Dental findings in Brazilian patients with Fanconi syndrome

Fanconi syndrome (FS) is a generalized dysfunction of renal proximal tubules resulting in impaired reabsorption of amino acids, phosphate, urate, bicarbonate, glucose, and electrolytes. FS can be a primary renal impairment due to genetic origin or secondary to inborn errors of metabolism or systemic diseases. Main clinical findings are polyuria, failure to thrive, rickets/osteomalacia, metabolic acidosis, bone deformities, and, occasionally, nephrocalcinosis. Although dental abnormalities have been reported in patients with tubulopathies, oral manifestations in FS were very rarely described.

Two patients with FS exhibiting important dental alterations were reported. Oral examination of the first patient revealed teeth with rough surfaces and white opaque areas. Early loss of primary teeth and delayed eruption of permanent teeth were also observed. Oral examination of the second patient detected delayed eruption of permanent teeth and X-ray images revealed loss of contrast between enamel and dentin. In both patients with FS, loss of bone density in maxilla and mandible was observed in X-ray images.

This study is the first report of dental abnormalities in Brazilian patients with FS. Furthermore, our findings support the general idea that renal tubular disorders might impact on mineralized tissues of the oral cavity.