What is the cause of benign transient hyperphosphatasemia? A study of 35 cases.
In a study of 35 children with benign transient hyperphosphatasemia, I found a marked seasonal clustering of cases after the summer months. Furthermore, plasma 25-hydroxyvitamin D concentrations were almost twice those of controls matched for age and time of year. Many children had evidence of weight loss and one had idiopathic hypercalcemia of infancy. Activities both of liver and bone isoenzymes of alkaline phosphatase (EC 3.1.3.1) in plasma were increased. The liver and (to a lesser extent) bone isoenzymes had enhanced electrophoretic mobility, and both showed increased binding to wheat-germ lectin by affinity electrophoresis. For the liver (and probably also the bone) isoenzyme, these changes were due to an increased content of sialic acid. A possible etiology for the condition is proposed involving (a) increased synthesis of alkaline phosphatase, mediated by vitamin D metabolites, and (b) decreased hepatic clearance caused by the high sialic acid content and exacerbated in some cases by the effects of some drugs on the liver.
Crofton
P M
PM
Department of Paediatric Biochemistry, Royal Hospital for Sick Children, Edinburgh, U.K.
D000118:Acetylglucosaminidase / Q000494:pharmacology; D000469:Alkaline Phosphatase / Q000097:blood*; D001835:Body Weight; D001842:Bone and Bones / Q000201:enzymology; D002112:Calcifediol / Q000097:blood; D002675:Child, Preschool; D004587:Electrophoresis, Agar Gel; D005260:Female; D006207:Half-Life; D006801:Humans; D007223:Infant; D007527:Isoenzymes / Q000097:blood*; D008099:Liver / Q000201:enzymology; D008297:Male; D012621:Seasons; D014909:Wheat Germ Agglutinins / Q000378:metabolism
D007527:Isoenzymes; D014909:Wheat Germ Agglutinins; D000469:Alkaline Phosphatase; D000118:Acetylglucosaminidase; D002112:Calcifediol