Abstract

Objective: (s): To assess and characterize healthcare resource utilization (HRU) in children with the rare, genetic, multisystem disorder, Alagille syndrome (ALGS).

Study design: This retrospective analysis reviewed commercially- and Medicaid-insured claims from October 1, 2015 through December 31, 2019 to assess HRU in patients with ALGS. As there is no specific International Classification of Diseases (ICD)-10 code for ALGS, patients were identified using the following algorithm: ≥1 claim with diagnosis code Q44.7 (other congenital malformations of the liver), <18 years of age, with no history of biliary atresia (ICD-10 code: Q44.2) and ≥6 months of insurance eligibility prior to diagnosis. HRU was summarized per patient per year over all available claims post-diagnosis.

Results: 171 commercially-insured and 215 Medicaid-insured patients with ALGS were available for analysis. Annually, commercially-insured and Medicaid-insured patients averaged 31 medical visits (range 1.5-237) and 48 medical visits (range 0.7-690), respectively. The most common visits were outpatient with the majority encompassing lab/imaging and primary care visits (commercially-insured: 21 [range 0.0-183]; Medicaid-insured: 26 [range 0.0-609]). Inpatient visits were the highest driver of costs in both the commercial and Medicaid populations.

Conclusion: Patients with ALGS have a substantial HRU burden driven largely by numerous outpatient visits and costly inpatient stays. Given the complexity and variability of ALGS presentation, patients may benefit from multidisciplinary and subspecialized care.