Zellweger syndrome
Definition
Zellweger syndrome refers to an inherited condition that is present at birth and usually causes death during the first six to twelve months of age. This syndrome is caused by a lack or reduction of peroxisomes, which are specialized organelles that help the body get rid of toxic substances. Zellweger syndrome is a disorder of metabolism. It is one of a group of genetic disorders called the leukodystrophies, diseases that involve abnormal growth of the fatty covering of nerve fibers (myelin sheath).
Description
In 1964, reserchers described a similar pattern of multiple birth defects in two unrelated pairs of siblings in Iowa and Maryland. Hans Zellweger identified the cases in Iowa. Passarge and...
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