OBJECTIVES: To evaluate deoxypyridinoline as a resorption marker in phenylketonuria (PKU) and to search for a relationship between deoxypyridinoline, calcium/creatinine index (Ca/Cr I), osteocalcin and bone alkaline phosphatase (BAP). METHODS: This was a transversal analytical study of 46 PKU patients [17.5 (4-38) years]. Deoxypyridinoline and osteocalcin were measured with a chemiluminescent assay and BAP was measured with an immunoradiometric assay. RESULTS: Deoxypyridinoline was significantly increased in patients aged 7-14 and >18 years old, being associated with age (r=-0.724, P<0.001). Adult patients showed significantly higher Ca/Cr I, which correlates with Phe values for the year prior to the study (P=0.014). Serum BAP was significantly increased in pediatric patients (9-13 years), while it was decreased in adult patients (P=0.003). Decreased osteocalcin levels were found in patients>15 years (P=0.028). Altered deoxypyridinoline and BAP values were related (P=0.042). CONCLUSION: PKU patients excreted increased D-Pyr, suggesting high bone resorption. Bone formation seems active in childhood but deteriorates in adult PKU patients. Periodic measurement of D-Pyr and BAP may be useful in the prevention of osteopenia in PKU patients.
OBJECTIVES: To evaluate deoxypyridinoline as a resorption marker in phenylketonuria (PKU) and to search for a relationship between deoxypyridinoline, calcium/creatinine index (Ca/Cr I), osteocalcin and bone alkaline phosphatase (BAP). METHODS: This was a transversal analytical study of 46 PKUpatients [17.5 (4-38) years]. Deoxypyridinoline and osteocalcin were measured with a chemiluminescent assay and BAP was measured with an immunoradiometric assay. RESULTS:Deoxypyridinoline was significantly increased in patients aged 7-14 and >18 years old, being associated with age (r=-0.724, P<0.001). Adult patients showed significantly higher Ca/Cr I, which correlates with Phe values for the year prior to the study (P=0.014). Serum BAP was significantly increased in pediatric patients (9-13 years), while it was decreased in adult patients (P=0.003). Decreased osteocalcin levels were found in patients>15 years (P=0.028). Altered deoxypyridinoline and BAP values were related (P=0.042). CONCLUSION:PKUpatients excreted increased D-Pyr, suggesting high bone resorption. Bone formation seems active in childhood but deteriorates in adult PKUpatients. Periodic measurement of D-Pyr and BAP may be useful in the prevention of osteopenia in PKUpatients.
Authors: Rani H Singh; Fran Rohr; Dianne Frazier; Amy Cunningham; Shideh Mofidi; Beth Ogata; Patricia L Splett; Kathryn Moseley; Kathleen Huntington; Phyllis B Acosta; Jerry Vockley; Sandra C Van Calcar Journal: Genet Med Date: 2014-01-02 Impact factor: 8.822
Authors: Hala M Koura; Sherif M Zaki; Nagwa A Ismail; Emad E Salama; Dalia H El Lebedy; Laila K Effat Journal: Iran J Pediatr Date: 2013-12-31 Impact factor: 0.364
Authors: Anne Daly; Wolfgang Högler; Nicola Crabtree; Nick Shaw; Sharon Evans; Alex Pinto; Richard Jackson; Catherine Ashmore; Júlio C Rocha; Boyd J Strauss; Gisela Wilcox; William D Fraser; Jonathan C Y Tang; Anita MacDonald Journal: Nutrients Date: 2021-06-17 Impact factor: 5.717