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At least 19 recordsLinked to original sources
PubMed1989

Inherited diseases in North American Mennonites: focus on Old Colony (Chortitza) Mennonites.

The patterns of migration and the genetic disorders occurring among North American Mennonites are reviewed, and inherited conditions recently recognized in a religious and genetic isolate, the Old Colony (Chortitza) Mennonites, are described. Old Colony Mennonites are of Dutch/German origin and descend from approximately 400 founding families who settled in the Old Colony, Chortitza (the Ukraine, USSR) in the late 1700s, and then migrated to Canada and Central and South America in the past century. We investigated over 6 generations of a Canadian Old Colony kindred in which there was extensive intermarriage, and in whom 28 individuals developed diabetes mellitus. Insulin-dependent diabetes mellitus (IDDM) occurred in 14 affected individuals in 10 closely related sibships; the 11 living IDDM patients were all concordant for the immunogenetic marker HLA-DR4. Fourteen close relatives had other disorders of carbohydrate metabolism, including gestational diabetes and non-insulin-dependent diabetes mellitus. Other close relatives had autoimmune diseases, including rheumatoid arthritis, hyper- and hypothyroidism, multiple sclerosis, and red cell aplasia. Other inherited diseases, including Alport syndrome, congenital defects, and inborn errors of metabolism were also found in the kindred. In the almost exclusively (99%) Old Colony Mennonite public health district in which the kindred was ascertained, there were multiple cases of Tourette syndrome, of malformations (including congenital heart defects and cleft lip +/- palate), and familial clusters of inborn errors of metabolism. We report this Old Colony (Chortitza) Mennonite isolate because 1) there are large familial aggregations of tissue-specific autoimmune diseases, malformations, inborn errors of metabolism, and of some other conditions whose genetic basis is still unknown; 2) there are multiple cases of rare genetic conditions, 3) we have established a computerized genealogic data base on over 1,000 kindred members as well as a cryopreserved lymphocyte/DNA bank on over 100 closely related individuals with various genetic conditions; and 4) this religious isolate, which extends across North, Central, and South America, offers an excellent opportunity for studying the epidemiology and molecular genetics of both common and rare inherited diseases.

Adrenal Hyperplasia, Congenital↗
PubMed1989

Genetic conditions among Canadian Mennonites: evidence for a founder effect among the old colony (Chortitza) Mennonites.

Distinctive disease patterns exist among Canadian Old Colony (Chortitza) Mennonites. This religious and genetic isolate is of 16th century Dutch/German ancestry. The group originated in the Netherlands, then settled in the Vistula delta area of western Prussia for 200 years. A small number of founding families later migrated to Chortitza, the "Old Colony", in the Ukraine in the late 18th/early 19th century, where they remained a distinct genetic isolate. This group has come to Canada over the past 100 years. The more conservative Canadian Mennonites of Chortitza descent practice strict endogamy, have a large family size and live predominantly in rural public health subdistricts in the four western provinces, and in southern Ontario. The world's largest reported familial aggregations of insulin-dependent diabetes mellitus, of autoimmune diseases and of Tourette syndrome were initially ascertained in a small northern Alberta public health subdistrict. Clusterings of malformations, inborn errors of metabolism, and other conditions were also found in the subdistrict, and in group descendents living in other provinces. A founder effect, or genetic drift, accounts for the familial aggregations of autosomal recessive and dominant conditions, some diseases of multifactorial determination, and other inherited conditions in Canadian kinships descending from this ancestral group. The medical literature on genetic conditions among Canadian Mennonites is reviewed and re-evaluated in the light of this information. There is biochemical, serologic, and molecular biologic evidence in favour of genetic homogeneity amongst patients with certain inherited conditions in this special population group. This genetic isolate offers potential for the study of the genetic epidemiology and molecular biology of inherited diseases. A computerized genealogic data base on about 1400 group members, as well as a cryopreserved lymphocyte/DNA bank on over 100 individuals with genetic conditions has been established in this special population group.

Alberta↗
PubMed2002

Evidence of common ancestry for the maple syrup urine disease (MSUD) Y438N allele in non-Mennonite MSUD patients.

Maple syrup urine disease (MSUD) is a rare (1/185,000) autosomal recessive inborn error of branched-chain amino acid metabolism characterized by increased plasma leucine, isoleucine, and valine levels. Though, genetically heterogeneous in the worldwide population, MSUD in Old Order Mennonites (1/150-176) is the result of a tyrosine to asparagine substitution (Y438N; previously Y393N) in the E1alpha subunit of the branched-chain alpha-keto acid dehydrogenase (BCKAD) complex. Due to endogamous practices, the presence of Y438N in all reported Mennonite MSUD patients has historically been attributed to a founder effect. However, we have also identified the Y438N defect in eight MSUD patients of non-Mennonite lineage. To evaluate the genetic origin of this defect in these non-Mennonite patients, we examined Mennonite MSUD families and non-Mennonite MSUD families using microsatellite markers located on chromosome 19q13.1-13.2 (location of E1alpha gene, BCKDHA). Haplotype analyses revealed a major and four minor haplotypes that cosegregate with the Y438N allele in the Old Order Mennonite MSUD patients and carrier relatives. Analyses of eight non-Mennonite MSUD patients reveal that three of the non-Mennonite MSUD patients shared common Mennonite Y438N haplotypes, strongly suggesting Mennonite ancestry. However, the remaining non-Mennonite patients carry Y438N haplotypes that are significantly different from the Mennonite Y438N haplotype, suggesting that the occurrence of the defect in these families is due to either pre-Mennonite or de novo events.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
PubMedDate not supplied

Cardiovascular risk factor status of an Old Order Mennonite community.

BACKGROUND: The Old Order Mennonites are members of rural farming communities whose religious and cultural traditions pervade their dietary and health practices. These cloistered Christian communities often present special challenges to primary care physicians unfamiliar with the Mennonite way of life. Anecdotal descriptions of Mennonite customs suggest heightened risk for cardiovascular disease because of a dairy and meat-based diet and a lack of utilization of the traditional health care system. METHODS: With approval from the church bishop, households from a New York State Mennonite community were interviewed about personal and family history of cardiovascular disease, knowledge of dietary cardiovascular risk factors, and personal awareness of their cardiovascular risk factor status. A standardized Saturated Fat/Cholesterol Avoidance Scale was used to assess qualitatively the community's traditional diet. Blood pressures and total serum cholesterol levels were measured in all willing participants. RESULTS: Two hundred fifty interviews were completed. A significant difference was found between the cholesterol level of the men (181 mg/dL) and the women (192 mg/dL). When compared with the general population, the Mennonite men had significantly lower total serum cholesterol levels and systolic and diastolic blood pressures. Only 8 percent of the study population had been previously screened for serum cholesterol. One-third of the population older than 55 years had a personal history of either myocardial infarction or stroke. The study population's score on the Saturated Fat/Cholesterol Avoidance Scale indicated a diet higher in saturated fat and cholesterol than that of other study populations. CONCLUSIONS: Our findings indicate a significant lack of preventive health care practices, minimal avoidance of dietary cardiovascular risk factors, and a significant difference between men's and women's cardiovascular risk factor status in this Old Order Mennonite population.

Adult↗
PubMed2003

Genetic heritage of the Old Order Mennonites of southeastern Pennsylvania.

The Old Order Mennonites of southeastern Pennsylvania are a religious isolate with origins in 16th-century Switzerland. The Swiss Mennonites immigrated to Pennsylvania over a 50-year period in the early 18th century. The history of this population in the United States provides insight into the increased incidence of several genetic diseases, most notably maple syrup urine disease (MSUD), Hirschsprung disease (HSCR), and congenital nephrotic syndrome. A comparison between the Old Order Mennonites and the Old Order Amish demonstrates the unique genetic heritage of each group despite a common religious and geographic history. Unexpectedly, several diseases in both groups demonstrate allelic and/or locus heterogeneity. The population genetics of the 1312T --> A BCKDHA gene mutation, which causes classical MSUD, are presented in detail. The incidence of MSUD in the Old Order Mennonites is estimated to be 1/358 births, yielding a corrected carrier frequency of 7.96% and a mutation allele frequency of 4.15%. Analysis of the population demonstrates that repeated cycles of sampling effects, population bottlenecks, and subsequent genetic drift were important in shaping the current allele frequencies. A linkage disequilibrium analysis of 1312T --> A mutation haplotypes is provided and discussed in the context of the known genealogical history of the population. Finally, data from microsatellite marker genotyping within the Old Order Mennonite population are provided that show a significant but modest decrease in genetic diversity and elevated levels of background linkage disequilibrium.

Chromosomes, Human, Pair 19↗
PubMed1999

Prevalence of mild apparent mineralocorticoid excess in Mennonites.

UNLABELLED: We have studied an unusual patient with mild low-renin hypertension due to a homozygous mutation in the HSD11B2 gene (PNAS 95:10200-10205, 1998). The patient came from an inbred Mennonite family, and though the mutation identified her as an AME patient, she had a normal birth weight and did not demonstrate the typical features of AME, such as hypokalemic alkalosis, low birth weight, failure to thrive, poor growth, and in many cases nephrocalcinosis. Biochemically, typical patients with AME have abnormal cortisol metabolites and an exceedingly diminished ability to convert [11-3H]cortisol to cortisone. In this patient with mild AME, the conversion of cortisol to cortisone was 58% compared to 0 to 6% in typical AME patients, while the normal conversion is 90 to 95%. Molecular analysis of the HSD11B2 gene of this patient showed a homozygous mutation in codon 227 (P227L). We studied this Mennonite population for the prevalence of the P227L mutation. Our hypothesis was that this mild form of AME would be prevalent in the somewhat inbred Mennonite population to which the patient belongs. Our proposed study was 1) to determine if there are other cases of this mild form of AME, and 2) to establish the heterozygote frequency of the mutation in the Mennonites. RESULTS: We did not detect any additional cases of mild AME. We detected 15 carriers of the P227L mutation out of 445 Mennonites, resulting in a heterozygote frequency of 0.03. CONCLUSION: Since this is an inbred population, the chance of two heterozygotes marrying would be 0.001, which is 1 in 1000 people. This population is known to have large families and therefore the possibility of having an affected child is high. The population consists of 2000 members and we have discovered one affected patient. Thus, there might be one other patient in this population.

11-beta-Hydroxysteroid Dehydrogenases↗
PubMed1998

Dietary cardiovascular risk factors and serum cholesterol in an Old Order Mennonite community.

OBJECTIVES: Dietary and coronary heart disease risk factors in Old Order Mennonite men and women were examined. METHODS: A food frequency questionnaire was mailed to 250 Mennonites who had participated in a previous study of coronary heart disease risk factors. RESULTS: Mennonites consumed a diet high in total fat, saturated fats, and cholesterol. Men had lower average serum cholesterol levels (174 mg/dL) than women (191 mg/dL). CONCLUSIONS: The Mennonites' diet was similar to that of the overall US population in saturated fat percentage and higher in cholesterol. Serum cholesterol levels, adiposity, and blood pressure were lower than expected among Mennonite men, perhaps because of their higher levels of physical activity.

Adolescent↗
PubMed1991

Occurrence of a Tyr393----Asn (Y393N) mutation in the E1 alpha gene of the branched-chain alpha-keto acid dehydrogenase complex in maple syrup urine disease patients from a Mennonite population.

Maple syrup urine disease (MSUD) is caused by a deficiency in the mitochondrial branched-chain alpha-keto acid dehydrogenase complex. The incidence of MSUD in the Philadelphia Mennonites is 1/176 births resulting from consanguinity. In this study, we amplified cDNAs for the decarboxylase E1 alpha subunit of the branched-chain alpha-keto acid dehydrogenase complex from a classical MSUD patient and from an obligatory heterozygote of a Mennonite family by the PCR. Sequencing of the amplified cDNAs disclosed at codon 393 of the mature E1 alpha polypeptide a base substitution changing a tyrosine (encoded by TAC) to an asparagine residue (encoded by AAC), which is designated Y393N. A segment of the E1 alpha gene containing the 5' portion of exon 9 was amplified. Probing of the amplified genomic DNA with allele-specific oligonucleotide probes showed that the mutation in the E1 alpha gene was homozygous in six Mennonites affected with classical MSUD and was present in heterozygous carriers. The identification of the MSUD mutation in the Philadelphia Mennonites will facilitate diagnosis and carrier detection for this population.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
PubMed1990

High fertility of Old Colony Mennonites in Mexico.

Old Colony Mennonites in Mexico appear to demonstrate natural fertility, using no form of artificial birth control and apparently not attempting to limit family size. The resulting fertility is nearly as high as that of the Hutterites, although the Mennonites lack the communal economic system of the latter. Most Mennonites in Mexico migrated from Canada in the 1920s, and the largest single settlement, called the Manitoba Colony, is one of four in the state of Chihuahua. A 1967 partial census obtained data from 38% of the Mennonite households. Family size in the sample was close to that in a local survey taken in the same year. Available church records matched with census forms permitted verification of and corrections to 560 female reproductive histories. The median number of live births to women over age 45 years was 9.5, compared with 10.4 in the Hutterites. Age-specific marital fertility rates and birth intervals closely resembled those of the Hutterites.

Adolescent↗
PubMed1994

Efficacy of a composite biological age score to predict ten-year survival among Kansas and Nebraska Mennonites.

In 1980 and 1981 Mennonite descendants of a group of Russian immigrants participated in a multidisciplinary study of biological aging. The Mennonites live in Goessel, Kansas, and Henderson, Nebraska. In 1991 the survival status of the participants was documented by each church secretary. Data are available for 1009 individuals, 177 of whom are now deceased. They ranged from 20 to 95 years in age when the data were collected. Biological ages were computed using a stepwise multiple regression procedure based on 38 variables previously identified as being related to survival, with chronological age as the dependent variable. Standardized residuals place participants in either a predicted-younger or a predicted-older group. The independence of the variables biological age and survival status is tested with the chi-square statistic. The significance of biological age differences between surviving and deceased Mennonites is determined by t test values. The two statistics provide consistent results. Predicted age group classification and survival status are related. The group of deceased participants is generally predicted to be older than the group of surviving participants, although neither statistic is significant for all subgroups of Mennonites. In most cases, however, individuals in the predicted-older groups are at a relatively higher risk of dying compared with those in the predicted-younger groups, although the increased risk is not always significant.

Adult↗
PubMedDate not supplied

Apolipoproteins (apoproteins) and LPL variation in Mennonite populations of Kansas and Nebraska.

Apoproteins (also known as apolipoproteins) have been studied extensively because of their role in lipid transport, association between specific genotypes and elevated serum lipid levels, and increased risk of heart disease. There is considerable genetic variation in the geographic distributions of these markers, with a north-south cline of the APOE*4 allele observed in Europe by Lucotte et al. ([1997] Hum Biol 69:253-262). This study compares the frequencies of seven APO (APOA1 -75 bp, APOA1 +83 bp, APOB Ins/Del, APOB XbaI, APOC3 SstI, and APOE) and LPL loci in Mennonite populations from Kansas and Nebraska. In total, 277 individuals were sampled from Goessel, Meridian, Garden View, and Lone Tree in 2002-2004. In addition, DNA samples that were collected in 1981 from Henderson, Nebraska, were genotyped for the seven APO and LPL loci. Of the seven APO and LPL loci tested, only one locus, APOB XbaI, departed significantly from Hardy-Weinberg equilibrium, with an unexpected excess of observed heterozygotes. The frequencies of the several APO loci are unique among the Mennonites, separating them from other European populations. A bidimensional scaling representation of Reynold's co-ancestry distances based on allelic frequencies of the seven APO and LPL markers in five Mennonite congregations fails to represent schematically the known patterns of fission. It is unclear whether the observed patterns are due to selection operating on these loci or whether genetic drift, small populations sizes, or a lack of statistical power of these biallelic loci distort the observed genetic relationship among congregations.

Apolipoproteins↗
PubMed1992

Canadian Mennonites and individuals residing in the Friesland region of The Netherlands share the same molecular basis of 17 alpha-hydroxylase deficiency.

A common mutation within the CYP17 gene that causes 17 alpha-hydroxylase deficiency, a form of congenital adrenal hyperplasia, has been found by direct sequencing of polymerase chain reaction (PCR) fragments of genomic DNA from six families residing in the Friesland region of the Netherlands. The mutation is a 4-base duplication within exon 8 of the CYP17 gene, which alters the reading frame encoding the C-terminal 26 amino acids of cytochrome P45017 alpha. This mutation has previously been found in two Canadian patients who are members of ostensibly unrelated Mennonite families. The Mennonite Churches derive their name from Menno Simons, an early leader of the sect in Friesland. Presumably this 4-base duplication appeared within the Friesian population prior to emigration of the Mennonites from the Netherlands.

Adrenal Hyperplasia, Congenital↗
PubMed1992

Gene analysis of Mennonite maple syrup urine disease kindred using primer-specified restriction map modification.

Maple syrup urine disease (MSUD) is an autosomal recessive inherited disease due to a deficiency of any of the subunits, E1 alpha, E1 beta or E2, of the branched-chain alpha-ketoacid dehydrogenase complex (BCKDH). A large Mennonite kindred of MSUD has been studied in Pennsylvania, USA. In the present investigation, genomes from 70 members, including 12 patients belonging to eight different Mennonite MSUD pedigrees, were examined for possible abnormalities in the E1 alpha gene of BCKDH, by primer-specified restriction map modification. A T-to-A substitution which generates an asparagine in place of a tyrosine at amino acid 394 of the mature E1 alpha subunit was present in both alleles in all the patients and in a single allele in all obligate carriers and several siblings. We describe a new technique for rapid and easy detection of the mutant gene in this population. These family studies provide additional evidence that Mennonite MSUD is caused by a missense mutation of the E1 alpha gene of BCKDH

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
PubMed2006

Addressing health disparities in highly specialized minority populations: case study of Mexican Mennonite farmworkers.

The Kansas Statewide Farmworker Health Program (KSFHP) has developed a unique set of culturally competent health interventions in response to the pressing public health needs of the state's underserved farmworker population. Key among these are its health education and translation efforts on behalf of the fast-growing Low German-speaking Mexican Mennonite farmworker population. Linguistic, religious, and cultural values have created unique and complex health disparities and barriers to care that can be broken down only through innovative approaches. KSFHP first conducted a health needs assessment survey of the farmworker population in 2003, which indicated prenatal care practices as a significant health disparity, especially among the Low German-speaking Mexican Mennonite population. In response, KSFHP successfully lobbied the state health department to implement a new standard of health behavior data collection that includes primary language data as a method of delineating population subgroups, making Kansas one of the first two states in the country to collect this information. KSFHP also developed culturally competent Low German-language recordings on health topics such as prenatal care in accordance with the information delivery needs of the Low German-speaking Mexican Mennonite farmworker population. Currently, a pilot program is in progress that offers additional outreach, health education, and interpretation, among other services. The work of the KSFHP has significant implications for further research into health disparities, specialized minority populations, and culturally competent data collection methods.

Adolescent↗
PubMed2001

Carrier detection and rapid newborn diagnostic test for the common Y393N maple syrup urine disease allele by PCR-RFLP: culturally permissible testing in the Mennonite community.

The turnaround time for diagnosis of maple syrup urine disease (MSUD) by classic serum amino acid analyses often requires 3-4 days. This is due to the need for branched-chain amino acids (BCAA) to accumulate in the serum of the newborn before testing. The accumulation of BCAAs in infants with MSUD during this time increases the risk of the infant becoming clinically symptomatic. We have developed a noninvasive DNA-based mismatch PCR-RFLP assay for the Y393N BCKDHA allele (E1alpha gene of the branched chain alpha-keto acid dehydrogenase complex), the primary cause of MSUD in Old Order Mennonite communities. The homozygosity and high frequency of this mutation in the Mennonite community and its prevalence in compound heterozygote non-Mennonite MSUD patients is of significance. We describe carrier testing, present the results of nine newborns diagnostically evaluated for the Y393N BCKDHA allele, and demonstrate the efficacy of this PCR-RFLP assay for determining clinical status within 24 h after birth. Analyses within the first 24 h of life allow for immediate diagnosis and treatment of infants homozygous for the Y393N MSUD defect. This is a significant improvement over the time required by current serum amino acid analysis methods.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
PubMed1993

Stable correction of maple syrup urine disease in cells from a Mennonite patient by retroviral-mediated gene transfer.

We have successfully used retroviral gene transfer to correct the deficiency of the branched-chain alpha-oxo acid dehydrogenase complex in lymphoblasts from a homozygous Mennonite maple syrup urine disease (MSUD) patient. The mutation in Mennonites is a Tyr-393 to Asn substitution in the branched-chain alpha-oxo acid decarboxylase (E1)alpha subunit of the enzyme complex. This promotes improper assembly of mutant E1 alpha with E1 beta subunits, leading to degradation of both polypeptides. For transduction studies, a full-length human E1 alpha CDNA was inserted into the retroviral vector LXSN to produce the recombinant LSN-E1 alpha. High-titre [6 x 10(5) colony-forming units/ml] amphotropic retroviral preparations free of helper viruses were obtained by co-cultivation of infected GP+E86 with PA317 cells. Transduction of MSUD lymphoblasts from the Mennonite patient with LSN-E1 alpha viruses restored the decarboxylation of alpha-oxo[1-14C]isovalerate to the normal level. The normal decarboxylation activity in transduced MSUD cells remained stable without G418 selection during the 14 weeks studied. Southern-blot analysis indicated that the recombinant E1 alpha cDNA was integrated into the host genome. Northern and Western blotting showed that both the normal E1 alpha mRNA and the subunit were properly expressed in transduced MSUD cells. However, the level of E1 beta subunits is lower than that of normal cells, suggesting competition of the recombinant E1 alpha with the mutant form for assembly with E1 beta. The results provide a paradigm for the development of somatic gene therapy for disorders involving mitochondrial multienzyme complexes.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
PubMed1989

Genetic structure of Mennonite populations of Kansas and Nebraska.

We describe the gene frequency distributions for 29 different blood group, serum, and erythrocytic proteins for three Mennonite communities from Kansas and Nebraska and compare their gene frequencies with those of Amish, Hutterite, and Mennonite populations using the topological method of Harpending and Jenkins (1973). Subdivision of these communities into congregations reveals that the "fission-fusion'h model best characterizes the relationship between the genetic patterns and historical events. These Mennonite populations, although reproductively isolated at the turn of this century, are presently entering the mainstream of US rural culture.

Blood Grouping and Crossmatching↗