Safety, tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease.

We report here our experience with regular automated red cell exchange transfusion for the management of chronic complications of sickle cell disease in 50 patients in our institution from June 2011 to December 2014. The mean sickle hemoglobin level was 44% and 8.5% pre- and post-transfusion, respectively. Platelets were reduced by a mean 70% during the procedure with a count of less than 50 × 109 /l in 6% of cases. The alloimmunization rate was 0.065/100 units of red cells with no hemolytic reactions. Patients with no iron overload at baseline showed no evidence of iron accumulation with a mean liver iron concentration of 1.6 mg/g dry tissue and 1.9 mg/g dry tissue at baseline and 36 months, respectively. All six patients with pre-existing iron overload and on chelation therapy, showed a gradual reduction of their liver iron concentration and two patients could discontinue chelation during the follow-up period. Seventy percentage of patients who were on the programme for recurrent painful crises showed a sustained reduction in the number of emergency hospital attendances; the mean number of days in hospital for emergency treatment was 103 in the year prior to commencing ARCET and reduced to 62 (40%) after the first 12 months, 51 (50%) after 24 months, and 35 days (66%) after 36 months. J. Clin. Apheresis 31:545-550, 2016. © 2015 Wiley Periodicals, Inc.

Illness perception clusters at diagnosis predict psychological distress among women with breast cancer at 6 months post diagnosis.

OBJECTIVE: This study aimed to examine the extent to which illness perceptions and coping strategies among women diagnosed with breast cancer explain psychological distress at diagnosis and at 6 months post diagnosis relative to demographic and illness-related variables. METHODS: Women were recruited to the study shortly after diagnosis. A total of 90 women completed study materials (Illness Perception Questionnaire-Revised, the Cancer Coping Questionnaire and the Hospital Anxiety and Depression Scale) at time 1. The same questionnaires were sent approximately 6 months later to those who had consented at time 1, and completed questionnaires were returned by 72 women. RESULTS: Cluster analysis was used to identify groups of respondents who reported a similar profile of illness perception scores. Regression analysis demonstrated that one of these clusters was more likely to experience psychological distress than the other both at diagnosis and at 6 months post diagnosis. Illness perception cluster membership and positive focus type coping were the most important and consistent predictors of lower psychological distress at diagnosis and at 6 months post diagnosis. CONCLUSIONS: Illness perceptions remained relatively stable over the study period, and therefore we are unable to clarify whether changes in illness cognitions are associated with a corresponding change in psychological symptoms. Future research should evaluate the impact on psychological distress of interventions specifically designed to modify illness cognitions among women with breast cancer.

Autism Heterogeneity in a Densely Sampled U.S. Population: Results From the First 1,000 Participants in the RI-CART Study.

The objective of this study was to establish a large, densely sampled, U.S. population-based cohort of people with autism spectrum disorder (ASD). The Rhode Island Consortium for Autism Research and Treatment (RI-CART) represents a unique public-private-academic collaboration involving all major points of service for families in Rhode Island affected by ASD. Diagnosis was based on direct behavioral observation via the Autism Diagnostic Observation Schedule, Second Edition. For the first 1,000 participants, ages ranged from 21 months to 64 years. Using Geographic Information System and published prevalence rates, the overall cohort is estimated to represent between 20% and 49% of pediatric age persons in Rhode Island with ASD, with demographics representative of U.S. Census. We observed a high rate of co-occurring medical and psychiatric conditions in affected individuals. Among the most prominent findings of immediate clinical importance, we found that females received a first diagnosis of ASD at a later age than males, potentially due to more advanced language abilities in females with ASD. In summary, this is the first analysis of a large, population-based U.S. cohort with ASD. Given the depth of sampling, the RI-CART study reflects an important new resource for studying ASD in a representative U.S. population. Psychiatric and medical comorbidities in ASD constitute a substantial burden and warrant adequate attention as part of overall treatment. Our study also suggests that new strategies for earlier diagnosis of ASD in females may be warranted. Autism Res 2020, 13: 474-488. © 2020 International Society for Autism Research, Wiley Periodicals, Inc. LAY SUMMARY: The Rhode Island Consortium for Autism Research and Treatment (RI-CART) represents a unique public-private-academic collaboration involving all major points of service for families in Rhode Island affected by autism spectrum disorder (ASD). In this article, we provide results from the first 1,000 participants, estimated to represent >20% of affected families in the state. Importantly, we find a later age at first diagnosis of ASD in females, which potentially calls attention to the need for improved early diagnosis in girls. Also, we report a high rate of co-occurring medical and psychiatric conditions in affected individuals.

Adherence to nebulised antibiotics in cystic fibrosis.

OBJECTIVE: To examine the adherence of patients with cystic fibrosis to nebulised antibiotics. METHODS: A longitudinal design with adherence data collected over 12 weeks. 38 patients (mean age 24.6 years, S.D. 5.3) were recruited from an adult cystic fibrosis (CF) clinic. Adherence was electronically monitored using a Prodose adaptive aerosol delivery (AAD) device. RESULTS: Three indices of adherence were calculated: mean percentage of times the nebuliser was used as prescribed was 50.0% (S.D.=39.7, range 1.1, 155.6); mean percentage of days fully adhered was 31.6% (S.D.=29.4, range 0, 97.2); mean percentage of days nebuliser used at least once was 57.1% (S.D.=34.2, range 3.3, 100). CONCLUSION: Rates of adherence were generally low. Adherence was not associated with any variables apart from age. There were wide variations between individuals, and differences in rates of adherence depending on how this was defined, with potentially important health consequences for the patients. PRACTICE IMPLICATIONS: Technology that improves medication administration may still be associated with low rates of adherence. Health professionals need to be mindful of the pattern of non-adherence for each individual, and factors which may be influencing this.

Caffeine as a performance-enhancing drug in rats: sex, dose, housing, and task considerations.

Past animal studies of the performance-enhancing properties of stimulant drugs, such as caffeine, may have suffered from a number of procedural and ethical problems. For example. the housing condition of the animals was often not taken into consideration. As well, endurance tests, such as the forced swim task, sometimes involved ethically (and procedurally) questionable interference with natural swimming behaviour. Some of the manipulations, such as attaching a weight to the swimming animal's tail to increase the difficulty of the task and using mortality as a dependent variable, seem grotesque, even unnecessary. In this experiment, the performance-enhancing effects of caffeine in a modified forced swim task and a dominance task were evaluated using male and female rats as subjects (N=60), housed in either enriched or isolated environments. Analysis indicated that rats respond to caffeine as an interactive function of sex, housing, dose, and task characteristics. It was concluded that performance-enhancing properties of stimulant drugs may be the result of a complex interplay of variables, making simple generalizations questionable.

Cognitive-behavioural therapy and motivational intervention for schizophrenia and substance misuse. 18-month outcomes of a randomised controlled trial.

BACKGROUND: Comorbid substance misuse in people with schizophrenia is associated with poor clinical and social outcomes. There are few studies of psychological treatments for this population and little long-term follow-up of their benefits. AIMS: To investigate symptom, substance use, functioning and health economy outcomes for patients with schizophrenia and their carers 18 months after a cognitive-behavioural treatment (CBT) programme. METHOD: Patients with dual diagnosis from a randomised controlled trial of motivational intervention, individual CBT and family intervention were assessed on multiple outcomes at 18-month follow-up. Carers were assessed on symptom, functioning and needs over 12 months. Health economy data were collected over 18 months. RESULTS: There were significant improvements in patient functioning compared with routine care over 18 months. No significant differences between treatment groups were found in carer or cost outcomes. CONCLUSIONS: The treatment programme was superior to routine care on outcomes relating to illness and service use, and the cost was comparable to the control treatment.