PATIENT CHARACTERISTICS AND HEALTH OUTCOMES IN CHILDREN DIAGNOSED WITH MARFAN SYNDROME IN THE UNITED STATES

Author(s)

Gordon BD1, Noone JM2, Zacherle E2, Whitmire SM3, Clark LA1, Howden R1, Blanchette CM2
1University of North Carolina at Charlotte, Charlotte, NC, USA, 2Precision Health Economics, Davidson, NC, USA, 3University of North Carolina; Precision Health Economics, Charlotte, NC, USA

OBJECTIVES: Marfan syndrome is a rare systemic connective tissue disorder caused by mutations in the gene encoding fibrillin-1, which can contribute to serious cardiovascular (CV) and pulmonary related complications. While signs and symptoms may not appear until adulthood, some patients experience disease complications much earlier in life. Therefore, the purpose of this study was to evaluate the burden of CV and pulmonary related manifestations in children diagnosed with Marfan syndrome. METHODS: We used data from the 2012 Kids National Inpatient Sample. Marfan syndrome patients >10 years were identified using ICD-9 code 759.82. Patients were stratified by primary diagnosis; CV (CCS: 096, 097, 100, 101, 103-113, 115-118 or DRG: 025-027, 031-033, 219-221, 229, 230, 307) or pulmonary-related (CCS: 128-131, 133, 134) conditions. The reference group for all analyses included hospital discharges without a primary CV or pulmonary-related diagnosis. Descriptive and inferential statistics were used to compare demographics, healthcare outcomes, and hospital charges. RESULTS:

Conference/Value in Health Info

2017-05, ISPOR 2017, Boston, MA, USA

Value in Health, Vol. 20, No. 5 (May 2017)

Code

PHS4

Topic

Epidemiology & Public Health

Topic Subcategory

Disease Classification & Coding

Disease

Cardiovascular Disorders, Pediatrics, Rare and Orphan Diseases

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