Bone mineral densitometry is recommended in pre-liver transplant evaluation in children suffering from Wilson disease

Abstract

Background: Wilson disease (WD) is an autosomal recessive disorder of copper metabolism with an estimated prevalence of 1 in 30,000. Osteoarticular manifestations are a common feature of WD and mainly involve osteopenia, osteoporosis, and arthropathy.

Objectives: This study aimed to investigate the prevalence of abnormal mineral density in a group of children with WD and evaluate if it is rational to recommend screening in pre-transplantation workups.

Methods: This study included all the children with a confirmed diagnosis of WD, followed at Nemazee Hospital affiliated with Shiraz University of Medical Sciences between 2016 and 2018. The researchers also excluded patients with other underlying diseases, abnormalities of calcium, phosphorus, or vitamin D, or those who used other medications leading to osteoporosis. Bone mineral content (BMC)/Bone mineral density (BMD) of the lumbar spine (LS-BMD) was performed for all included patients with DXA scans.

Results: Evaluation of z-scores showed osteopenia in 40% and osteoporosis in 53.33% of the patients. There was no significant association between the z-score values and cirrhosis in WD patients (P=0.559). There was a significant correlation between the value of z-scores with weight (P=0.007) and BMI (P=0.001) in patients with WD.

Conclusion: The results suggest that WD is intrinsically associated with osteoporosis. Also, patients with WD are at risk of osteopenia and osteoporosis, and screening for evaluation of bone mineral density and prophylactic supplementation may be logical, especially for those who are candidates for liver transplant due to the probability of deterioration of osteopathy in the first few months after liver transplantation