Adrenoleukodystrophy (ALD) is a rare debilitating neurodegenerative genetic disorder and is characterized by multiple phenotypes. The most severe phenotypes are the childhood cerebral forms, which can initially display as hyperactivity and can progress to a vegetative state or death. The objective of this study was to characterize pediatric ALD patient inpatient stays and identify differences between age cohorts. The 2016 Kids Inpatient Database (KID) was utilized to identify discharges containing a primary or secondary ICD-10-CM code for ALD (‘E7152’). Patients were divided into age cohorts: child (ages 0-10) and adolescent (ages 11-21). Demographic information was compiled in aggregate, while clinical and economic characteristics were compared between age cohorts using t-tests and chi-squared tests. In total, 161 ALD discharges were identified (52% child, 48% adolescent). The mean age was 11.29 (SD: 4.59), and the majority of patients were male (98.1%). The demographic breakdown revealed an over-representation of Black and Hispanic patients in the sample (White: 27.3%, Black: 22.4%, Hispanic: 24.8%, and Other: 25.5%). The most common primary payer was Medicare (57.8%) followed by Medicaid (37.3%). When stratified by age, adolescents had significantly higher Charlson Comorbidity Index (CCI) scores (0.71 vs. 0.27; p=0.0005) and higher rates of comorbid COPD (21.79% vs. 7.23%; p=0.008) and paralysis (20.51% vs. 8.43%; p=0.026). There were no significant differences between mean length of stay or total charges between age cohorts. While the population was divided by age, there are still important unknowns within the comparison groups. The majority of patients were coded as “X-linked ALD, unspecified type”, which may have resulted in multiple ALD phenotypes being included in the adolescent cohort, such as adolescent patients formerly diagnosed with childhood cerebral ALD. This lack of specificity in coding could potentially lead to ranges of severity within each group, which could be addressed with better coding or clinical detail.
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