Genealogical analysis of cystic fibrosis families and chromosome 7q RFLP haplotypes in the Hutterite Brethren.

In the 100-year period 1880-1980 the Hutterite population increased from about 442 to 23,000 individuals in North America. There are three endogamous subdivisions in this Caucasian genetic isolate. A total of 11 cystic fibrosis (CF) families from Canada and the United States were investigated, including at least two families from each of the three subdivisions, the Dariusleut, Lehrerleut, and Schmiedeleut. A study of RFLPs for the loci D7S8, D7S23, MET, and D7S18 (also called D7S16) in the region of the CF gene in 10 families shows considerable genetic variability. There were three different extended CF gene-region haplotypes on CF chromosomes (CF haplotypes), and there were 13 different extended CF gene-region haplotypes on normal chromosomes (normal haplotypes). The three CF haplotypes have different D7S23 and MET haplotypes. Parents who have the same CF haplotype are, on the average, more closely related than parents who have different haplotypes, but only within the same subdivision. A marriage node graph of 11 families illustrates the complexity of Hutterite genealogies. The frequency distribution of CF haplotypes in the Hutterite sample differs notably from those of larger agglomerates of family data from collaborative studies, with respect to D7S8, MET haplotypes, and D7S23 haplotypes. We propose that there were at least three CF carriers among the founders of the Hutterite population and that copies of a particular CF haplotype in current individuals are identical by descent. The alternative that one or more genetically distinguishable CF haplotypes resulted from recombination since the founding of the population is considered to be less likely.     

Genetic variability of HLA in the Dariusleut Hutterites. A comparative genetic analysis of the Hutterities, the Amish, and other selected Caucasian populations.

There are three endogamous subdivisions of the Hutterite population, a North American religious isolate. These individuals live on communal farms, and residence is strictly patrilocal. We report on the distributions of HLA-A and B alleles and haplotypes in 203 married women from one subdivision--the Dariusleut--in Alberta, Canada. We demonstrate that there is significant linkage disequilbrium among a large fraction of the distinct haplotypes in the Dariusleut Hutterite data; there is a restriction in the number of distinct haplotypes present in the Dariusleut; the Hutterites and the Old Order Amish (Lancaster County, Pennsylvania) are the most genetically distant pair of populations in an ensemble of 11 Caucasian populations; and, finally, the Old Order Amish and the Hutterites are approximately as distant from the Indiana Amish as they are from the eight other Caucasian populations, which are tightly clustered in the space of gene frequencies. These results are consistent with the fact that the Amish and the Hutterites are genetic isolates with small numbers of founders. Certain haplotypes show significant linkage disequilibrium in these as well as in other Caucasian samples. Thus, some of the linkage disequilibrium antedates the formation of these Anabaptist sects.     

Segregation of HLA A,B haplotypes and the distribution of antigen mismatches between mother and offspring in Hutterite families

Segregation of HLA haplotypes and offspring genotype distributions were analyzed in families from an inbred Caucasoid population, the Dariusleut Hutterite Brethren. Both parents and from one to 12 offspring were typed for HLA-A and -B antigens in 108 families. Segregation of paternal haplotypes was analyzed conditional on sibship size in 95 sibships (a total of 547 offspring), and segregation of maternal haplotypes, in 90 sibships (a total of 515 offspring). The distribution of the number of different genotypes among the offspring was analyzed conditional on sibship size in 90 families (515 offspring) where four equiprobable genotypes were expected. The distribution of the number of antigenic differences or mismatches for broad specificities between mother and offspring was analyzed in pooled family data consisting of a total of 377 offspring comprising 68 families. Compared with the multinomial distribution of segregation classes of haplotypes, there was no significant departure (probability .05 or less) from the expected segregation ratio for either paternal or maternal haplotypes. Compared with the multinomial distribution of the number of genotypes among the offspring, only two of the 11 sibship sizes had configurations that exceeded the 5% level of significance. Given the number of statistical tests performed, it is likely that these results could be explained by chance variation. Finally, there was no relative deficiency of offspring who were less mismatched with their mother for HLA-A and -B broad specificities. Therefore, if HLA-A,B region variation does have a major effect on the differential survival of fetuses in some families, it is an uncommon factor among fertile couples from this inbred population.     

Declining fertility among North American Hutterites: the use of birth control within a Dariusleut colony

Historically, population researchers considered the Hutterites the champions of high fertility, since they most closely approximated the human fertility potential. Yet contemporary researchers have found evidence of a decline in fertility and a shift from natural to controlled fertility behavior among the Hutterite population. Various reasons for these recent changes have been suggested, including the use of birth control. Using interview data from a Dariusleut colony, this is the first paper to document the presence and prevalence of birth control among the North American Hutterites. All interviewed women recognized the use of birth control and reported that female sterilization was the most commonly used method. I address how the various forms of birth control and the reasons for its use relate to the Hutterite ideology.     

Cystic fibrosis mutations in the Hutterite Brethren.

The presence or absence of the major cystic fibrosis (CF) mutation, delta F508, in the general patient population was determined by Kerem et al. using allele-specific oligonucleotides for the mutant and normal sequences in the polymerase chain reaction (PCR). delta F508 was identified by Riordan et al., and it is a 3-bp deletion of the phenylalanine codon at position 508. The Hutterite Brethren are an inbred North American population who have three different DNA marker haplotypes of CF chromosomes. Genomic DNA from both a CF child and one parent from each of 10 Hutterite families was analyzed for the presence or absence of the deletion mutation. delta F508 is associated with one of the three CF haplotypes in the Hutterite population, and this is the most common haplotype in a subset of the linkage family data of Kerem et al. The other two Hutterite CF haplotypes are generally rate in Caucasian populations. Since these two CF haplotypes do not carry the deletion mutation, they must carry a different CF mutation(s). The results of the PCR analysis for the deletion mutation lend additional support to our previous conclusion that there were at least three original carriers of CF mutations among the founders of the Hutterite population and that all copies of the same CF haplotype were identical by descent. One Hutterite CF patient has both of the haplotypes which do not carry delta F508. Analysis of this individual's DNA should allow identification of two additional CF mutations in this population.     

Illness in the family

OBJECTIVE: To update reports of increases in the rates of admission to hospital and death from asthma among children and young adults in Canada during the 1970s by examining data for the 1980s. DESIGN: Age-standardized rates were calculated from data for people less than 35 years of age at the time of death from asthma, bronchitis or other respiratory conditions (from 1980 through 1989) and at the time of admission to hospital for treatment of these diseases (from 1980 through 1988). Standardized mortality ratios were calculated with the death rate for Canada as the expected rate. SETTING: Data for all of Canada were examined by sex, age group and province. RESULTS: In contrast to sharp increases in the rate of death from asthma observed from 1970 through the early 1980s among Canadians less than 35 years of age, the rate showed no net change between 1980 and 1989; on average, there were 58 deaths in this age group annually. During the decade, the rates of death from asthma were three times higher in Saskatchewan and Alberta than in Newfoundland. The national rate of hospital admission/separation for asthma, however, increased greatly, though changes in the rate varied by province. Increases of over 90% were observed in Prince Edward Island and New Brunswick, whereas little overall change occurred in Newfoundland, Manitoba and Saskatchewan. The rate of hospital admission/separation for asthma was highest in Prince Edward Island and lowest in Manitoba and British Columbia. Although the rates of hospital admission/separation for asthma among boys aged less than 15 years of age were consistently 50% higher than those among girls of that age, the rate among people aged 15 through 34 years was twice as high among females as males. A slight decrease in the rates of death from respiratory conditions other than asthma was observed, together with a steady, fairly substantial decline in the rates of hospital admission/separation for these conditions. CONCLUSIONS: Whether there is any relation between increases in rates of admission to hospital for asthma and trends in the rates of death from asthma during the decade will require further study.     

Dental agenesis in the Dariusleut Hutterite Brethren: comparisons to selected Caucasoid population surveys

We report the results of a study of the prevalence of nonsyndromic dental agenesis among a sample of 208 individuals (105 females, 103 males) between the ages of 15 and 29 years from a North American religious and genetic isolate, the Dariusleut Hutterites of Western Canada. Direct examination of dental casts, oral examination reports, dental treatment records, and a limited number of dental radiographs reveals congenital absence and/or obvious morphometric reduction of at least one tooth (excluding third molars) in 98 subjects (55 females, 43 males), yielding a prevalence estimate of approximately 47%. This estimate is nearly four times those reported for nonisolate Caucasoid populations of European descent and substantially higher than the elevated prevalences observed in several other isolated populations. Although the prevalence of dental agenesis in the Dariusleut is indeed high, neither the incidence of bilateral agenesis (exhibited at least once in 58% of affected dentitions), number of affected teeth per person (mean, 2.4), morphologic tooth classes affected, or combinations of tooth classes affected ostensibly distinguish them from other populations with similar geographic origins. We conclude that the dental agenesis observed in this North American genetic isolate does not represent a private polymorphism or rare developmental variant. Consequently, the results of further study in these Dariusleut Brethren will be directly relevant to critically testing as yet unresolved hypotheses for the mode of gene action and the relative contributions of hereditary and environmental factors to the reduction of tooth numbers in human dentitions.     

Potential role of soil properties in the spread of CWD in western Canada

Chronic wasting disease (CWD) is a horizontally transmissible prion disease of free ranging deer, elk and moose. Recent experimental transmission studies indicate caribou are also susceptible to the disease. CWD is present in southeast Alberta and southern Saskatchewan. This CWD-endemic region is expanding, threatening Manitoba and areas of northern Alberta and Saskatchewan, home to caribou. Soil can serve as a stable reservoir for infectious prion proteins; prions bound to soil particles remain infectious in the soils for many years. Soils of western Canada are very diverse and the ability of CWD prions to bind different soils and the impact of this interaction on infectivity is not known. In general, clay-rich soils may bind prions avidly and enhance their infectivity comparable to pure clay mineral montmorillonite. Organic components of soils are also diverse and not well characterized, yet can impact prion-soil interaction. Other important contributing factors include soil pH, composition of soil solution and amount of metals (metal oxides). In this review, properties of soils of the CWD-endemic region in western Canada with its surrounding terrestrial environment are described and used to predict bioavailability and, thus, potential spread of CWD. The major soils in the CWD-endemic region of Alberta and Saskatchewan are Chernozems, present in 60% of the total area; they are generally similar in texture, clay mineralogy and soil organic matter content, and can be characterized as clay loamy, montmorillonite (smectite) soils with 6–10% organic carbon. The greatest risk of CWD spread in western Canada relates to clay loamy, montmorillonite soils with humus horizon. Such soils are predominant in the southern region of Alberta, Saskatchewan and Manitoba, but are less common in northern regions of the provinces where quartz-illite sandy soils with low amount of humus prevail.     

Molecular genetic variation and geographical structuring in Fusarium graminearum

Inter‐simple sequence repeat markers were employed to study the genetic structure of Fusarium graminearum populations collected from three Canadian provinces. Our study suggested high genetic diversity and frequent gene flow among population samples analysed. The analysis of molecular variance indicated that most of the gene diversity (91.78% to 97.23%) was distributed within populations. Frequent gene flow among the western prairie provinces and cluster analysis results indicated that the population sample from Alberta was closer to Saskatchewan than Manitoba, which could be the result of movement of the pathogen via infected grain or through wind‐borne ascospore dispersal. Analysis of multilocus associations showed that all populations were in linkage equilibrium, indicating that sexual recombination is a frequent phenomenon in F. graminearum populations from western Canada.     

Non-native insects in agriculture: strategies to manage the economic and environmental impact of wheat midge, Sitodiplosis mosellana, in Saskatchewan

Wheat midge, Sitodiplosis mosellana (Géhin) (Diptera: Cecidomyiidae), was first detected as early as 1901 in western Canada. The first major outbreak in Saskatchewan was recorded in 1983. Today wheat midge infests much of the wheat-growing area of Manitoba, Saskatchewan and North Dakota (USA), and is beginning to invade Alberta and Montana (USA). In 1984, Saskatchewan wheat midge populations were found to be parasitized by the egg-larval parasitoid, Macroglenes penetrans (Kirby) (Hymenoptera). Through the successful implementation of conservation techniques, this parasitoid now controls an average of 31.5% of the wheat midge across Saskatchewan. Estimated value of the parasitoid, due to reduction in insecticide costs in Saskatchewan alone, was estimated to be in excess of $248.3 million in the 1990s. The environmental benefits of not having to apply this amount of chemical insecticide are a bonus. To minimize the economic and ecological impact of S. mosellana today, wheat producers in western Canada have access to one of the most comprehensive management programs of any insect pest of field crops. Forecasts and risk warnings, monitoring tools, cultural control, agronomic practices, chemical control, biological control and plant resistance are all available for producers to manage wheat midge.     

Plant diversity in wooded moderate-rich fens across boreal western Canada: an ecoregional perspective

Ecoregions are increasingly being used as a framework for conservation planning. The Mid-Boreal Uplands Ecoregion stretches across Canada from Manitoba to British Columbia. From the perspective of conservation and to understand the dynamics of plant diversity and community composition in a common wetland type, we examined the plant communities and environmental variables in 80 wooded moderate-rich fens within this ecoregion. Regional diversity totalled 273 species, with 86 bryophytes and 187 vascular plants. Total diversity was greatest in Manitoba and decreased in a longitudinal trend west through Saskatchewan and Alberta. This may be related, in part, to orographic precipitation at Manitoba sites and a gradient of growing degree days. Richness of locally rare vascular plants exhibited a clear west to east gradient. Ten species of provincially rare vascular plants were observed across the ecoregion, but without pattern. Ordinations and other analyses revealed distinct plant communities for all three locations, with vascular plant assemblages more discrete than bryophyte assemblages. Bryophyte diversity increased with latitude and longitude, whereas vascular plant diversity decreased. Additionally, elevation, precipitation, surface water alkalinity, water temperature, percent overstory density, and peat organic C played a role in determining species richness and community composition. Overall, species composition and diversity in a single wetland type exhibited continuous change across multiple political jurisdictions at the ecoregion scale. Conservation plans for wetlands at the ecodistrict scale may be preferable.     

Distribution and ecology of meningeal worm,Parelaphostrongylus tenuis (Nematoda), in northcentral North America

Meningeal worm (Parelaphostrongylus tenuis), a common nematode parasite in white-tailed deer (Odocoileus virginianus) and pathogenic for several species of ungulates in eastern North America, is not known to occur in the west. Heads of 1,902 white-tailed deer were examined for adult meningeal worm to determine geographic distribution of the parasite in Saskatchewan and Manitoba (Canada) and North Dakota (USA). Finding the parasite in a deer in eastern Saskatchewan near the Manitoba border established the current northern and western limits in Canada. Prevalence of infection was < 1, 18.6, and 8.2% in Saskatchewan, Manitoba, and North Dakota, respectively. Infected deer occurred throughout southern Manitoba and eastern North Dakota. Distribution appears to have changed little since the last published survey for P. tenuis in the region in 1972. We examined precipitation, temperature, deer density, and forest cover as likely correlates to prevalence and distribution of P. tenuis. Deer management units used for hunting purposes were the scale of analysis in the three jurisdictions. Presence of P. tenuis was positively correlated with precipitation during frost-free periods and deer density, and it was negatively correlated with winter and spring temperatures. Landscapes with > 25 and < 75% forest cover were most likely to have infected deer. Low rainfall and low density of white-tailed deer likely influence the westernmost limit of P. tenuis.     

Rabies in insectivorous bats of western Canada, 1979 to 1983

A total of 1,745, 362, and 536 bats collected in Alberta, British Columbia, and Saskatchewan, respectively, was tested for rabies virus between 1979 and 1983. Only one (0.1%) of 769 bats collected at random from buildings was infected with rabies virus in contrast to 95 (5%) of 1,874 symptomatic, rabies-suspect bats submitted for testing. The pattern of infection in the rabies-suspect bats was similar in Alberta and Saskatchewan, but differed in British Columbia. Rabies was diagnosed in four species of bats in each of Alberta and Saskatchewan, but in seven species in British Columbia. Annual prevalence in rabies-suspect bats was similar in colonial species within each province. Rabies was found rarely in suspect little brown bats (Myotis lucifugus) (less than 1%). In suspect big brown bats (Eptesicus fuscus), the prevalence was low in Saskatchewan (3%), moderate in Alberta (10%), and high in British Columbia (25%). Big brown bats accounted for over 55% of the rabid bats detected in each province. Annual prevalence reported in silver-haired bats (Lasionycteris noctivagans) and hoary bats (Lasiurus cinereus) was variable in all three provinces. Rabies is enzootic in northern insectivorous bats.     

Genetic diversity and recombination within populations of Fusarium pseudograminearum from western Canada.

Genetic diversity within populations of Fusarium pseudograminearum isolated from wheat grains from the Canadian provinces of Alberta and Saskatchewan was investigated. Three restriction enzymes (EcoRI, HaeIII, and PstI) were used to carry out restriction analysis of the nuclear ribosomal DNA (nrDNA) intergenic spacer region (IGS region) and eight primers were used to generate inter-simple sequence-repeat (ISSR) molecular markers. Our study indicated substantially high genetic diversity within these two populations, but low genetic differentiation and frequent gene flow among populations. The IGS data showed no genetic distinction between the two Alberta populations and only minor genetic differentiation between the Saskatchewan and Alberta populations. Analysis of molecular variance indicated that most genetic variability resulted from differences among isolates within populations. Multilocus linkage disequilibrium analysis suggested a panmictic population genetic structure and the occurrence of significant recombination in F. pseudograminearum. Regular gene flow and random mating between isolates from different populations could result in novel genotypes with both improved pathological and biological traits.     

Mitochondrial DNA (mtDNA) reveals that female Bechstein's bats live in closed societies

We present a microgeographic analysis of mitochondrial DNA (mtDNA) in Bechstein's bats using three sources of control region sequence variability, including a novel mtDNA microsatellite, to assess individual relatedness both within and among 10 maternity colonies. Comparison of marker variability among 268 adult females revealed little genetic variability within each colony. However, most colonies were clearly distinguished by colony-specific mitochondrial haplotypes (total n = 28). Low intracolony variability and strong haplotype segregation among colonies, was reflected by an extraordinary high FST of 0.68, indicating a very low intercolony dispersal rate of approximately one female in five generations. Haplotype distribution among 18 solitary males showed that males frequently disperse between colony locations, indicating the absence of dispersal barriers. Bechstein's bat maternity colonies are thus closed groups that comprise 20-40 females probably belonging to only one or, at most, two matrilines. The genetic population structure of Bechstein's bats is in agreement with the hypothesis that females seek familiar and, at least, partially related cooperation partners for raising their young. Alternatively strong philopatry might reflect the importance of profound roost or habitat knowledge for successful reproduction in female Bechstein's bats.     

Variability and trends of corn heat units on the Canadian prairies

Seasonal accumulations of corn heat units were calculated from temperature records of 85 stations in Alberta, Saskatchewan, and Manitoba. The number of years of data varied from 16 to 72. The objectives of the study were to determine how many years of data were necessary to obtain a reliable estimate of CHU for each station and to examine possible CHU trends with time. Stein's two-stage sampling technique was used to show that about 20 years of data should be used to obtain a reliable estimate of the location mean. When years were taken at random, the number of years of data needed for a location was larger than when taken in sequence. Statistically significant indications of trends in CHU with time were found for only about 11% of the stations. Year-to-year variability was considerably greater than any possible trends in the last half-century. Means and standard errors of the deviations from the 30-year mean (1943–1972) were calculated for all stations. Although these data were not independent because of their geographic proximity, there was an indication that CHU totals have changed in the past half-century. However, future changes in the CHU totals could not be predicted.     

The role of swarming sites for maintaining gene flow in the brown long-eared bat (Plecotus auritus)

Bat-swarming sites where thousands of individuals meet in late summer were recently proposed as 'hot spots' for gene flow among populations. If, due to female philopatry, nursery colonies are genetically differentiated, and if males and females of different colonies meet at swarming sites, then we would expect lower differentiation of maternally inherited genetic markers among swarming sites and higher genetic diversity within. To test these predictions, we compared genetic variance from three swarming sites to 14 nursery colonies. We analysed biparentally (five nuclear and one sex-linked microsatellite loci) and maternally (mitochondrial D-loop, 550 bp) inherited molecular markers. Three mtDNA D-loop haplolineages that were strictly separated at nursery colonies were mixed at swarming sites. As predicted by the 'extra colony-mating hypothesis', genetic variance among swarming sites (V(ST)) for the D-loop drastically decreased compared to the nursery population genetic variance (V(PT)) (31 and 60%, respectively), and genetic diversity increased at swarming sites. Relatedness was significant at nursery colonies but not at swarming sites, and colony relatedness of juveniles to females was positive but not so to males. This suggests a breakdown of colony borders at swarming sites. Although there is behavioural and physiological evidence for sexual interaction at swarming sites, this does not explain why mating continues throughout the winter. We therefore propose that autumn roaming bats meet at swarming sites across colonies to start mating and, in addition, to renew information about suitable hibernacula.     

Review of human and animal cases of coccidioidomycosis diagnosed in Canada

The first Canadian case of coccidioidomycosis in a human was reported in 1952 and 11 more cases since then. This study provides details of other cases of coccidioidomycosis that have been diagnosed in Canada. Based on clinical details, isolation of Coccidioides immitis, detection of a specific antibody (F band) for coccidioidomycosis by macro- or microimmunodiffusion tests, concurrently used with the complement fixation procedure, and histopathological findings, 116 more cases of this disease were verified. The great majority (94%) of these cases were diagnosed in the western Canadian provinces of British Columbia, Alberta, Saskatchewan and Manitoba, and the others in Quebec, Ontario and Nova Scotia (5,1, and 1 cases, respectively). Available information indicates that the C. immitis infections were contracted during visits to endemic areas in the United States (Arizona, California and New Mexico), Mexico, and Bolivia. Pulmonary infections were the most common type of coccidioidomycosis (93%) followed by the disseminated or meningeal types C. immitis infections occurred in individuals with or without predisposing factor(s) and were more common in males than in females. The exoantigen procedure was very useful and reliable in the accurate and rapid identification of suspected C. immitis isolates. Two cases of coccidioidomycosis were reported in animals in Ontario, Canada.