Article

Inherited diseases in North American Mennonites: Focus on old colony (Chortitza) Mennonites

  1. Dr. M. A. Jaworski1,*,
  2. A. Severini1,
  3. G. Mansour1,
  4. K. Hennig1,
  5. J. D. Slater1,
  6. R. Jeske1,
  7. J. Schlaut2,
  8. J. W. Yoon3,
  9. N. K. Maclaren4,
  10. G. T. Nepom5,
  11. John M. Opitz Editor,
  12. James F. Reynolds Editor

Article first published online: 5 JUN 2005

DOI: 10.1002/ajmg.1320320204

Issue

American Journal of Medical Genetics

American Journal of Medical Genetics

Volume 32, Issue 2, pages 158–168, February 1989

Additional Information

How to Cite

Jaworski, M. A., Severini, A., Mansour, G., Hennig, K., Slater, J. D., Jeske, R., Schlaut, J., Yoon, J. W., Maclaren, N. K., Nepom, G. T., Opitz, J. M. and Reynolds, J. F. (1989), Inherited diseases in North American Mennonites: Focus on old colony (Chortitza) Mennonites. Am. J. Med. Genet., 32: 158–168. doi: 10.1002/ajmg.1320320204

Author Information

  1. 1

    Department of Pediatrics and Muttart Diabetes Research and Training Centre, University of Alberta, Canada

  2. 2

    Canadian Red Cross Transfusion Services/University of Alberta Hospitals Tissue Typing Laboratory, Canada

  3. 3

    Edmonton; and Department of Microbiology and Infectious Diseases, University of Calgary, Calgary Alberta, Canada

  4. 4

    Department of Pathology, University of Florida, Gainsville, Florida

  5. 5

    Virginia Mason Research Centre, University of Washington School of Medicine, Seattle

*Ottawa Centre, Canadian Red Cross Society, 180 Plymouth Street, Ottawa, Canada K1S 3E2

Publication History

  1. Issue published online: 5 JUN 2005
  2. Article first published online: 5 JUN 2005
  3. Manuscript Revised: 23 JUN 1988
  4. Manuscript Received: 16 FEB 1988

Funded by

  • Alberta Heritage Foundation for Medical Research
  • Diabetes Canada
  • Diabetes Canada and the Alberta Heritage Foundation for Medical Research
  • Special Services and Research Fund of the University of Alberta Hospitals, the Muttart Diabetes Research and Training Centre at the University of Alberta
  • Foundation and the Medical Research Council of Canada
  • Canadian Diabetes Association and the Alberta Children's Hospital Foundation
  • National Institutes of Health
  • Juvenile Diabetes Foundation

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Keywords:

  • Mennonite;
  • Chortitza (Old Colony);
  • insulin-dependent diabetes mellitus;
  • public health units;
  • gestational diabetes mellitus;
  • non-insulin-dependent diabetes mellitus;
  • autoimmune diseases;
  • malformations;
  • inborn errors of metabolism;
  • cleft lip± cleft palate;
  • congenital heart defects;
  • 21 and 17 OH deficiency;
  • nonspecific mental retardation;
  • Tourette syndrome;
  • genetic epidemiology

Abstract

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.

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