Abstract
Background
The goal of this study was to determine bone mineralization in children with Wilson’s disease (WD).
Methods
Twenty-seven patients (16 males) and two age- and gender-matched healthy children for each patient were enrolled in the study. Bone mineral content (BMC, grams) and density (BMD, g/cm2) at lumbar 1–4 vertebrae were measured by dual-energy X-ray absorptiometry. Urinary calcium excretion was calculated in 19 patients. The effect of cirrhosis and hypercalciuria on BMC and BMD was also evaluated in WD patients.
Results
There was no statistically significant difference between patients and healthy controls regarding mean BMC (33.0 ± 13.9 vs. 35.8 ± 13.8 g) (p = 0.940) and mean BMD values (0.66 ± 0.16 vs. 0.71 ± 0.18 g/cm2) (p = 0.269), respectively. Nine (47.4 %) patients had hypercalciuria. Hypercalciuric patients had statistically significant lower BMC and BMD values than those without hypercalciuria. A significant difference continued to be present after age, weight, height, and pubertal stage adjustment was done, but disappeared after weight, height, follow up duration, and pubertal stage adjustment was done. The presence of cirrhosis did not affect BMC and BMD significantly in WD patients.
Conclusions
BMC and BMD in children with WD were normal. The presence of hypercalciuria but not cirrhosis may affect BMC and BMD negatively in the patients.
Similar content being viewed by others
References
Bull PC, Thomas GR, Rommens JM, et al. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat Genet. 1993;5:327–37.
Tanzi RE, Petrukhin K, Chernov I, et al. The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene. Nat Genet. 1993;5:344–50.
Petrukhin K, Fischer SG, Pirastu M, et al. Mapping, cloning and genetic characterization of the region containing the Wilson disease gene. Nat Genet. 1993;5:338–43.
Yüce A, Koçak N, Demir H, et al. Evaluation of diagnostic parameters of Wilson’s disease in childhood. Indian J Gastroenterol. 2003;22:4–6.
Yüce A, Koçak N, Demirtaş M, et al. DNA haplotype analysis for the diagnosis of Wilson disease in siblings. Acta Paediatr. 2000;89:1142–4.
Lopez-Larramona G, Lucendo A, Gonzalez-Castillo S, Tenias JM. Hepatic osteodystrophy: an important matter for consideration in chronic liver disease. World J Hepatol. 2011;3:300–7.
Yadav A, Carey EJ. Osteoporosis in chronic liver disease. Nutr Clin Pract. 2013;28:52–64.
Uslu N, Saltık-Temizel IN, Demir H, et al. Bone mineral density in children with cirrhosis. J Gastroenterol. 2006;41:873–7.
Rodriguez Nieva N, Febrer Rotger A, Melendez Plumed M, Vernet Bori A. Osteoarthropathy in three siblings with Wilson’s disease. An Pediatr (Barc). 2004;61:181–4.
Golding DN, Walshe JM. Proceedings: The musculoskeletal features of Wilson’s disease: a clinical, radiological, and serological survey. Ann Rheum Dis. 1975;34:201.
Hegedus D, Ferencz V, Lakatos PL, et al. Decreased bone density, elevated serum osteoprotegerin, and beta-cross-laps in Wilson disease. J Bone Miner Res. 2002;17:1961–7.
Xie YZ, Zhang XZ, Xu XH, et al. Radiologic study of 42 cases of Wilson disease. Skelet Radiol. 1985;13:114–9.
Selimoglu MA, Ertekin V, Doneray H, Yildirim M. Bone mineral density of children with Wilson disease: efficacy of penicllamine and zinc therapy. J Clin Gastroenterol. 2008;42:194–8.
Alon U, Hellerstein S. Assessment and interpretation of the tubular threshold for phosphate in infants and children. Pediatr Nephrol. 1994;8:250–1.
Hayran M, Özdemir O. Bilgisayar, İstatistik ve Tıp (Statistics and Medicine). Ankara-Turkey: Hekimler Yayın Birliği; 1995.
Rouillard S, Lane NE. Hepatic osteodystrophy. Hepatology. 2001;33:301–7.
Atkinson M, Nordin BE, Sherlock S. Malabsorption and bone disease in prolonged obstructive jaundice. Q J Med. 1956;25:299–312.
Argao EA, Specker BL, Heubi JE. Bone mineral content in infants and children with chronic cholestatic liver disease. Pediatrics. 1993;91:1151–4.
Kobayashi A, Kawai S, Utsunomiya T, et al. Bone disease in infants and children with hepatobiliary disease. Arch Dis Child. 1974;49:641–6.
Glasgow JF, Thomas PS. The osteodystrophy of prolonged obstructive liver disease in childhood. Acta Paediatr Scand. 1976;65:57–64.
Sokhi RP, Anantharaju A, Kondaveeti R, et al. Bone mineral density among cirrhotic patients awaiting liver transplantation. Liver Transpl. 2004;10:648–53.
Finby N, Bearn AG. Roentgenographic abnormalities of the skeletal system in Wilson’s disease (hepatolenticular degeneration). Am J Roentgenol Radium Ther Nucl Med. 1958;79:603–11.
Rosenoer VM, Michell RC. Skeletal changes in Wilson’s disease (hepatolenticular degeneration). Br J Radiol. 1959;32:805–9.
Mindelzun R, Elkin M, Scheinberg IH, Sternlieb I. Skeletal changes in Wilson’s disease: a radiological study. Radiology. 1970;94:127–32.
Aksoy M, Camli N, Dincol K, et al. Osseous changes in Wilson’s disease: a radiologic study of nine patients. Radiology. 1972;102:505–9.
Blake GM, Fogelman I. Technical principles of dual energy X-ray absorptiometry. Semin Nucl Med. 1997;27:210–28.
Voruganti VS, Klein GL, Lu HX, et al. Impaired zinc and copper status in children with burn injuries: need to reassess nutritional requirements. Burns. 2005;31:711–6.
Yamaguchi M. Role of nutritional zinc in the prevention of osteoporosis. Mol Cell Biochem. 2010;338:241–54.
Strause L, Saltman P, Smith KT, et al. Spinal bone loss in postmenopousal women supplemented with calcium and trace minerals. J Nutr. 1994;124:1060–4.
Flynn A. The role of dietary calcium in bone health. Proc Nutr Soc. 2003;62:851–8.
Smith BJ, King JB, Lucas EA, et al. Skeletal unloading and dietary copper depletion are detrimental to bone quality of mature rats. J Nutr. 2002;132:190–6.
Jonas J, Burns J, Abel EW, et al. Impaired mechanical strength of bone in experimental copper deficiency. Ann Nutr Metab. 1993;37:245–52.
Rucker RB, Riggins RS, Laughlin R, et al. Effects of nutritional copper deficiency on the biomechanical properties of bone and arterial elastin metabolism in the chick. J Nutr. 1975;105:1062–70.
Conflict of interest
AC, HÖ, AY, İNS-T, HD, and FG have no conflict of interest to report.
Source of support
None
Ethics statement
The study was performed in a manner that conforms with the Helsinki Declaration of 1975, as revised in 2000 and 2008 concerning Human and Animal Rights, and the authors followed the policy concerning Informed Consent as shown on Springer.com.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Çetinkaya, A., Özen, H., Yüce, A. et al. Bone mineralization in children with Wilson’s disease. Indian J Gastroenterol 33, 427–431 (2014). https://doi.org/10.1007/s12664-014-0468-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12664-014-0468-9