Lindex #0783

Brinn LB

Jews, Genetics and Disease

Harofe Haivri

1962; 2:261-275

The author proposes to study whether Jews are related by religion only or whether they constitute a true ethnic group by studying Jews of Eastern European origin. Whereas, there are diseases whose sufferers are disproportionately Jewish this may be due to numerous factors such as Jews' tendency to live in urban areas and avail themselves of major medical centers where obscure diseases are more likely to be diagnosed. There may be any number of external, environmental factors to which Jews are exposed predisposing them to certain diseases. Numerous diseases are discussed including:

1. Tay-Sachs Disease - a condition characterized by mental retardation, blindness, flaccid paralysis and eventually death. This is particularly common in Eastern European Jews. Of 58 cases of Tay-Sachs stated by death certificates 53 were Jews, and one case per 8,300 Jewish births as opposed to 1/500,000 for non- Jews. The disease is 10 times more common in Ashkenazi Jews. Of 12 cases seen in Chicago over 17 years, 11 were Jewish.

2. Niemann-Picks Disease - a condition in which sphingomyelin accumulates in reticulo-endothelial cells. There is a 3 to 1 ratio of Jewish to non-Jewish cases. However in one review of 73 cases, 29 were Jewish, 26 were non-Jewish with 18 unknown. In a review of cases in Boston, 5 were Jewish.

3. Gaucher's' Disease - a rare metabolic defect in which kerasin accumulates in the reticuloendothelial - endothelial cells. In this review of 355 cases a disproportionate number were believed to be Jewish, although no statistics were calculated.

4. Dystonia Musculorum Deformans - a disease of the central nervous system in which twisting movements of the trunk and extremities occur. The disease was once thought to occur exclusively in Jews but is now believed to occur in non-Jews as well. Of 30 cases in New York, 24 were Jewish.

5. Riley-Day Syndrome or familial dysautonomia affects almost exclusively Jewish children. Of the 5 original descriptions of the disease, all were Jewish. In another series of 33 cases, all had at least one Jewish parent. Of the 80 cases reported to date, only 5 have been non-Jewish.

6. Pemphigus-Vulgaris is a disease in which the skin is covered with numerous blebs. Of 77 cases in New York, 56 were Jews who were largely from Eastern Europe. Of 170 patients at Bellevue Hospital, 84 were Jews, many of Russian origin.

7. Idiopathic Hypercholesterolemia - a condition of abnormally high blood cholesterol levels. In a study comparing the prevalence of coronary artery disease, a manifestation of I.H. among Italian and Jewish men found 7.9 percent of 232 Italians had CAD whereas 15.8 percenr of 232 Jewish men had it. In another study 21 percent of 266 Jewish patients at Mt. Sinai Hospital in New York City had hypercholesterolemia, while only 9 percent of 212 non-Jewish patients did.

8. Diabetes Mellitis - Many studies show evidence of increased prevalence of Diabetes Mellitus among Jews, but this is contradicted by others. A study in Arizona showed that Jews constituted 0.5 percent of the state's population, but 2 percent of the state's diabetics. Another study found that 6.3 percent of 1,000 non-diabetic Jews had a family history of Diabetes as opposed to 1.23 percent of non- Jews in another study.

9. Polycythemia Vera - a disease which causes chronic elevation of hemoglobin and red blood count. Of 20 cases in one series, 10 were Jewish, and in another series of 134 cases 48 percent were Jewish.

10. Regional Enteritis - a chronic inflammatory disease of the small bowel, which occurs in disproportionately high numbers of Jewish people. Of 600 cases at the Mayo Clinic 153 were Jewish, and in another series 43 of 100 cases were Jewish.

11. Thromboangiitis Obliterans - Buerger's Disease, an inflammation of blood vessels. The original descriptions of this disease in a series of 30 cases showed that a preponderance of sufferers were Polish and Russian Jews. However, numerous other studies have shown that the disease probably occurs among a variety of populations.

12. Idiopathic Pentosuria - is not so much a disease as a harmless metabolic abnormality that causes no harm to patients. About 200 cases have been documented, all but 2 being Jews. It is estimated that 1/50 American Jews carries the gene for this defect.