Bone metabolism in cholestatic children before and after living-related liver transplantation--a long-term

Edyta Kryskiewicz1, Joanna Pawlowska, Pawel Pludowski

  • 1Department of Biochemistry and Experimental Medicine, The Children's Memorial Health Institute, Warsaw, Poland.

Insights

Successful living-related liver transplantation (LRLTx) in cholestatic children improves bone metabolism and bone mineral density in the short term. However, long-term follow-up shows moderately impaired bone status and metabolism, with bone markers predicting these changes.

Area of Science:

  • Pediatric Hepatology
  • Pediatric Endocrinology
  • Orthopedics

Background:

  • Children with end-stage liver disease, particularly cholestasis, frequently develop bone disorders.
  • Risk factors include impaired liver function, altered vitamin D and calcium-phosphorus metabolism, malnutrition, and immunosuppressive therapy.
  • Liver transplantation is a potential treatment, but its long-term effects on bone health require investigation.

Purpose of the Study:

  • To evaluate the long-term impact of successful living-related liver transplantation (LRLTx) on skeletal status and bone metabolism in children with cholestatic liver disease.

Main Methods:

  • A cohort of 18 cholestatic children undergoing LRLTx was studied.
  • Bone metabolism markers (osteocalcin, P1NP, CTx, IGF-I, IGFBP-3, PTH, vitamin D metabolites) and bone mineral status (TBBMC, TBBMD via DXA) were measured before and up to 4.4 years post-transplantation.
  • Data were compared to age-matched controls and analyzed for correlations.

Main Results:

  • Pre-LRLTx, children exhibited decreased bone markers and bone mineral density compared to controls.
  • Post-LRLTx, bone markers and bone mineral density normalized in the short term, correlating with IGF-I and PTH.
  • Long-term (V4), P1NP and PTH levels deviated from reference ranges, coinciding with reduced bone mineral density Z-scores; P1NP and CTx showed predictive value for bone status changes.

Conclusions:

  • LRLTx initially restores bone metabolism and improves bone mineral content and density in cholestatic children.
  • Long-term monitoring reveals persistent, moderately impaired bone status and metabolic disturbances.
  • Bone turnover markers like P1NP and CTx are valuable predictors of bone status evolution after LRLTx.

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