A UK national audit of hereditary and acquired angioedema.

Hereditary angioedema (HAE) and acquired angioedema (AAE) are rare life-threatening conditions caused by deficiency of C1 inhibitor (C1INH). Both are characterized by recurrent unpredictable episodes of mucosal swelling involving three main areas: the skin, gastrointestinal tract and larynx. Swelling in the gastrointestinal tract results in abdominal pain and vomiting, while swelling in the larynx may be fatal. There are limited UK data on these patients to help improve practice and understand more clearly the burden of disease. An audit tool was designed, informed by the published UK consensus document and clinical practice, and sent to clinicians involved in the care of HAE patients through a number of national organizations. Data sets on 376 patients were received from 14 centres in England, Scotland and Wales. There were 55 deaths from HAE in 33 families, emphasizing the potentially lethal nature of this disease. These data also show that there is a significant diagnostic delay of on average 10 years for type I HAE, 18 years for type II HAE and 5 years for AAE. For HAE the average annual frequency of swellings per patient affecting the periphery was eight, abdomen 5 and airway 0·5, with wide individual variation. The impact on quality of life was rated as moderate or severe by 37% of adult patients. The audit has helped to define the burden of disease in the UK and has aided planning new treatments for UK patients.

Assessment of sarcoplasmic reticulum Ca2+ depletion during spontaneous Ca2+ waves in isolated permeabilized rabbit ventricular cardiomyocytes.

In this study, Ca2+ release due to spontaneous Ca2+ waves was measured both from inside the sarcoplasmic reticulum (SR) and from the cytosol of rabbit cardiomyocytes. These measurements utilized Fluo5N-AM for intra-SR Ca2+ from intact cells and Fluo5F in the cytosol of permeabilized cells. Restricted subcellular volumes were resolved with the use of laser-scanning confocal microscopy. Local Ca2+ signals during spontaneous Ca2+ release were compared with those induced by rapid caffeine application. The free cytoplasmic [Ca2+] increase during a Ca2+ wave was 98.1% +/- 0.3% of that observed during caffeine application. Conversion to total Ca2+ release suggested that Ca2+ release from a Ca2+ wave was not significantly different from that released during caffeine application (104% +/- 6%). In contrast, the maximum decrease in intra-SR Fluo-5N fluorescence during a Ca2+ wave was 82.5% +/- 2.6% of that observed during caffeine application. Assuming a maximum free [Ca2+] of 1.1 mM, this translates to a 96.2% +/- 0.8% change in intra-SR free [Ca2+] and a 91.7% +/- 1.6% depletion of the total Ca2+. This equates to a minimum intra-SR free Ca2+ of 46 +/- 7 microM during a Ca2+ wave. Reduction of RyR2 Ca2+ sensitivity by tetracaine (50 microM) reduced the spontaneous Ca2+ release frequency while increasing the Ca2+ wave amplitude. This did not significantly change the total depletion of the SR (94.5% +/- 1.1%). The calculated minimum [Ca2+] during these Ca2+ waves (87 +/- 19 microM) was significantly higher than control (p < 0.05). A computational model incorporating this level of Ca2+ depletion during a Ca2+ wave mimicked the transient and sustained effects of tetracaine on spontaneous Ca2+ release. In conclusion, spontaneous Ca2+ release results in substantial but not complete local Ca2+ depletion of the SR. Furthermore, measurements suggest that Ca2+ release terminates when luminal [Ca2+] reaches approximately 50 microM.

Autonomy: a professional issue of concern for nurse practitioners.

Autonomy has been a professional issue for nurses and for nurse practitioners (NPs). Health care reform will be critical to continuing and improving the autonomous roles of NPs. Without autonomy to practice their skills freely as primary health care providers, the roles of NPs will most likely be limited in any reformed health care system. Insights into the issue of autonomy for NPs are discussed.

Managed outcomes: a strategy to improve the nation's health.

Current health care delivery systems in the United States have led to high cost, uneven quality, less than universal coverage, undue emphasis on a medical/clinical model, and scant attention to primary care and prevention. In the context of health care reform, a new strategy is introduced that reverses present trends and incentives, called managed outcomes. This strategy is not specific to any particular health care delivery system. Managed outcomes encourages experimentation and flexibility in the design of health care systems and fosters primary care, health promotion, and disease prevention models. It links purchasing decisions to established specific and measurable goals that can provide quality and cost-effective services to improve health.

The games of politics.

Understanding and learning to play the games of politics can increase the political efficiency of nurse practitioners (NPs) and assist them in advancing their roles and functioning in the crowded health care arena. One framework that can be applied to the political process is Allison's Governmental Political Model. Allison's model approaches the process of politics as competitive games. This article presents Allison's political model and demonstrates how understanding and learning to play political games has potential to promote NP political effectiveness.

Nasopharyngeal neoplasms and their association with adult onset otitis media with effusion.

Patients with adult onset otitis media with effusion are generally subjected to an examination and biopsy of the nasopharynx under a general anaesthetic to exclude a neoplasm of the post-nasal space. The likelihood in Caucasian countries of a tumour being detected has not been clearly defined. The clinical features of the 55 patients with nasopharyngeal malignancy presenting to the Glasgow hospitals since 1970 have been analysed, and the incidence of malignancy in all adults presenting with middle ear effusions has been assessed in order to define the association of the 2 conditions. The study confirmed that while an effusion is commonly associated with a nasopharyngeal tumour (33%), in only 2% was it the sole clinical manifestation at the initial consultation. In addition, adults with effusions, but without other symptoms and signs suggestive of a tumour, are unlikely to harbour a tumour, the incidence in this review being 0.4%. Therefore, an examination and biopsy of the nasopharynx under general anaesthesia is likely to give a very low yield of additional information in cases of isolated middle ear effusions in adults, and the cost effectiveness of the procedure should be questioned.