BACKGROUND: @entity2033 ( @entity2033 ) is a rare @entity179 with a prevalence of 6 per 100.000. Despite increasing research activity on @entity2033 , evidence on healthcare utilization, @entity1 ' needs for healthcare services and Health-Related Quality of Life (HRQoL) is still sparse. The present study describes HRQoL in a Norwegian cohort of @entity2033 @entity1 , and assesses associations between unmet healthcare and social support service needs and HRQoL. METHODS: In this cross-sectional population-based study, 84 @entity1 with a clinical diagnosis of @entity2033 living in the South-East of Norway completed the HRQoL questionnaire EuroQol, EQ-5D-3L. Unmet needs for healthcare and social support services were assessed by the Needs and Provision Complexity Scale (NPCS). Furthermore, functional ability was determined using the Unified @entity2033 Rating Scale (UHDRS) Functional assessment scales. Socio-demographics (age, gender, marital status, occupation, residence, housing situation) and clinical characteristics (disease duration, total functional capacity, comorbidity) were also recorded. Descriptive statistics were used to describe the @entity1 ' HRQoL. Regression analyses were conducted in order to investigate the relationship between unmet healthcare needs and self-reported HRQoL. RESULTS: The @entity1 were divided across five disease stages as follows: Stage I: n   =   12 (14%), Stage II: n   =   22 (27%), Stage III: n   =   19 (23%), Stage IV: n   =   14 (16%), and Stage V: n   =   17 (20%). Overall HRQoL was lowest in @entity1 with @entity1442 (Stages IV and V), while @entity1 in the middle phase (Stage III) showed the most varied health profile for the five EQ-5D-3L dimensions. The regression model including level of unmet needs, clinical characteristics and demographics (age and education) accounted for 42% of variance in HRQoL. A higher level of unmet needs was associated with lower HRQoL (b value - 0.228; p   =   0.018) whereas a better total functional capacity corresponded to higher HRQoL (b value 0.564; p   <   0.001). CONCLUSIONS: The study findings suggest that @entity1 with @entity2033 do not receive healthcare services that could have a positive impact on their HRQoL.

Health-related quality of life and unmet healthcare needs in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity1442 :: ('MESH:D020178', 'Disease') :: ['advanced disease'] @entity179 :: ('MESH:D019636', 'Disease') :: ['neurodegenerative disorder'] @entity2033 :: ('MESH:D006816', 'Disease') :: ["Huntington's Disease", "Huntington's disease", 'HD']
@entity2033 :: (MESH:D006816,Disease) :: ['Huntington's disease']
