Sera from 31 of 146 patients with cystic fibrosis (CF) contained levels of serum alkaline phosphatase (AP) which were elevated by age-related criteria. Prominent liver isoenzyme bands were present after polyacrylamide gel electrophoresis of all 31 abnormal sera. Larger than normal amounts of bone and intestinal AP were not present in these sera. These findings indicate that liver abnormalities consistently are responsible for elevations of total AP in sera of patients with CF. Several causes of high liver AP levels in CF patients were identified: chronic hepatic congestion, drug hepatotoxicity, overt biliary cirrhosis, and most commonly subclinical focal biliary cirrhosis.
Kattwinkel, J., Taussig, L. M., Slatland, B. E. and Verter, J. I.: The effects of age on alkaline phosphatase and other serologic liver function tests in normal subjects and patients with cystic fibrosis. J. Ped.82: 234, 1973.
3.
Feigelson, J., Pecau, Y., and Sauvegrain, J.: Liver function studies and biliary tract investigations in mucoviscidosis. Acta. Paediatr. Scand.59: 539, 1970.
4.
di Sant'Agnese, P. A. and Blanc, W. A.: A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas. Pediatrics18: 387, 1956.
5.
Craig, J. M., Haddad, H. and Shwachman, H.: The pathological changes in the liver in cystic fibrosis of the pancreas. Am. J. Dis. Child.93: 357, 1957.
6.
Kopel, F. B.: Gastrointestinal manifestations of cystic fibrosis. Gastroenterology62: 483, 1972.
7.
Simopoulous, A. P., Taussig, L. M., Murad, F., Arnaud, C. D., di Sant'Agnese, P. A., Kaltwinkel, J. and Banter, F.: Parathyroid function in patients with cystic fibrosis. Pediatr. Res.6: 355, 1972.
8.
Winkelman, J., Nadler, S., Demetriou, J. and Pileggi, V. J.: The clinical usefulness of alkaline phosphatase isoenzyme determinations. Am. J. Clin. Pathol.57: 625, 1972.
9.
Bodansky, A. J.: Phosphatase studies. II. Determination of serum phosphatase. Factors influencing the accuracy of the determination. J. Biol. Chem.101: 93, 1933.
10.
Clark, L. C., Jr. and Beck, E.: Plasma “alkaline” phosphatase activity. I. Normative data for growing children. J. Pediatr.36: 335, 1950.
11.
Bessey, O. A., Lowry, O. H. and Brock, M. J.: A method for the rapid determination of alkaline phosphatase with five cubic millimeters of serum. J. Biol. Chem.164: 321. 1946.
12.
Kaplan, M. and Rogers, L.: Separation of human serum-alkaline-phosphatase isoenzymes by polyacrylamide gel electrophoresis. LancetII: 1029, 1969.
13.
Doershuk. C. F., Matthews, L. W., Tucker, A., Nudelman, Y., Eddy, G. Wise, M. and Spector, S.: A therapeutic regimen for patients with cystic fibrosis. J. Pediatr.65: 558, 1964.
14.
Richman, S. M., Delman, A. J. and Grob, D.: Alterations in indices of liver function in congestive heart failure with particular reference to serum enzymes. Am. J. Med.30: 211, 1961.
