Pb2674: clinical, neuroimaging, and behavioral risk factors for neurocognitive impairment in chinese patients with hemophilia: a multicenter study
HemaSphere(2023)
摘要
Topic: 35. Quality of life and palliative care Background: Despite a lack of clinical symptoms, subclinical microstructural changes in the brain might contribute to cognitive impairment in patients with hemophilia. Few studies have evaluated the impact of neuroimaging and psychosocial factors on cognitive function. Aims: To determine the prevalence and characteristics of cognitive impairment in patients with hemophilia, and identify associated risk factors in this population. Methods: We recruited hemophilia A or B patients who were aged ≥ 10 years old from three public hospitals in Hong Kong. A neurocognitive battery was administered to evaluate their attention, memory, processing speed and cognitive flexibility performances. They also underwent magnetic resonance imaging to identify cerebral microbleeds. Validated self-reported questionnaires were administered to assess their mental health status and adherence to prophylactic treatment. General linear modeling was used to investigate the association of neurocognitive outcomes with risks factors, adjusting for age and education attainment. Results: Forty-two patients were recruited (median age 32.0 years; 78.6% hemophilia A; 80.9% moderate-to-severe disease severity). Six patients (14.3%) had developed cerebral microbleeds. As a cohort, the mean cognitive scores of patients with hemophilia were significantly worse than the population norms on multiple cognitive domains (Figure 1), A subgroup of patients demonstrated impairments in cognitive flexibility (50%), motor processing speed (47.6%), and attention (19%). Hemarthrosis in the previous year was associated with worse attention (Estimate=7.62, standard error [SE]=3.81; p=0.049) and cognitive flexibility (Estimate=8.64, SE=5.84; p=0.043). Compared to patients with a normal weight, patients who were overweight/obese had worse cognitive flexibility (p=0.026). Depressive (Estimate=0.22, SE=0.21; p=0.023) and anxiety (Estimate=0.26, SE=0.07; p=0.0069) symptoms were associated with inattentiveness. Among patients who were receiving prophylactic treatment (71.4%), medication adherence was positively correlated with cognitive flexibility (p=0.037). Summary/Conclusion: A substantial proportion of patients with hemophilia demonstrated cognitive impairment, particularly higher-order thinking skills. Screening for cognitive deficits should be incorporated into routine care. Future studies should evaluate the association of neurocognitive outcomes with functional capacity and occupational/vocational outcomes.Keywords: Cerebral hemorrhage, Quality of life, Magnetic resonance imaging, Hemophilia
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关键词
hemophilia,neurocognitive impairment,chinese patients,neuroimaging
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