No abstract

• Bleeding in US hemophilia patients has decreased dramatically in parallel with increased use of prophylaxis.• Only prophylaxis started before age 4 years preserves normal joint function.This analysis of the US Hemophilia Treatment Center Network and the Centers for Disease Control and Prevention surveillance registry assessed trends in prophylaxis use and its impact on key indicators of arthropathy across the life-span among participants with severe hemophilia A. Data on demographics, clinical characteristics, and outcomes were collected prospectively between 1999 and 2010 at annual clinical visits to 134 hemophilia treatment centers. Trends in treatment and outcomes were evaluated using crosssectional and longitudinal analyses. Data analyzed included 26 614 visits for 6196 males; mean age at first registry visit was 17.7 years; and median was 14 (range, 2 to 69). During this time, prophylaxis use increased from 31% to 59% overall, and by 2010, 75% of children and youths <20 years were on prophylaxis. On cross-sectional analysis, bleeding rates decreased dramatically for the entire population (P < .001) in parallel with increased prophylaxis usage, possibly because frequent bleeders adopted prophylaxis. Joint bleeding decreased proportionately with prophylaxis (22%) and nonprophylaxis (23%), and target joints decreased more with prophylaxis (80% vs 61%). Joint, total, and target joint bleeding on prophylaxis were 33%, 41%, and 27%, respectively, compared with nonprophylaxis. On longitudinal analysis of individuals over time, prophylaxis predicted decreased bleeding at any age (P < .001), but only prophylaxis initiation prior to age 4 years and nonobesity predicted preservation of joint motion (P < .001 for each). Using a national registry, care providers in a specialized health care network for a rare disorder were able to detect and track trends in outcomes over time. (Blood. 2017;129(17):2368-2374

No abstract

No abstract

Background The cornerstone of effective management in heart failure (HF) is the ability to self-care. Aims include i) To determine factors influencing self-care in HF patients with cognitive impairment (CI) and ii) to determine the influence of cognitive domains on self-care in patients with HF and CI. Methods MEDLINE, CINAHL, EMBASE, EBSCOHost, PsychINFO, ProQuest Research Library, Health Technology Assessment Database, The Cochrane Library, Web of Science and Scopus databases were systematically searched. Original research describing the relationship between cognition and HF self-care in community-dwelling older persons with dementia/CI in English, published in a peer-reviewed journal from 1 st January(2000)-22 nd March(2016) was identified. Study and population characteristics, data sources, self-care processes, methods of cognitive assessment, cognitive domains affected, study outcomes, impact of impairment, and other risk factors of self-care impairment were abstracted by two reviewers. Results Of 10,688 studies identified, 14 met the inclusion criteria. Patients with HF and CI ranged from 14 to 73%. Where reported, self-care maintenance adequacy ranged from 50 to 61%; self-care management adequacy ranged from 14 to 36% and self-care confidence adequacy ranged from 0 to 44% on the Self-care of Heart Failure Index (SCHFI). All but one study predicted poor self-care ability according to poor outcome on cognitive testing. Additionally, specific cognitive domain deficits impaired self-care. Subjects with lower cognitive scores were less likely to seek assistance while subjects with depression had poor self-care abilities. Conclusions Clinicians must consider the type and severity of impairments in cognitive domains to tailor management. Awareness of depression, self-confidence and support access may modulate self-care ability. Electronic supplementary material The online version of this article (10.1186/s12872-019-1077-4) contains supplementary material, which is available to authorized users.