title: Serum concentrations of albumin, C-reactive protein, alpha 2-macroglobulin, prealbumin, fibronectin, fibrinogen, transferrin, and retinol binding protein in 55 patients with hepatic glycogen storage diseases.
abstract: Hepatic glycogen storage diseases are hereditary metabolic disorders involving the metabolism of glycogen. This study was designed to investigate the serum protein status in such diseases. Fifty-five patients with glycogen storage disease types I, III, VI, and IX, whose ages ranged from 1 month to 27 years, were included in this work. C-reactive protein, fibrinogen, alpha 2-macroglobulin, albumin, transferrin, fibronectin, retinol binding protein, and prealbumin serum concentrations were measured in each patient. In patients affected with type I glycogen storage disease, serum concentrations of alpha 2-macroglobulin, fibrinogen, C-reactive protein, and transferrin were significantly increased. In patients with types III, VI, and IX glycogen storage diseases, the concentration of alpha 2-macroglobulin was the only one that was significantly increased. Thus, even though this study raises more questions than it answers, it seems likely that the hepatic synthesis of some proteins may be increased in patients affected by hepatic glycogen storage diseases. This may indicate some degree of mild hepatic dysfunction in such metabolic disorders. However, further investigations are required to elucidate the discrepancies observed among the different types of diseases.
authors.affiliation: Service de Pédiatrie, Hôpital Antoine Béclère, Clamart, France.
authors.forename: P
authors.initials: P
authors.lastname: Labrune
authors.forename: C
authors.initials: C
authors.lastname: Benattar
authors.forename: N
authors.initials: N
authors.lastname: Ammoury
authors.forename: J
authors.initials: J
authors.lastname: Chalas
authors.forename: A
authors.initials: A
authors.lastname: Lindenbaum
authors.forename: M
authors.initials: M
authors.lastname: Odièvre
mesh_terms: D000293:Adolescent; D000328:Adult; D001798:Blood Proteins / Q000378:metabolism*; D002097:C-Reactive Protein / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D005353:Fibronectins / Q000378:metabolism; D006008:Glycogen Storage Disease / Q000097:blood*; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000097:blood*; D008297:Male; D011228:Prealbumin / Q000378:metabolism; D012709:Serum Albumin / Q000378:metabolism; D000511:alpha-Macroglobulins / Q000378:metabolism
chemical_list: D001798:Blood Proteins; D005353:Fibronectins; D011228:Prealbumin; D012709:Serum Albumin; D000511:alpha-Macroglobulins; D002097:C-Reactive Protein