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

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