Experience of a Single Center in NTBC Use in Management of Hereditary Tyrosinemia Type I in Libya

Background: Hereditary Tyrosinemia type I (HTI) is a metabolic disease caused by deficiency of fumarylacetoacetate hydrolase enzyme. Objectives: This study reports beside its clinical and biochemical presentation, the outcome of NTBC [2- (2-nitro-4-trifloro-methylbenzoyl)-1, 3-cyclohexanedion] treatment of the disease and evaluates its biochemical markers in 16 pediatric Libyan patients. Patients and Methods: The diagnosis was based on presence of high tyrosine levels in blood and succinylacetone in urine. Results: The consanguinity rate was 81.2%, the median age at onset, at diagnosis and at starting treatment were 4.5, 8, and 9.5 months respectively. At presentation hepatomegaly, jaundice, rickets and high gamma glutamyl transferase (GGT) were observed in 87.5% of patients. All patients had extremely high alpha fetoprotein (AFP) and high alkaline phosphatase (ALP) levels. Fifteen patients were treated with NTBC, normalization of PT (Prothrombine time) was achieved in average in 14 days. The other biochemical parameters of liver function (transaminases, GGT, ALP, bilirubin and albumin) took longer to improve and several months to be normalized. Survival rate with NTBC was 86.6%. Patients who started treatment in a median of 3 months post onset observed a fast drop of AFP in 90.6% of patients (P = 0.003). Abnormal liver function and rickets were the common presentations, GGT was an early cholestatic sensitive test. ALP was constantly high even in asymptomatic patients. Conclusions: In HT1 a faster dropping of AFP is a marker of good prognosis.

Development of an intervention to support medication management in patients with multimorbidity in primary care

The overarching aim of this thesis was to develop an intervention to support patient-centred prescribing in the context of multimorbidity in primary care. Methods A range of research methods were used to address different components of the Medical Research Council, UK (MRC) guidance on the development and evaluation of complex interventions in health care. The existing evidence on GPs’ perceptions of the management of multimorbidity was systematically reviewed. In qualitative interviews, chart-stimulated recall was used to explore the challenges experienced by GPs when prescribing for multimorbid patients. In a cross-sectional study, the psychosocial issues that complicate the management of multimorbidity were examined. To develop the complex intervention, the Behaviour Change Wheel (BCW) was used to integrate behavioural theory with the findings of these three studies. A feasibility study of the new intervention was then conducted with GPs. Results The systematic review revealed four domains of clinical practice where GPs experienced difficulties in multimorbidity. The qualitative interview study showed that GPs responded to these difficulties by ‘satisficing’. In multimorbid patients perceived as stable, GPs preferred to ‘maintain the status quo’ rather than actively change medications. In the cross-sectional study, the significant association between multimorbidity and negative psychosocial factors was shown. These findings informed the development of the ‘Multimorbidity Collaborative Medication Review and Decision-making’ (MY COMRADE) intervention. The intervention involves peer support: two GPs review the medications prescribed to a complex multimorbid patient together. In the feasibility study, GPs reported that the intervention was appropriate for the context of general practice; was widely applicable to their patients with multimorbidity; and recommendations for optimising medications arose from all collaborative reviews. Conclusion Applying theory to empirical data has led to an intervention that is implementable in clinical practice, and has the potential to positively change GPs’ behaviour in the management of medications for patients with multimorbidity.

What Are Effective Program Characteristics of Self-Management Interventions in Patients With Heart Failure? : An Individual Patient Data Meta-analysis

Background To identify those characteristics of self-management interventions in patients with heart failure (HF) that are effective in influencing health-related quality of life, mortality, and hospitalizations. Methods and Results Randomized trials on self-management interventions conducted between January 1985 and June 2013 were identified and individual patient data were requested for meta-analysis. Generalized mixed effects models and Cox proportional hazard models including frailty terms were used to assess the relation between characteristics of interventions and health-related outcomes. Twenty randomized trials (5624 patients) were included. Longer intervention duration reduced mortality risk (hazard ratio 0.99, 95% confidence interval [CI] 0.97–0.999 per month increase in duration), risk of HF-related hospitalization (hazard ratio 0.98, 95% CI 0.96–0.99), and HF-related hospitalization at 6 months (risk ratio 0.96, 95% CI 0.92–0.995). Although results were not consistent across outcomes, interventions comprising standardized training of interventionists, peer contact, log keeping, or goal-setting skills appeared less effective than interventions without these characteristics. Conclusion No specific program characteristics were consistently associated with better effects of self-management interventions, but longer duration seemed to improve the effect of self-management interventions on several outcomes. Future research using factorial trial designs and process evaluations is needed to understand the working mechanism of specific program characteristics of self-management interventions in HF patients.

Health information management : What business are we in?

Developments in information technology will drive the change in records management; however, it should be the health information managers who drive the information management change. The role of health information management will be challenged to use information technology to broker a range of requests for information from a variety of users, including he alth consumers. The purposes of this paper are to conceptualise the role of health information management in the context of a technologically driven and managed health care environment, and to demonstrat e how this framework has been used to review and develop the undergraduate program in health information management at the Queensland University of Technology.