Bone remodeling in children with hepatic forms of glycogen storage disease

E.A. Vasilieva¹, T.V. Strokova^1,2, A.G. Surkov¹, M.E. Bagaeva^1,2, E.V. Pavlovskaya¹, A.I. Zubovich¹, I.V. Prokhorova¹

¹Federal Research Center for Nutrition, Biotechnology and Food Safety, Moscow

²Pirogov Russian National Research Medical University, Moscow

Aim: to evaluate bone remodeling biomarkers in children with hepatic forms of glycogen storage disease (GSD).

Patients and Methods: the study included 72 children (aged 7 months to 16 years). Group 1 included 16 children with type I GSD, group 2 — 14 children with type III GSD, group 3 — 42 children with VI and IX types. Bone mineral density (BMD) of the lumbar spine was assessed in all children with a height of not less than 100 cm. Markers of bone tissue metabolism and osteoporosis and insulin-like growth factor-1 (IGF-1) level in the blood were determined in all patients.

Results: in 23 (51%) children aged 3 to 16 years, there was a decrease in BMD of varying severity: osteopenia was detected in 38% of patients and osteoporosis — in 6 (12%) children. The median BMD values, depending on the GSD type, did not significantly differ (p = 0.51). Osteopenia was registered in all GSD types, e xcept type Ia. The osteoporosis highest incidence was found in patients with type Ib GSD (42%). Children with reduced IGF-1 were more likely to have decreased BMD (p<0.05). The authors found IGF-1 correlation in the blood with osteocalcin and collagen C-terminal telopeptide, which confirmed the IGF-1 effect on the processes intensification of bone metabolism. This indicator can be used as an early marker of osteopenia and osteoporosis. In children with type Ib and type III GSD, a decrease in serum markers of bone tissue formation and resorption was registered. It was evidenced by a decrease in the level of osteocalcin and C-terminal telopeptide in the absence of significant differences in serum levels of type I procollagen N-terminal propeptide.

Conclusion: bone metabolism markers diagnosis in patients with GSD allows determining the process severity of bone resorption and the remodeling rate without instrumental examination.

Keywords: glycogen storage disease, bone remodeli ng, bone mineral density, BMD, osteoporosis, osteopenia, P1NP, osteocalcin, b-Crosslaps, IGF-1.

For citation: Vasilieva E.A., Strokova T.V., Surkov A.G. et al. Bone remodeling in children with hepatic forms of glycogen storage disease. RMJ. 2019;7:34–38.