Serum creatine kinase isoenzymes in children with osteogenesis imperfecta

P D'Eufemia1, R Finocchiaro2, A Zambrano2

  • 1Department of Pediatrics, "Sapienza", University of Rome, Rome, Italy. patdeufemia@inwind.it.

Insights

Serum creatine kinase isoenzyme (CKbb) levels increase with prolonged bisphosphonate treatment in children with osteogenesis imperfecta and decrease upon discontinuation, aiding in monitoring treatment.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Orthopedics

Background:

  • Creatine kinase isoenzyme BB (CKbb) is crucial for osteoclast function and bone resorption.
  • Previous studies indicated elevated CKbb in children with osteogenesis imperfecta (OI) type 1 on bisphosphonate therapy.
  • This study investigates CK isoenzyme changes during and after neridronate treatment in OI patients.

Purpose of the Study:

  • To evaluate serum creatine kinase isoenzyme activity as a biochemical marker in children with osteogenesis imperfecta undergoing bisphosphonate treatment.
  • To assess the impact of continuous versus discontinued neridronate treatment on CK isoenzyme levels.
  • To explore the potential for CKbb monitoring to prevent over-treatment risks.

Main Methods:

  • Included 18 children with OI type 1, divided into continuous (n=12) and discontinued (n=6) neridronate groups.
  • Measured auxological data, bone metabolism markers, bone mineral density, and serum total CK and isoenzyme activities over 2 years.
  • Analyzed changes in CKbb, CKmb, and C-terminal telopeptide (CTx) levels.

Main Results:

  • Serum CKbb significantly increased with continuous neridronate treatment (p < 0.004) and decreased to undetectable levels after discontinuation.
  • Cardiac muscle creatine kinase isoenzyme (CKmb) levels markedly decreased in both groups.
  • Serum CTx levels remained largely unchanged throughout the study.

Conclusions:

  • Neridronate administration has a cumulative effect on increasing serum CKbb levels, which is reversible upon treatment cessation.
  • Serum CKbb can serve as a valuable biochemical marker for monitoring prolonged bisphosphonate therapy in OI patients.
  • Decreased serum CKmb suggests potential systemic effects of bisphosphonate drugs.

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