Disease Information for Cerebrohepatorenal syndrome/Zellweger

Synonyms:

Cerebro Hepato Renal Syndrome, Cerebro Hepato Renal Syndromes, cerebrohepatorenal syndrome, Cerebrohepatorenal Syndromes, CHR SYNDROME, CHRS, congenital iron overload, Zellweger, ZELLWEGER DIS, Zellweger Disease, Zellweger Syndrome, Zellweger syndrome (disorder), Zellwegers Syndrome, Zellweger's Syndrome, ZS, ZWS

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Cerebro Hepato Renal Syndrome, Cerebro Hepato Renal Syndromes, cerebrohepat... (more)

Definition:

Zellweger Syndrome; Bowen Syndrome; Cerebrohepatorenal Syndrome; this is a rare, hereditary disorder characterized by a deficiency or absence of peroxisomes in the cells of the liver, kidneys, and brain; Peroxisomes are very small, membrane-bound structures within the cytoplasm of cells that function as part of the body’s waste disposal system; In the absence of the enzymes normally found in peroxisomes, waste products, especially very long chain fatty acids (VLCFA), accumulate in the cells of the affected organ; The accumulation of these waste products has profound affects on the development of the fetus;

Zellweger syndrome is one of a group of genetic disorders known as leukodystrophies that affect growth of the myelin sheath, which lines nerve fibers in the brain and speeds the conduction of nerve impulses;

-----------------------[nord website 2006]-------------The most common features of Zellweger syndrome include an enlarged liver, high levels of iron and copper in the blood, and vision disturbances; Some affected infants may show prenatal growth failure; Symptoms at birth may include lack of muscle tone and an inability to move; Other symptoms may include unusual facial characteristics, mental retardation, seizures, and an inability to suck and/or swallow; Jaundice and gastrointestinal bleeding may also occur; There is no cure for Zellweger syndrome, nor is there a standard course of treatment; Infections should be guarded against to prevent such complications as pneumonia and respiratory distress; Other treatment is symptomatic and supportive; prognosis:The prognosis for individuals with Zellweger syndrome is poor; Death usually occurs within 6 months after onset, and may be caused by respiratory distress, gastrointestinal bleeding, or liver failure----------------------[NINDS Website 2006

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Zellweger Syndrome; Bowen Syndrome; Cerebrohepatorenal Syndrome; this is a ... (more) (edit)

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