. RESULTS: Northern Manitoban Natives have lower levels of FAS knowledge than the general public. Of this sample, 80% believed drinking alcohol could adversely affect the unborn baby and 36% had heard of FAS, compared to 90% and 64%, respectively, in the general U.S. population. Natives in their 20s and 30s were more knowledgeable than Natives in their 50s and 60s. Females tended to be more knowledgeable than males. Drug and alcohol use during pregnancy is high (51% of women report drinking during one or more pregnancies) and the relationship between FAS knowledge and drug use during pregnancy appears weak. Young people were the most knowledgeable about FAS but also the most likely to report having used drugs or alcohol during pregnancy. CONCLUSIONS: The low levels of knowledge about FAS among the Native population supports the need for continued education. However, the results also suggest that education by itself may be insufficient to make dramatic changes in behavior.

Factors associated with human papillomavirus infection detected by polymerase chain reaction among urban Canadian aboriginal and non-aboriginal women.

OBJECTIVES: To determine the prevalence and correlates of human papillomavirus (HPV) infection among women attending an ethnically mixed, predominantly low-income, inner-city primary care clinic. STUDY DESIGN: Cross-sectional survey (N = 1,477). Demographic, behavioral, and reproductive history data were collected by questionnaire. Cervical swabs were analyzed for gonorrhea and chlamydial infections, abnormal cytology, and HPV infection assessed by PCR followed by hybridization for types 6, 11, 16, 18, 31, 33, and 35. RESULTS: Human papillomavirus was detected in 33% of specimens, with no significant difference between Aboriginal (AB) and non-Aboriginal women. Adjusting for AB status, HPV infection was associated with marital status, condom use, number of sexual partners (last year and lifetime), age at first sexual intercourse, a history of sexual abuse, and current abnormal Papanicolaou (Pap) smear. In multivariate analyses excluding abnormal Pap smear as an independent variable, marital status and the number of lifetime sexual partners were found to be significant independent predictors of HPV infection. Comparing individuals with 20+ lifetime sexual partners and those with 1 or fewer partners, the odds ratio (OR) for HPV infection was 1.90 (95% confidence interval [CI] 1.66-2.17) among AB women and 1.54 among non-AB women (95% CI 1.36-1.73). The OR for infection with HPV types 6 and 11 was 1.39 (95% CI 1.22-1.58), whereas for the high oncogenic risk types of 16, 18, 31, 33, and 35, the OR was 1.82 (95% CI 1.65-2.01). CONCLUSIONS: No differences were found between AB and non-AB women in the detection of HPV DNA, despite the higher risk for cervical cancer and the prevalence of recognized behavioral and reproductive risk factors among AB women. This study also indicates that the association of sexual activity with HPV infection holds true for both high- and low-oncogenic HPV types.

Risk factors for infection in women undergoing testing for Chlamydia trachomatis and Neisseria gonorrhoeae in Manitoba, Canada.

BACKGROUND AND OBJECTIVES: Despite sharing common modes of transmission, characteristics of individuals infected with Chlamydia trachomatis differ in several respects from those with Neisseria gonorrhoeae infection. Further characterization of women at high risk for chlamydial infection is needed to deliver appropriate and effective preventive, diagnostic, and therapeutic care to this population. GOAL OF THIS STUDY: The demographic and socioeconomic characteristics of women with laboratory confirmed chlamydia, gonorrhea, or coinfection were compared with those of control women who tested negative for both pathogens. STUDY DESIGN: A random sample of 400 women in Manitoba, Canada, who had undergone testing for sexually transmitted diseases at a public health laboratory in 1988 were studied. After linkage with medical insurance and census databases, logistic regression analysis was used to compare age, ethnicity, urban status, and mean income (using postal codes) of women with gonorrhea alone, chlamydia alone, and coinfection, with the same data for women who tested negative for both organisms. RESULTS: Young age, North American Indian status, urban residence, and low mean income according to postal code were significantly associated with gonococcal and chlamydial infection in the study population, compared with women who tested negative for both infections. Young age, Indian status, and urban residence also were associated with gonorrhea infection alone. Only young age and Indian status were associated with chlamydial infection. Mean incomes of women with chlamydial infection alone and control subjects were higher than those of women with gonorrhea and gonorrhea and chlamydia coinfection. CONCLUSIONS: Differences in the demographic and socioeconomic characteristics of women with gonorrhea, chlamydia, and coinfection suggest the existence of multiple reservoirs of infection due to these agents in the study population. The preventive, diagnostic, and therapeutic strategies of sexually transmitted disease control programs must be adapted to the individual needs of identified high-risk groups.

Increased prevalence of hereditary metabolic diseases among native Indians in Manitoba and northwestern Ontario.

OBJECTIVE: To compare the prevalence of hereditary metabolic diseases in the native and non-native populations of Manitoba and northwestern Ontario. DESIGN: Retrospective analysis. SETTING: Children's Hospital, Winnipeg. PATIENTS: Patients were selected by three methods: laboratory tests designed to screen patients suspected of having a metabolic disease, laboratory investigation of newborn infants with abnormalities detected through screening, and investigation of near relatives of probands with disease. RESULTS: A total of 138 patients with organic acid, amino acid and carbohydrate disorders were seen from 1960 to 1990. Of these, 49 (36%) were native Indians (Algonkian linguistic group). This was in sharp contrast to the proportion of native Indians in the total study population (5.8%). Congenital lactic acidosis due to pyruvate carboxylase deficiency (13 patients), glutaric aciduria type I (14 patients) and primary hyperoxaluria type II (8 patients) were the most common disorders detected. Other rare disorders included glutaric aciduria type II (one patient), 2-hydroxyglutaric aciduria (one patient) and sarcosinemia (one patient). Underreporting, especially of glutaric aciduria type I and hyperoxaluria type II, was likely in the native population. CONCLUSIONS: Hereditary metabolic diseases are greatly overrepresented in the native population of Manitoba and northwestern Ontario. We recommend that native children who present with illnesses involving disturbances of acid-base balance or with neurologic, renal or liver disease of unknown cause by investigated for a possible metabolic disorder.