Data from the Bloom’s Syndrome Registry, 2009
The Registered Population | ||
---|---|---|
Vital Statistics |
||
Number* | Mean (range) | |
Total | 265 | |
Male | 140 | |
Female | 125 | |
Ages of those living† | 150 | 30 (<1-53) |
Male | 71 | 25 (3-50) |
Female | 78 | 23 (4-53) |
Ages at death† | 103 | 26 (<1-49) |
Male | 62 | 27 (1-49) |
Female | 41 | 25 (<1-49) |
Sizes |
||
Birth weights (kg.)† | 204 | 1.8 (0.7-3.2) |
Male | 108 | 1.8 (0.9-3.2) |
Female | 96 | 1.8 (0.7-2.9) |
Adult heights (cm.)† | 95 | 144 (126-164) |
Men | 53 | 149 (128-164) |
Women | 42 | 138 (115-160) |
Major Medical Complications |
||
Chronic obstructive lung disease, fatal | 5 | 23‡ (18-38) |
Male | 1 | 24‡ |
Female | 4 | 25‡ (18-38) |
Diabetes mellitus | 42 | 26§ (4-45) |
Male | 21 | 27§ (14-40) |
Female | 21 | 25§ (4-45) |
Cancer | 122 | 26# (<1-49) |
Male | 69 | 27# (1-48) |
Female | 53 | 26# (<1-49) |
* Of registered persons
† For whom data are available
‡ Age at death
§ Age at diagnosis
# For all cancers
Nationalities and Ethnic Groups in the Registry | |||
---|---|---|---|
Jewish | Non-Jewish | Total | |
Registered Persons* | 69 (26.0%) | 196 (76.9%) | 265 |
National and Ethnic Groups | |||
United Statesan | 43 | 56 | 99 |
Israeli | 16 | 0 | 16 |
Brazilian | 1 | 15 | 16 |
German | 0 | 14 | 14 |
Italian | 0 | 13 | 13 |
Japanese | 0 | 12 | 12 |
Turkish | 0 | 12 | 12 |
Canadian | 2 | 10 | 12 |
African, Sub-Saharan | 0 | 9 | 9 |
Dutch | 0 | 7 | 7 |
Hispanic, North American | 0 | 6 | 6 |
English and Welsh | 3 | 6 | 9 |
Belgian | 1 | 5 | 6 |
Indian, Asian | 0 | 5 | 5 |
Australian | 1 | 3 | 4 |
Gypsy | 0 | 4 | 4 |
Argentinian | 1 | 3 | 4 |
Mexican | 1 | 3 | 4 |
Iraqi | 0 | 3 | 3 |
Portuguese | 0 | 2 | 2 |
Spanish | 0 | 2 | 2 |
African, North | 0 | 2 | 2 |
Afghan | 0 | 1 | 1 |
French | 0 | 1 | 1 |
Greek | 0 | 1 | 1 |
Lebanese | 0 | 1 | 1 |
* The 265 persons with BSyn are from 222 families (sibships). 167 (75.2%) of the affected families are/were non-Jewish. That only the remaining 55 (24.8%) are Jewish argues against the use any longer of the term Jewish genetic disease to describe BSyn.
Major Medical Complications in Registered Persons | ||
---|---|---|
Complications | Number of Persons Affected | Percentage of Registered Persons |
Chronic lung disease | 7 | 2.6 |
Diabetes | 42 | 15.8 |
Cancer | 122 | 46.0 |
Cellular Types of Cancers in Registered Persons | ||
Cellular Types of Cancer | Number, All Sites | Percentage of the Cancers Diagnosed |
Hematopoietic | 20 | 11.3 |
Lymphoid | 44 | 24.9 |
Epithelial | 93 | 52.5 |
Other | 20 | 11.3 |
Top of page