The following is a summary of “Health Care Resource Utilization by Patients with Alagille Syndrome,” published in the FEBRUARY 2023 issue of Pediatrics by Ebel, et al.

For a study, researchers sought to analyze the utilization of healthcare resources in children with Alagille syndrome, a rare genetic disorder affecting multiple systems in the body. 

To evaluate HRU in individuals with Alagille syndrome, they examined Medicaid- and commercially insured claims from October 1, 2015, to December 31, 2019. Alagille syndrome does not have a specific International Classification of Diseases-10 code, so patients were found using the following algorithm: ≥1 claim with diagnosis code Q44.7 (other congenital malformations of the liver); <18 years of age; no history of biliary atresia (International Classification of Diseases-10 code: Q44.2); ≥6 months of insurance eligibility before diagnosis. Across all available claims postdiagnosis, HRU was summed by the patient per year.

There were 215 patients with Alagille syndrome covered by Medicaid and 171 with commercial insurance. Patients with commercial insurance made an average of 31 medical visits per year, with a range of 1.5 to 237, and patients with Medicaid insurance made an average of 48 visits per year, with a range of 0.7 to 690. The majority of the appointments included lab/imaging and primary care visits, and the most frequent ones were outpatient (commercially insured: 21 [range, 0.0-183]; Medicaid-insured: 26 [range, 0.0-609]). For commercial and Medicaid populations, inpatient visits were the major cost driver.

The study concluded that children with Alagille syndrome have a significant burden of healthcare resource utilization due to multiple outpatient visits and expensive inpatient stays. Given the complex and variable presentation of Alagille syndrome, they suggested that multidisciplinary and subspecialized care may be beneficial for these patients.

Source: jpeds.com/article/S0022-3476(22)00855-1/fulltext