Why is it called Zellweger syndrome?

It is one of a family of disorders called Zellweger spectrum disorders which are leukodystrophies. Zellweger syndrome is named after Hans Zellweger (1909–1990), a Swiss-American pediatrician, a professor of pediatrics and genetics at the University of Iowa who researched this disorder.

What is the Zellweger syndrome?

What is Zellweger syndrome? Zellweger syndrome (ZS) is a genetic disorder found in newborn babies. ZS is the most severe of the four disorders in the Zellweger spectrum. It causes serious problems with nerves and metabolism (changing food into energy) soon after birth. ZS affects the brain, liver and kidneys.

How long can you live with Zellweger syndrome?

Zellweger syndrome life expectancy Children with Zellweger syndrome usually do not survive beyond the first year of life 12). Most infants do not survive past the first 6 months, and usually succumb to respiratory distress, gastrointestinal bleeding, or liver failure.

Can you live with Zellweger syndrome?

Children with severe Zellweger spectrum disorder typically do not survive beyond the first year of life. People with intermediate or mild Zellweger spectrum disorder have more variable features that progress more slowly than those with the severe form.

Is there a cure for Zellweger syndrome?

There is no cure for Zellweger syndrome, nor is there a standard course of treatment. Since the metabolic and neurological abnormalities that cause the symptoms of Zellweger syndrome are caused during fetal development, treatments to correct these abnormalities after birth are limited.

What are the signs symptoms of Zellweger syndrome?

The signs and symptoms of Zellweger syndrome typically appear during the newborn period and may include poor muscle tone (hypotonia), poor feeding, seizures, hearing loss, vision loss, distinctive facial features, and skeletal abnormalities.

What organelle is affected by Zellweger syndrome?

Zellweger syndrome is a rare, inherited metabolic disorder that affects peroxisomes, organelles found in almost all body cells. Peroxisomes are responsible for many important cell processes, including energy metabolism, which means that Zellweger syndrome can severely impact the body.