MadSci Network: Medicine

Re: Progeria--is this disease found in only one race?

Date: Mon Mar 20 22:41:14 2000
Posted By: Will Rosner, Staff, Clinical Biochemistry / Biotechnology, INTELLIgene Expressions
Area of science: Medicine
ID: 952900635.Me

Progeria has been observed in several races.  Japanese, Egyptian, Libyan  
are just a few case histories described at the NIH Online Mendelian 
Inheritance in Man web page

When people have progeria the appearance of the skin is altered, and 
maturation of facial features is also distorted.  This may result in loss 
of visual cues which tell us how to interpret the race of someone we are 
observing.  The picture at this link shows a 
boy with Progeria in a swimming pool who is not white, but the 
discoloration and blotching of his skin makes it very difficult to 
identify race.  It may therefore be that you have previously seen only 
Caucasian patients in pictures because of 1) Random chance  2) Biases in 
the medical community which are more likely to give better care (and thus 
better records) to Caucasians  3)  You may mistakenly identified non-
Caucasians as Caucasians as result of the changes in skin color and 
skeletal structure.

This disease is exceptionally rare, since first described at the turn of 
the century there have been only 86 patients reported in the medical 
literature. The author of the Nelson Textbook of Pediatrics is aware of 16 
patients who have not been reported.  This leaves us with a grand total of 
102 patients in 100 years. 

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