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K Hennig

Publications and source records attributed to K Hennig.

At least 19 recordsLinked to original sources

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

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

[Experimental studies of the distribution of intravenously injected iodine-labeled cytonal (diethylstilbestrol diphosphate)].

After intravenous injection the distribution of iodine-labelled diethylstilbestral-diphosphate (cytonal) was investigated in various organs of the rat. For this purpose the concentration of activity in the organs of the animals were established by measurements of specimens in the bore-hole counter and scintigrams were made by means of a small animal scanner. The result was an unequivocal temporary dependence of the enrichment of the substance in the organs. The greatest enrichment was found 10 minutes after injection in the liver, then followed blood, kidneys, adrenal glands, seminal vesicles, prostate, abdominal wall and the fatty tissue surrounding the prostate. The blood activity relatively decreased. 7 days after injection an unambiguously higher activity was to be proved in the liver, kidney, adrenal gland than in the blood, whereas the enrichment of iodine-labelled diethylstilbestraldiphosphate in prostate, seminal vesicle, fatty and muscular tissue was always below the enrichment of the blood. But in every case the relation of organ activity to blood activity changed in favour of the organ activity, also with regard to the prostate. 10 minutes after injection the concentration of the activity of the seminal vesicle was somewhat higher than that one of the prostate, but in the further course it was below the concentration of the activity of the prostate. During the first two hours the fatty tissue surrounding the prostate showed a clearly and after the third day a slightly lower activity concentration than the prostate. On the basis of this distribution of the activity for the scintigraphy of the prostate above all a temporary interval of about 10 minutes to 3 hours after the injection of labelled cytonal is involved. The results cause us to perform investigations in men concerning the pharmacokinetics and the possibility of using radioactively labelled cytonal.

Abdominal Muscles