This is a rare, recessive inherited
condition, that usually causes death before ten years of age
. Thought to be associated with the first chromosome
, this syndrome is named after the twentieth century physician
s A. Chediak and O. Higashi.
Symptoms include pseudoalbinism, enlargement of the liver and pancreas, as well as eye abnormalities. Death is usually caused by infection (often by the Epstein-Barr virus ), neurological degeneration or lymphomas. Those sufferers that survive to adulthood suffer from many neural problems, including difficulty in balancing.