ABSTRACT
Systemic sclerosis is a rare, systemic autoimmune disease, characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. The disease is associated with a significantly increased morbidity and mortality, and can be rapidly progressive. Interstitial lung disease, renal hypertensive crisis, cardiac involvement and pulmonary arterial hypertension are life-threatening complications. Early treatment with immunosuppressive drugs can prevent progression and decrease morbidity and mortality.
Subject(s)
Raynaud Disease/etiology , Scleroderma, Systemic/complications , Adult , Aged , Disease Progression , Female , Gastrointestinal Diseases/epidemiology , Gastrointestinal Diseases/etiology , Humans , Hypertension/epidemiology , Hypertension/etiology , Joint Diseases/epidemiology , Joint Diseases/etiology , Male , Middle Aged , Muscle Weakness/epidemiology , Muscle Weakness/etiology , Raynaud Disease/epidemiologyABSTRACT
BACKGROUND: Today, treatment of the nondystrophic myotonias consists of mexiletine, although care has to be taken because of the proarrhythmogenic potential of this drug. In this article, we report years of experience with the carbonic anhydrase inhibitor acetazolamide. PATIENTS: We present three children with nondystrophic myotonias. RESULTS: During acetazolamide treatment, symptoms and signs of myotonia decreased in our children. CONCLUSIONS: Based on this clinical experience and the favorable pharmacologic profile of acetazolamide, it may be a good treatment option for children with nondystrophic myotonias.
Subject(s)
Acetazolamide/pharmacology , Carbonic Anhydrase Inhibitors/pharmacology , Myotonia/drug therapy , Acetazolamide/administration & dosage , Adolescent , Carbonic Anhydrase Inhibitors/administration & dosage , Child , Female , Humans , Male , Myotonia/diagnosis , Treatment OutcomeABSTRACT
BACKGROUND: Transparency in diabetes care requires quality indicators that are of interest to stakeholders in order to optimise their usage. Indicator development is often focused on consensus, and little is known about stakeholders' preferences for information on quality. AIM: To explore the preferences of consumers, providers, purchasers and policy makers for different quality domains and indicators in relation to the intended use of quality indicators. METHODS: Between June and December 2009, 14 semi-structured interviews were held with stakeholders who have a decisive vote in the selection of the national indicator set for diabetes care in the Netherlands. The following subjects were explored: (1) the aims of using information on quality; (2) the interpretation of and preferences for the quality domains of safety, timeliness, effectiveness and patient-centredness in relation to the user aims; and (3) the preferences for structure, process or outcome indicators. Content analysis was used to analyse qualitative data. RESULTS: Stakeholders had similar and different aims according to their roles. The interpretations of quality domains varied greatly between the stakeholders. Besides differences in interpretation, their preferences were similar. Most stakeholders prioritised patient-centredness above the other domains of quality, ranked in order of priority as safety, effectiveness and timeliness, whereas purchasers also prioritised efficiency. All stakeholders preferred to use process indicators or a mix of process and outcome indicators. CONCLUSIONS: The preferences of the stakeholders for quality indicators seem to be neither well-refined nor congruent. The implementation of an indicator set can probably be improved if the stakeholders' definitions and preferences for quality domains become more explicit during the selection process for indicators.
Subject(s)
Diabetes Mellitus/therapy , Quality Indicators, Health Care/organization & administration , Administrative Personnel , Health Personnel , Humans , Patient Preference , Patient Safety , Qualitative Research , Quality Indicators, Health Care/standards , Quality of Health Care/organization & administration , Time FactorsABSTRACT
BACKGROUND: Information on prescribing quality of diabetes care is required by health care providers, insurance companies, policy makers, and the public. Knowledge regarding the opinions and preferences of all involved parties regarding prescribing quality information is important for effective use of prescribing quality indicators. METHODS: Between June and December 2009 we conducted semi structured interviews with 16 key-informants representing eight different organizations in the Netherlands involved in healthcare quality measurement and improvement. The interview guide included topics on participants' opinions and preferences regarding existing types of prescribing quality indicators in relation to their aim of using quality information. Content analysis methods were used to process the resulting transcripts following the framework of predetermined themes. RESULTS: Findings from this qualitative study of stakeholder preferences showed that indicators focusing on undertreatment are found important by all participants. Furthermore, health care providers and policy makers valued prescribing safety indicators, insurance companies prioritized indicators focusing on prescribing costs, and patients' organization representatives valued indicators focusing on interpersonal side of prescribing. Representatives of all stakeholders preferred positive formulation of the indicators to motivate health care providers to participate in health improvement programs. A composite score was found to be most useful by all participants as a starting point of prescribing quality assessment. Lack of information on reasons for deviating from guidelines recommendations appeared to be the most important barrier for using prescribing quality indicators. According to the health care providers, there are many legitimate reasons for not prescribing the recommended treatment and these reasons are not always taken into account by external evaluators. The latter may cause mistrust of health care providers towards external stakeholders and limit the use of PQI in external quality improvement programs. CONCLUSION: Prescribing quality indicators are considered to be an important tool for assessing quality of provided diabetes care by all participants, although the preferences for specific types of indicators may differ by stakeholder depending on their user aim. Introduction of information systems to register the reasons for deviating from the recommended drug treatment may contribute to a more widespread use of PQI for assessment of provided health care quality to diabetic patents. This study identified the potential preferences regarding quality indicators for diabetes care, and this could be used for development of questionnaires to conduct a survey among a larger group of participants.
