LB757 Hospitalization for Chediak-Higashi Syndrome: A national inpatient sample analysis

Abstract

Chediak Higashi syndrome (CHS) is a rare autosomal recessive disease that presents as oculocutaneous albinism and immunodeficiency. We performed the first ever national inpatient analysis of CHS in the United States. Patients with a primary or secondary diagnosis of CHS during 2015-2018 were selected using ICD-10 codes to determine the most common reasons for hospitalization, demographic features, and inpatient burden and outcomes. An estimated 155 discharges had a primary or secondary diagnosis of CHS nationwide, representing an inpatient prevalence of 1.3 per million discharges.