acid-phosphatase and Liver-Cirrhosis--Biliary

The influence of a nonskeletal disease with increased connective tissue synthesis or degradation in the collagen-related markers of bone turnover has been evaluated in 34 women with primary biliary cirrhosis (PBC; age range 41-81 years), a disease with increased hepatic fibrosis, often associated with osteoporosis. Serum osteocalcin (BGP), and carboxy-terminal (PICP) and amino-terminal (PINP) propeptides of type I collagen were assessed as indexes of bone formation, whereas serum tartrate-resistant acid phosphatase (TRAP), and cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), and urinary hydroxyproline (HYP), pyridinoline (PYR), deoxypyridinoline (DPYR), and type I collagen cross-linked N- (NTX) and C-telopeptide (CTX) were measured as markers of bone resorption. The histologic stage of the disease and serum amino-terminal propeptide of type III collagen (PIIINP) as an index of liver fibrogenesis were also evaluated. BGP levels were significantly lower, whereas PICP and PINP levels were higher in patients than in controls. Among the bone resorption markers, serum ICTP and urinary PYR, DPYR, HYP, NTX, and CTX levels were significantly higher in patients than in controls. Serum PIIINP levels were also increased in PBC patients. BGP did not correlate with PICP and PINP, but these markers of bone formation as well as ICTP, PYR, DPYR, and NTX correlated with serum PIIINP levels. Serum TRAP did not correlate with collagen-related markers of bone resorption. Moreover, patients with PIIINP and bilirubin above normal levels had higher PICP, PINP, ICTP PYR, DPYR, CTX, and NTX. These markers correlated with the histologic stage of the disease, but not with osteopenia measured by densitometric procedures in 22 patients. In conclusion, collagen-related markers of bone turnover do not reflect bone remodeling in PBC. The close association of these markers with PIIINP and the clinical and histologic stage of the liver disease suggests that they are influenced by liver collagen metabolism.

Topics: Acid Phosphatase; Adult; Aged; Aged, 80 and over; Amino Acids; Biomarkers; Bone and Bones; Bone Density; Bone Development; Bone Resorption; Female; Humans; Hydroxyproline; Isoenzymes; Liver; Liver Cirrhosis, Biliary; Liver Function Tests; Middle Aged; Osteocalcin; Peptide Fragments; Procollagen; Tartrate-Resistant Acid Phosphatase

Topics: Acid Phosphatase; Adenosine Triphosphatases; Alkaline Phosphatase; Animals; Biopsy; Cholestasis; Dogs; Esterases; gamma-Glutamyltransferase; Glucose-6-Phosphatase; Glucuronidase; Histocytochemistry; Humans; Kupffer Cells; L-Lactate Dehydrogenase; Leucyl Aminopeptidase; Liver; Liver Cirrhosis, Biliary; Microscopy, Electron; Monoamine Oxidase; Oxidoreductases; Phosphoric Monoester Hydrolases; Rats; Succinate Dehydrogenase

Topics: Acid Phosphatase; Adenosine Triphosphatases; Alanine Transaminase; Alkaline Phosphatase; Animals; Aspartate Aminotransferases; Bile Ducts; Bilirubin; Cholestasis; Cholesterol; Dogs; Hepatic Veins; Liver; Liver Cirrhosis, Biliary; Lymphatic Diseases; Lymphatic System; Nucleotidases; Vena Cava, Superior

Ceramidase activity could not be demonstrated in the kidney and cerebellum from a deceased patient with Farber's disease, whereas the activities of six control acid hydrolase enzymes appeared normal. This enzyme defect presumably accounts for the accumulation that has been described in two patients and may represent the biochemical basis of this disorder.

Topics: Acid Phosphatase; Adult; Carbon Isotopes; Ceramides; Cerebellum; Cerebrosides; Child; Child, Preschool; Congenital Abnormalities; Female; Galactose; Galactosidases; Glucose; Glycoside Hydrolases; Heart Defects, Congenital; Hexosaminidases; Humans; Hydrolases; Infant; Infant, Newborn; Intellectual Disability; Kidney; Lipidoses; Liver Cirrhosis, Biliary; Male; Metabolism, Inborn Errors; Neuraminidase; Pigmentation Disorders; Respiratory Distress Syndrome, Newborn

Topics: Acid Phosphatase; Alanine Transaminase; Alkaline Phosphatase; Animals; Bile Pigments; Bilirubin; Blood Proteins; Cholecystectomy; Common Bile Duct; Drainage; Haplorhini; Jejunum; Ligation; Liver; Liver Cirrhosis, Biliary; Liver Function Tests; Sulfobromophthalein; Urobilinogen