Dental findings in Lowe syndrome

This paper presents the dental findings of a child with the oculocerebrorenal syndrome of Lowe. The genetic abnormality in this condition results in an inborn error of inositol phosphate metabolism. Renal tubular dysfunction leads to metabolic acidosis and phosphaturia. At 4 years, generalised mobility of all primary teeth was noted. It is postulated that a defective inositol phosphate metabolism was responsible for the periodontal pathology found in this case. This is in direct contrast with previous reports of prolonged retention of primary teeth in children with this condition. Histology of extracted primary incisors demonstrated enlarged pulp chambers and mildly dysplastic dentin formation. This is consistent with a chronic subrachitic state, a known feature of Lowe syndrome, but no prominent interglobular dentin was present.