-transparent.png){kind=logo}
The bile acid pattern in bile and serum from two infants with the cerebro-hepato-renal syndrome of Zellweger was severely disturbed. An increased concentration particularly of trihydroxycoprostanic acid and also of dihydroxycoprostanic acid could be demonstrated. A generalized mitochondrial defect could explain these increased concentrations. This hypothesis is supported by the abnormal structure of the mitochondria in the liver biopsy of one of our patients. It is possible that the abnormal bile acids contribute to the liver damage of infants with Zellweger syndrome.
Related Publications
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
July 1985,
Acta paediatrica Scandinavica,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
November 1974,
Orvosi hetilap,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
January 1975,
Clinical genetics,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
January 1987,
Journal belge de radiologie,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
March 1970,
American journal of diseases of children (1960),
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
February 1975,
Orvosi hetilap,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
January 1986,
Journal of pediatric gastroenterology and nutrition,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
January 2007,
Neurology India,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
August 1983,
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques,
L Monnens, and
J Bakkeren, and
G Parmentier, and
G Janssen, and
U van Haelst, and
F Trijbels, and
H Eyssen
October 1999,
Archives of pediatrics & adolescent medicine,
