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Renal Tubular Function, Bone Health and Body Composition in Wilson’s Disease: A Cross-Sectional Study from India

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Abstract

There is limited literature from India with regard to the prevalence and magnitude of renal tubular and bone manifestations in Wilson’s disease (WD). Thus, we studied the prevalence of renal tubular acidosis among Indian patients with WD and also evaluated bone health and body composition in them. It was a cross-sectional study conducted at a south Indian tertiary care center. Twenty-five consecutive patients with WD aged more than 12 years attending the hepatology and neurology departments and 50 age, sex and BMI-matched controls were recruited. After clinical assessment, they underwent biochemical testing to assess renal tubular dysfunction. Bone mineral density (BMD) and body composition were assessed using a dual energy X-ray absorptiometry (DXA) scanner. Fifty-six percent (14/25) of patients with WD had renal tubular acidosis (RTA). Of them, 24% were diagnosed to have distal RTA. RTA was more common in hepatic WD patients who had prolonged duration of illness. Patients with WD had significantly lower BMD as compared to control subjects (p < 0.05). Low BMI, low IGF-1 and a shorter duration of therapy were key determinants of low bone mass in them (p < 0.05). Patients with WD had significantly more body fat (p = 0.01) and lower lean muscle mass (p = 0.03) when compared to age, sex and BMI-matched controls. In conclusion, renal tubular acidosis was common in patients with Wilson’s disease. These patients had a lower bone mineral density, higher body fat percentage and lower lean muscle mass as compared to controls.

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Acknowledgements

We would like to thank the study participants for their participation in the study. This study was funded by the fluid research grant, Christian Medical College, Vellore. The funding body had no role in design, collection and analysis, interpretation of data, writing of the manuscript or decision to submit for publication.

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Authors and Affiliations

Authors

Contributions

NK, KGS, MT, CEE and TVP coordinated and conducted the study. NK, KEC and TVP wrote the first draft. CEE and NT revised the draft. All authors reviewed and approved the final version of the draft.

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Correspondence to Thomas V. Paul.

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Conflict of interest

Nitin Kapoor, Kripa Elizabeth Cherian, Kattiparambil G. Sajith, Maya Thomas, Chundamannil E. Eapen, Nihal Thomas, Thomas V. Paul confirm that they have no conflict of interest related to this study.

Human and Animal Rights and Informed Consent

All tests on participants were performed in accordance with ethical standards stipulated by the CMC Research ethics committee. This study protocol was approved by the institutional review board prior to initiation of this study vide IRB Min No. 7917, dated 04.07.2016. No amimals were used in this study. All subjects were recruited following a detailed written informed consent, and in those below 18 years of age, an assent form was signed by their parents.

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Kapoor, N., Cherian, K.E., Sajith, K.G. et al. Renal Tubular Function, Bone Health and Body Composition in Wilson’s Disease: A Cross-Sectional Study from India. Calcif Tissue Int 105, 459–465 (2019). https://doi.org/10.1007/s00223-019-00588-z

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  • DOI: https://doi.org/10.1007/s00223-019-00588-z

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