Dental Complication Burden in Patients with Phosphorus Metabolism and Phosphatase Disorders: Insights from a 14-Year Nationwide Longitudinal Retrospective Study in South Korea

OBJECTIVES: This study evaluated the burden of dental complications in patients with phosphorus metabolism and phosphatase disorders, a rare disease characterized by significant dental manifestations in Korea.

METHODS: This longitudinal retrospective study was conducted using the Health Insurance Review and Assessment Service databases from January 2007 to August 2022, covering entire population in Korea. The diagnosis of phosphorus metabolism and phosphatase disorders was defined using ICD-10 code (E83.3) and registration code (V189) for the patients eligible for co-payment assistance for rare intractable diseases between January 2008 and August 2020. The occurrence of dental complications was defined with ICD-10 codes (K00, K02, K04, K05, K08), the most common dental disease in patients with phosphorus metabolism and phosphatase disorders. Among these patients, we assessed the proportion, duration, and distribution of dental diagnoses by age, from the date of the first assessment of phosphorus metabolism and phosphatase disorders.

RESULTS: A total of 14,408 patients with phosphorus metabolism and phosphatase disorders were identified during the study period, with a mean age of 44.10±18.90 years at diagnosis and 10,870 patients (75.44%) were diagnosed with dental complications. Patients under the age of five showed the highest proportion of dental diagnoses at 82.56%. The onset time of dental disease was 20.95 months (interquartile range: 5-29 months) from their first diagnosis of phosphorus metabolism and phosphatase disorders. The most prevalent dental issue was gingivitis and periodontal disease, affecting 63.07% of patients. Notably, disorders of tooth development and eruption were particularly high, at 80.95%, among those under five.

CONCLUSIONS: We found a high prevalence of dental diseases in phosphorus metabolism and phosphatase disorders patients with the highest vulnerability in patients under five. This study showed the disease burden of rare disease in the population level enhancing understanding of phosphorus metabolism and phosphatase disorders.