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At least 19 recordsLinked to original sources

Comparison of bone parameters by dual-energy X-ray absorptiometry and peripheral quantitative computed tomography in Hutterite vs. non-Hutterite women aged 35-60 years.

A previous report of elevated dual-energy X-ray absorptiometry (DXA) bone mineral density (BMD) Z scores suggests that Hutterite females might be significantly less likely to develop osteoporosis compared with other U.S. females. In the present study, we sought to determine if high Hutterite DXA BMD Z scores were elevated because of larger bone size. Hutterites reside in isolated, self-sufficient colonies with an emphasis on agricultural production, and girls enter a strenuous task rotation at age 15 years. We obtained cross-sectional bone measurements of the 66% distal tibia using peripheral quantitative computed tomography (pQCT) to compare bone size and geometry on 97 Hutterite and 30 non-Hutterite women, aged 35-60 years. Total body (TB) and lumbar bone mineral content (BMC), BMD, and bone area measurements by DXA were available on a subset of the study population. We identified no differences between groups in pQCT total bone area, cortical bone area, or cortical bone density. Larger bone area by DXA was apparent in Hutterites compared with non-Hutterites at the TB (least square means: 2038 +/- 8 cm2 vs. 1953 +/- 19 cm2, p < 0.05) and lumbar (least square means: 58 +/- 0.5 cm2 vs. 57 +/- 2 cm2, p < 0.01) sites. TB BMC adjusted for TB bone area was marginally higher in Hutterites compared with non-Hutterites (least square means: 2341 +/- 15 g vs. 2281 +/- 30 g, p = 0.08). Hutterites had marginally higher TB BMD Z scores when controlling for weight and age (least square means: 1.3 +/- 0.1 vs. 0.8 +/- 0.2, p = 0.07). Hutterites had higher lumbar BMC adjusted for lumbar bone area and weight (least square means: 65 +/- 1 g vs. 58 +/- 2 g, p < 0.01) and higher weight-and age-adjusted lumbar BMD Z scores (least square means: 1.1 +/- 0.1 vs. 0.1 +/- 0.4, p = 0.01). Our data indicate that a true advantage in trabecular bone density probably exists among Hutterite women aged 35-60 years. Hutterite women might be protected against age-related fractures because of their larger bone size and higher bone density at normally susceptible trabecular sites.

Absorptiometry, Photon↗

Respiratory symptoms and asthma in two farming populations: a comparison of Hutterite and non-Hutterite children.

OBJECTIVE: To determine the prevalence of asthma and respiratory symptoms in a farming population of Hutterite and non-Hutterite children. PARTICIPANTS AND SETTING: A population of 830 school-age, farm-dwelling children in rural Saskatchewan that included 83 children residing in Hutterite farming colonies. METHODS: A cross-sectional survey questionnaire was sent to parents of children attending grades 1 to 5 in schools within a grain-growing and mixed-farming region of central Saskatchewan. The questionnaire was used to identify respiratory symptoms, environmental and host characteristics, and reported doctor-diagnosed asthma. RESULTS: The prevalence of asthma in Hutterite children was 2.4% compared with 9.2% in non-Hutterite children. While Hutterite children did not differ from their farming counterparts with regard to the frequency of reported respiratory allergies, they were less likely to be exposed to cigarette smoke and to participate in farm-related activities. In the multivariate analysis, being Hutterite continued to be protective for asthma (odds ratio 0.21; 95% CI 0.05 to 0.89). CONCLUSION: The observed lower rate of asthma in Hutterite children than in non-Hutterite children may be related to both genetic factors and differences in farming and household factors between the two groups. Key Words: Asthma; Children; Farming; Hutterite

Asthma↗

Recent fertility decline in Dariusleut Hutterites: an extension of Eaton and Mayer's Hutterite fertility study.

A church book that has been routinely updated by the Dariusleut Hutterites enabled us to update some fertility tables presented by Eaton and Mayer in the 1950s. The age-specific (nuptial) fertility rates and the total fertility rates (TFRs) were calculated for every 5-year period from 1901-1905 to 1981-1985. Our calculations for Dariusleut, one of the three sects of the Hutterites, gave slightly lower age-specific nuptial fertility rates before 1951 compared with the figures given by Eaton and Mayer (1953) for all Hutterites in the corresponding time periods. The recent decline in Hutterite fertility, especially at higher maternal ages, was confirmed in this study. The TFR, given as a sum of 5-year-grouped age-specific nuptial fertility rates from 15 to 49 years of maternal age, was higher (8.80-9.83) in the years 1946-1965, but it declined to 8.13 in 1966-1970, to 7.22 in 1971-1975, to 6.39 in 1976-1980, and to 6.29 in 1981-1985. For the age groups 35-39 years and older the age-specific nuptial fertility rate in 1981-1985 decreased by more than 50% compared with the rate during the peak period of 1951-1955. The probability of a married woman having a live birth at each age from 15 to 49 years still peaked around the twentieth year of life, as found by Eaton and Mayer (1953), but it decreased more rapidly from the late twenties among the recently married women, most evidently in their thirties.

Adolescent↗

Hutterite cerebro-osteo-nephrodysplasia: autosomal recessive trait in a Lehrerleut Hutterite family from Montana.

We are reporting on two Lehrerleut Hutterite sisters who have a syndrome of congenital shortness with mild spondylorhizomelic dwarfism; later failure to thrive, ie deceleration of weight gain presumably due to CNS-based severe feeding problems; a CNS defect, probably developmental (not biochemical) with normal prenatal brain growth but later deceleration from 50th to 2nd centile associated with severe mental retardation and decorticate disturbances of neurologic function; and possible renal involvement with terminal nephrotic syndrome. This seems to be a previously undescribed pleiotropic autosomal recessive trait.

Abnormalities, Multiple↗

Relationships between bone mass and circulating leptin concentrations in Hutterites.

A limited number of previous studies have shown inverse associations between bone mass or density and circulating leptin in humans. Relationships between bone mass and circulating leptin in Hutterites, who have elevated bone density, are unknown. Knowledge gained from studies in mice suggests that Hutterites exhibit traits consistent with a deficiency in circulating leptin or in leptin signaling. We examined relationships between whole body (WB) and regional (lumbar, total hip, femoral neck, trochanter) bone mineral content (BMC) by dual energy X-ray absorptiometry and circulating leptin in 249 Hutterites (137 female) ages 20-55 years and 72 similarly aged non-Hutterites (37 female). We tested the hypothesis that (1) Hutterites will have low circulating leptin concentrations for a given amount of body fat compared to non-Hutterites, and (2) controlling for body fat, there will be an inverse relationship between BMC and circulating leptin among Hutterites. Hutterites had higher BMC than non-Hutterites at all skeletal sites after adjusting for site-specific bone area, age, and sex (P </= 0.02). Hutterite females had higher leptin concentrations than non-Hutterite females [geometric mean and 95% confidence interval (CI): 18.38 (17.18, 19.67) vs. 14.30 (12.55, 16.28), P </= 0.001) after adjusting for WB fat mass. Hutterite males also had higher leptin concentrations than non-Hutterite males [geometric mean and 95% CI: 6.53 (6.11, 6.98) vs. 5.62 (4.98, 6.35), P = 0.03) after adjusting for WB fat mass. We used backward stepwise regression to determine significant (P </= 0.10) covariates to include in models predicting WB and regional BMC among Hutterites (separately by sex). Subsequently, we entered leptin (log-transformed) to models to test for significance (P </= 0.05). After adjusting for covariates, leptin concentration was not a significant predictor of BMC at any site, in either sex, among Hutterites. It is possible that genetic influences that interfere with hypothalamic leptin signaling, in a manner unrelated to adipocyte leptin production, contribute to elevated Hutterite bone density.

Adult↗

Low prevalence of psychoses among the Hutterites, an isolated religious community.

OBJECTIVE: The authors estimated the prevalence of psychoses among the Hutterites in Manitoba, Canada, who lived in 102 communal farms or colonies. The study stemmed from an earlier epidemiological survey of North American Hutterite colonies (1950-1953), in which a low prevalence of psychoses was documented. METHOD: Psychiatrically ill individuals identified during the previous survey were rediagnosed with DSM-IV criteria. A current provincial health insurance claims database was queried anonymously for the period June 1992-May 1997, and the prevalence rate of disease among Hutterites, identified by distinctive surnames and unique postal addresses, was compared with the rate in the entire population of the province of Manitoba and in a comparison group of persons with Hutterite surnames but with addresses outside the Hutterite colonies. RESULTS: The annual prevalence of schizophrenia among the communal Hutterites, estimated from the database search by using ICD-9 criteria, was consistent with the prevalence found in the prior epidemiological survey (annual mean of 1.2/1,000 population, compared with 1.3/1,000 in the prior survey). The database search yielded a significantly lower prevalence for schizophrenia and other functional psychoses among communal Hutterites as well as among the comparison group, compared to the total Manitoba population. There was also lower prevalence for affective psychoses and adjustment reaction disorders among the communal Hutterites, compared to the total Manitoba population. Rates for neurotic disorders were elevated both among the communal Hutterites and the comparison group. CONCLUSIONS: The prevalence of specific psychoses was reduced among the Hutterites, although neurotic disorders were more prevalent. These findings suggest some specificity, although possible artifacts such as ascertainment bias must be considered. Further research is needed to examine genetic and environmental factors that may contribute to reduced prevalence of specific psychoses among the Hutterites.

Age of Onset↗

Influence of age, sex, and obesity on blood pressure of Hutterites in South Dakota.

Anthropometric and blood pressure data were evaluated in 724 male and female Hutterites and 273 randomly sampled male and female subjects in southeastern South Dakota who served as the control group. Hutterite males and females showed significant age-dependent increases of both weight and obesity (p less than 0.001) not seen in the control group. Both systolic and diastolic blood pressures (SP and DP) of the Hutterites were higher than those of the control subjects (p less than 0.001). There were highly significant correlations between age, weight, obesity (all p less than 0.001), and SP and DP of the Hutterites. Correlations between the weight and SP and DP were significant (p less than 0.001) in the control groups. The prevalence of systolic blood pressures greater than or equal to 140 mm Hg among Hutterite males ranged from 34.6 to 79.3 percent in age groups by decade vs 8.7 to 34.2 percent in age-matched control males. The prevalence of diastolic blood pressure levels greater than or equal to 90 mm Hg ranged from 0 percent in the youngest (less than or equal to 29 years) to 7.9 percent oldest age group (60+ years) of control males. The prevalence of elevated DP among male Hutterites ranged from 5.9 to 37.9 percent in corresponding age groups. A similar age-dependent trend of prevalence rates of elevated DP and SP were noted in female Hutterites but to a lesser extent than in control subjects. Elevated blood pressure levels among Hutterites may be an important contributing risk factor to the younger mean age of death of Hutterites compared to the control population.

Adolescent↗

The age pattern of fecundability: an analysis of French Canadian and Hutterite birth histories.

This paper analyzes the age pattern of effective fecundability from populations with no evidence of deliberate fertility control using a new convolution model of fecundability. The analysis is based on a sample of Hutterite birth histories from the mid-20th century, and birth histories of French Canadians from the 17th and 18th centuries. The main findings are as follows: 1) the level of effective fecundability is higher among the French Canadians compared to the Hutterites; 2) effective fecundability peaks at age 20 for the Hutterites, and in the early to mid-20s for the French Canadians; 3) Hutterite effective fecundability declines almost linearly from age 20 to 45, and French Canadian effective fecundability declines slowly from its peak to the early 30s, and more rapidly at older ages; and 4) the duration of postpartum amenorrhea is longer for the French Canadians than for the Hutterites. Because of the shorter periods of postpartum amenorrhea the Hutterites have about the same average number of children as the French Canadians, even though the French Canadians have higher effective fecundability.

Adolescent↗

Developing cultural competence in the baccalaureate nursing student: a population-based project with the Hutterites.

The purpose of this article is to articulate the process of educating baccalaureate students to apply culturally competent, population-based nursing care to the Hutterites. The Hutterites are a German dialect-speaking, ethnoreligious population. Campinha-Bacote's culturally competent model guided the student learning process. Groups of students developed culture-specific, population-based assessment models to identify the health-related needs of the Hutterites. One example of a Hutterite health assessment model that was derived from Dever's epidemiologic model and Purnell and Paulanka's model for cultural competence is presented. Priority health needs of Hutterite colonies are described, and examples of health programs are given. Recommendations for working with the Hutterite population as part of an undergraduate public health nursing course are put forth. The culturally congruent process of teaching and learning for nursing students can be applied to other health-related professions and adapted to other cultural contexts.

Clinical Competence↗

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.

Base Sequence↗

A comparison of physical and laboratory measures between two Hutterite leute and the rural Saskatchewan population.

This study compares physical, laboratory and anthropometric measurements from each of two groups (leute) of Hutterites (N = 846) with a population of non-Hutterites from rural Saskatchewan (N = 750). Marked interleute differences were observed in the comparative analysis with the non-Hutterite population. Dariusleut males had significantly greater mean diastolic and systolic blood pressures, total cholesterols, LDL cholesterols and Body Mass Indexes (BMI) than non-Hutterites. Compared with non-Hutterite women, the Dariusleut females had significantly greater mean diastolic and systolic blood pressure, HDL cholesterols, BMIs and waist-hip ratios. In contrast, the only significant difference between non-Hutterites and Lehrerleut males was in BMI. Lehrerleut females had significantly greater mean systolic blood pressures, HDL cholesterol levels and BMIs. While the surprising inter-leute differences may be due to subtle variations in lifestyle, the genetic isolation of the groups suggests heredity may offer a more likely explanation for the differences.

Adolescent↗

Hepatic carnitine palmitoyl transferase 1 (CPT1 A) deficiency in North American Hutterites (Canadian and American): evidence for a founder effect and results of a pilot study on a DNA-based newborn screening program.

We describe six patients with hepatic carnitine palmitoyl transferase (CPT1 A) deficiency who are members of a large extended Hutterite kindred living in widely scattered communities in the United States and Canadian Prairies. Two patients have significant neurological impairment due to severe recurrent hypoglycemic crises. The remaining four patients with earlier detection and treatment have near normal outcomes. The Canadian and American Hutterite families share two common ancestors who married in 1812, about 60 years before the Hutterites arrived in North America and prior to their subdivision into the three groups (Schmiedeleut, Dariusleut, and the Lehrerleut). These patients share a common haplotype on chromosome 11q13 and are all homozygous for a common CPT1 A G710E mutation, suggesting a founder effect. The clustering of such a rare disorder of fatty acid oxidation prompted us to initiate a pilot DNA-based neonatal screening program to determine the carrier frequency of this mutation in Hutterite newborns with the participation and support of the community. To date our carrier frequency is 1/16, close to the predicted frequency based on diagnosed patients and number of births. We believe our newborn screening program for CPT1 A deficiency in the Hutterite community will serve as a prototype model for delivery of targeted genetic services to other similar unique genetic isolates.

Adolescent↗

The occurrence of multiple sclerosis in the Hutterites of North America.

OBJECTIVE: To report the occurrence, clinical characteristics and genealogical analysis of multiple sclerosis in the Hutterites of North-Western United States and Western Canada. BACKGROUND: The incidence of multiple sclerosis is reported to be lower or rare in certain ethnic groups and genetic isolates and was previously observed to be absent in the Hutterite population. METHODS: After long-term surveillance, six patients were identified and clinical examinations and laboratory investigations including VER and MRI were completed. RESULTS: The six cases included two brothers, two first cousins, male and female, another male and female, all representing two of the three endogamous groups of Hutterites, are linked to two common ancestors through lines of descent dating to 1723. The individual pedigrees were analyzed from extensive genealogical records covering eight generations. CONCLUSION: The incidence of multiple sclerosis in Hutterites is low in a high risk area of North America. A specific mode of inheritance pattern has not been established and a common founder effect may play a role in the development of multiple sclerosis. The genetic contribution of the Hutterites seems greater than previously recognized.

Adult↗

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.

Adult↗

Identification of the M1101K mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and complete detection of cystic fibrosis mutations in the Hutterite population.

The Hutterite population is a genetic isolate with an increased incidence of cystic fibrosis (CF). Previously we identified three CF haplotypes defined by polymorphisms flanking the CF transmembrane conductance regulator (CFTR) gene. delta F508 was present on one of the haplotypes in only 35% of CF chromosomes. We hypothesized that the other two CF haplotypes, one of which was the most common and the other of which is rare, each harbored different non-delta F508 mutations. Single-strand conformation polymorphism analysis detected a missense mutation, M1101K, in both chromosomes of a Hutterite patient carrying the two non-delta F508 haplotypes. M1101K appears to have originated on an uncommon CFTR allele and to be infrequent outside the Hutterite population. The presence of M1101K on two haplotypes is likely the result of a CFTR intragenic recombination which occurred since the founding, 10-12 generations ago, of the Hutterite population. The crossover was located between exons 14a and 17b, an interval of approximately 15 kbp. delta F508 and M1101K accounted for all of the CF mutations in patients from 16 CF families representing the three subdivisions of the Hutterite population.

Adolescent↗

Congenital anomalies in the Hutterite population: a preliminary survey and hypothesis.

The Alberta Provincial Congenital Anomaly (CA) Surveillance reporting forms were scanned for infants whose surnames and addresses identified them as belonging to the Hutterite Brethren. Death registrations (of infants up to 1 year) and stillbirth registrations were similarly scanned. While the overall percentage of total malformations (5%) and major malformations (2%) was no different from that of the total population of Alberta, closer examination of the actual entities showed a large number of monogenic disorders among the Hutterites. The frequency of multifactorial congenital anomalies was approximately 1%. The inbreeding coefficients, using a genealogic data base, were computed for each "case baby" and two Hutterite "control babies," the latter being births preceding and succeeding the case baby. There was no statistical difference in the distribution of inbreeding coefficients between the case and control groups. While the data are based on small numbers and therefore are preliminary, they suggest that the Hutterite lifestyle, of good nutrition (using largely home prepared foods), absence of tobacco and minimal alcohol consumption, may be one factor responsible for fewer multifactorial CAs whose occurrence is environmentally susceptible to such influences. Studies of populations with a low incidence of disorders are just as important as those with a high incidence.

Alberta↗

The most common mutation in FKRP causing limb girdle muscular dystrophy type 2I (LGMD2I) may have occurred only once and is present in Hutterites and other populations.

Limb girdle muscular dystrophy (LGMD) is common in the Hutterite population of North America. We previously identified a mutation in the TRIM32 gene in chromosome region 9q32, causing LGMD2H in approximately two-thirds of the 60 Hutterite LGMD patients studied to date. A genomewide scan was undertaken in five families who did not show linkage to the LGMD2H locus on chromosome 9. A second LGMD locus, LGMD2I, was identified in chromosome region 19q13.3, and the causative mutation was identified as c.826C>A (L276I), a missense mutation in the FKRP gene. A comparison of the clinical characteristics of the two LGMD patient groups in this population reveals some differences. LGMD2I patients generally have an earlier age at diagnosis, a more severe course, and higher serum creatine kinase (CK) levels. In addition, some of these patients show calf hypertrophy, cardiac symptoms, and severe reactions to general anesthesia. None of these features are present among LGMD2H patients. A single common haplotype surrounding the FKRP gene was identified in the Hutterite LGMD2I patients. An identical core haplotype was also identified in 19 other non-Hutterite LGMD2I patients from Europe, Canada, and Brazil. The occurrence of this mutation on a common core haplotype suggests that L276I is a founder mutation that is dispersed among populations of European origin.

Chromosome Mapping↗

Ancestral and recombinant 16-locus HLA haplotypes in the Hutterites.

Prior studies in the Schmiedeleut Hutterites of South Dakota have demonstrated associations between human leukocyte antigen (HLA) haplotype matching and fetal loss (Ober et al. 1992) and mate preferences (Ober et al. 1997), as well as deficiencies of homozygotes for HLA haplotypes (Kostyu et al. 1993). These studies were based on the serologically-defined five-locus HLA-A, -C, -B, -DR, -DQ haplotype. To further elucidate the effects of specific major histocompatibility (MHC) loci or regions on fetal loss and mate choice, we genotyped a sample of Hutterites for 14 MHC loci by DNA or biochemical methods. Typing for additional loci in the HLA-A to HLA-DPB1 region increased the number of recognized Hutterite MHC haplotypes to 67, and further localized the site of crossover in 9 of 15 recombinant haplotypes. Hutterite MHC haplotype sequences are similar to those observed in outbred Caucasians, suggesting that the influence of HLA haplotypes on fetal loss and mating structure may be general.

Alleles↗