OBJECTIVES

: XLH is a rare hereditary metabolic bone disease covering 80% of all cases of hereditary hypophosphatemia, characterized by phosphate-wasting due to an excess of FGF23. It causes bone deformity, which tends to appear when children begin to grow. Previous SHI claims data analyses showed that the mean age of incident XLH patients is 55 years. Hence, further analysis for the detection of incident children with that disease was conducted.

METHODS

: Based on a retrospective analysis of a six-year database from the German SHI system, a representative sample of ~4 million insurants was obtained. Patients received at least one full or semi-residential inpatient diagnosis or two confirmed outpatient diagnoses in different quarters of the year (E83.30 ICD-10 GM) and continuous insurance coverage. For detecting children with XLH different combinations of diagnoses, medication and procedures, which are associated with XLH, were analysed.

RESULTS

: CONCLUSIONS

: Analyses indicate, that a certain amount of pediatric XLH patients may be misdiagnosed. Up to 60% of all XLH patients are not treated with documented conventional treatment (independent of age) indicating a gap of care of XLH patients and a need for further disease awareness. Oral phosphorus solution as a medicinal product used in treating children with XLH is not available in Germany and the healthcare quality in this cohort is difficult to judge.

Formularende