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Technology and the environment: supportive resource or barrier for people with developmental disabilities?

Findings from needs assessments and abandonment studies point to issues with health care providers, particularly in their ability to listen to the needs of the consumer and important others regarding AT-EI. Professionals need to listen to what people are telling them or, in many cases, what they are not telling them. Actions and nonverbal messages can speak very loudly. Strategies to communicate and collaborate with consumers need to be developed. Regardless of ability to communicate or the severity of the impairments the person may be experiencing, it is important to withhold judgments that may underestimate a person's potential or desire to be in control of life decisions. AT-EI service have often seen people labeled with severe or profound intellectual disabilities challenge that diagnosis after accessing a communication or access system. Likewise, a person with a severe disability has the right to supportive resources and to the same level of respect, dignity, and quality of life as any other member of society. Using the technology and adapting the environment to provide opportunities for consumers to "voice" their wishes and control their lives can be an effective strategy to collaborate. When focusing on a rights-based philosophy, recognizing the difference between physical independence (e.g., physical and/or cognitive ability to do a task by oneself) and self-care management (e.g., access to and power to manage the supportive resources to live in the community regardless of level of physical ability) is important. We all rely on supports in our lives, whether it be tools or technology to help us do a job or another person, yet when we evaluate people with disabilities, the expectation is for people to function independently [23,24]. They even receive lower scores on functional assessments if they are using a piece of technology to do an activity. By shifting the focus to management of and access to resources versus level of physical dependence or burden, health care professionals can play a role in linking people to such resources as AT-EI and related services and strategies to support community living. Such a shift in focus also enables professionals to validate interdependence; that is, the give-take relationships that people have with each other to support each other [25]. The use of AT-EI by people with developmental disabilities often involves an interdependent relationship in which another person may help set up the environment or technology and, in turn, the consumer can then reciprocate and engage in an activity or a relationship [1]. Health care professionals also need to better understand and take into consideration the social context, its influence on consumers' use or nonuse of AT-EI, and the long-term influence on community living and participation decisions [1]. Nurses can involve important others in the process by listening to and considering their needs, and ensuring that they are informed about options, the benefits of using AT-EI for the consumer and themselves, and how to set up and troubleshoot the AT-EI. In cases where important others are not supportive, health care professionals may be in a position to link the consumer with other consumers and advocacy groups such as Centers for Independent Living or Self Advocates Becoming Empowered that may offer that support as well as membership in a collective community engaged in systems change. Health care professionals can serve as a system interface by linking people to information and resources to make informed decisions [26]. Resources on developmental disability and health, common issues that may occur, and life course planning help people identify functional issues and early signs of accelerated aging and proactively use the environment and technology to stay in living situations of choice. Few health care professionals are well prepared to provide services to people with developmental disabilities as they age; a great need exists for providers of such specialized services and for proactive later-life screenings that can identify issues early and make the most use of AT-EI strategies to address aging issues [26,28]. At any given point in time in the life of people with developmental disabilities, many different professionals and systems may be involved in decisions that could include AT-EI. Medical, educational, vocational, independent living, and case management systems may all be working with the person; however, there is often limited or no communication between them, particularly as the person ages or transitions between settings. Health care professionals, even when they are working with an individual on a limited basis, can and should take on active roles in linking consumers and important others with other systems and should ensure that information about their AT-EI needs is transferred accurately between systems. Most likely, nurses may be in a role to refer a person to specialized services, whether they may be medical, rehabilitative, AT-EI-specific, or disability advocacy groups that can help support the person as they face barriers or seek out AT. Nurses may also be in a role to pass on important information about the person's health and medical status that can help to better inform AT-EI decisions to ensure the AT meets the person's needs across contexts. As an interface, nurses may assume a role as a supporting advocate for accessing resources, not as a gatekeeper who makes decisions for people. This includes referring individuals with developmental disabilities to people and groups that know how to get AT-EI, how to fund it, and how to troubleshoot it, and linking them to other people with disabilities who are sharing strategies in person and on-line. It also includes focusing beyond basic self-care and considers AT-EI strategies that enable a person to participate in high meaning activities and roles in the home and the community. Participation in activities identified as highly meaningful and important to the person, such as participating in a religious community, networking with other people on-line, gardening, or being a member of a community group, to name a few, can positively contribute to health, wellness, and quality of life; the challenge is to create and adapt the environment (social, physical, and societal) to support participation choices and control.

Adolescent↗

Minimal, progressive, and fluctuating hearing losses in children. Characteristics, identification, and management.

Referring to specific types of hearing loss as "minimal" or "mild" seems to imply that their effects are equally mild or negligible. A growing body of literature, however, supports the notion that such losses can have a significant impact on the communicative and educational development of young children. Although OME is considered a common childhood ailment, mounting evidence suggests that it is not always benign and may contribute to significant educational and communicative difficulties in some young children when accompanied by conductive hearing loss. Even very mild bilateral and unilateral SNHL seems to contribute to problems in the areas of social and emotional function, educational achievement, and communication in some children. Because these hearing losses are so mild, they may not be immediately recognized as the source of such difficulties. The purpose of this report is to heighten the general pediatrician's awareness of the significance of even very mild or minimal hearing losses in children. As the gatekeepers for children's health care, pediatricians are typically the primary recipients of parental expressions of concern and the initiators of evaluations or referrals to address such.

Adolescent↗

Managed care and the pediatric emergency department.

The growth of managed care has provided health benefits to millions of children while attempting to control the increase in health care costs. In adhering to these goals, MCOs are often at odds with emergency departments, and the emergency department physicians providing emergency care. The appropriateness or inappropriateness of emergency department visits can be disputed, but no criteria have been established. Even the definition of emergency is debated, although many states are adopting a prudent layperson standard. Emergency medicine physicians, primary care providers, and MCOs must cooperate to fully educate parents about the appropriate use of pediatric emergency services. Patients and MCOs should use facilities that can deliver pediatric emergency and critical care or provide appropriate transport systems to facilities that can. COBRA and EMTALA set the legal requirements to which emergency departments must comply when patients present for care. The basic caveats under COBRA require a medical screening examination for every patient and the stabilization of all patients with emergency medical conditions before inquiring about insurance or patients' ability to pay. A part of gatekeeping, MCOs often require authorization for treatment. MCOs authorize payment only. Evaluation and emergency treatment should not be withheld pending authorization. After the medical screening examination, recommended treatment should be in patients' best interests. All patients with potentially life-threatening conditions should be stabilized before transport, and all transfers must comply with the EMTALA. The transfer of unstable patients purely for economic reasons is a violation of the EMTALA. When stable, patients may be transferred to other facilities, but patients requiring specialty care should be taken to facilities best able to provide that care. Financial considerations should be superseded by medical necessity. Finally, improvements can be made in the way emergency medical service is provided to children within the current managed care system. The primary care provider is in a key position to inform parents about the types of pediatric emergencies, what to do in case one occurs, and to provide follow-up care. MCOs should incorporate clear information on pediatric emergencies. A mutual understanding of services needed, and how best to provide those services, are needed to forge a system that is responsive to children's emergency care needs.

Child↗

Review of studies using multivariable analysis of clinical and exercise test data to predict angiographic coronary artery disease.

Multivariable analysis of clinical and exercise test variables has the potential to become both a useful tool for assisting in the diagnosis of coronary artery disease and reducing the cost of evaluating patients with suspected coronary disease. Managed care and capitation require that tests such as the exercise test or its replacements, be used only when they can accurately and reliably identify which patients need medications, counseling, or further evaluation or intervention. The replacements for the standard exercise electrocardiogram test require expensive equipment and personnel, and their incremental value is currently being evaluated. Because general practitioners are to function as gatekeepers and decide which patients must be referred to the cardiologist, they will need to use the basic tools they have available (ie, history, physical exam, and the exercise test) in an optimal fashion. However, the discriminating power of the variables from the medical history and exercise test remains unclear because of inadequate study design and differences in study populations. There is a need for further evaluation of these routinely obtained variables to improve the accuracy of prediction algorithms especially in women. Of paramount concern is the need to avoid workup bias by having patients agree to testing before the decision for angiography is made. The portability and reliability of these equations must be shown because access to specialized care must be safeguarded. By reviewing the available studies considering clinical and exercise test variables to predict coronary angiographic findings, we have attempted to provide guidelines and recommendations for a more uniform approach to this endeavor in future investigations. Hopefully, the next generation of multivariable equations will be robust and portable, and empower the clinician to assure the cardiac patient access to appropriate cardiac care.

Coronary Angiography↗

Reevaluation of organ transplantation criteria. Allocation of scarce resources to borderline candidates.

Organ transplantation in the 1960s proceeded according to a "lifeboat" ethics formula with physicians acting as "gatekeepers." Selection of transplant recipients is now based on medical urgency and waiting time. Some candidates continue to be given low priority by virtue of psychological impairment. The three As--advanced age, acquired immune deficiency syndrome (or positive human immunodeficiency virus status), and alcoholism--also stand out as characteristics that tend to exclude candidates. Cancer is another relative or absolute contraindication to transplantation. This article focuses retrospectively on the psychosocial and medical aspects of the decision to include six patients at Massachusetts General Hospital who were selected for organ transplantation despite their borderline candidacy. The authors introduce four lines of thinking that decision-makers might use to either include or exclude marginal candidates (e.g., the physician's interpretation of what duty requires, the patient's or surrogate's wishes, cost-benefit considerations, or the need for research to improve our scientific understanding of transplantation issues) and discuss an ethical approach that supports each line of thinking. The authors conclude that not all of the ethical approaches lead practitioners and policymakers to the same conclusions regarding the optimum size of or who should be a part of the recipient pool. The future of who receives transplants and why depends at least in part on the underlying ethical considerations that are deemed appropriate as determinants of practice and policy.

Adult↗

An agenda for primary care psychiatry.

With the advent of managed care and the primary care gatekeeper, psychiatry's relationship to primary care is shifting. Four recent surveys suggest that, in general, departments of psychiatry have done little to restructure their relationships with primary care. This article proposes an agenda for developing primary care psychiatry programs in departments of psychiatry. The rationale for shifting resources toward primary care psychiatry is followed by a discussion of the goals of primary care psychiatry programs. The agenda presents specific high-priority projects in the areas of research, education, and clinical care, citing examples of existing initiatives and discussing the resources needed for funding primary care psychiatry programs.

Education, Medical↗

The sheep kidney contains a novel unidirectional, high affinity NADP(+)-dependent 11 beta-hydroxysteroid dehydrogenase (11 beta-HSD-3).

The 11 beta-hydroxysteroid dehydrogenase (11 beta-HSD) enzymes convert corticosterone and cortisol to 11-dehydrocorticosterone and cortisone, and are thought to convey extrinsic specificity to the mineralocorticoid receptor by limiting access of the relatively more abundant glucocorticoids to it. Two different 11 beta-hydroxysteroid dehydrogenases (11 beta-HSD) have been described and cloned. The liver-type, NADP(+)-dependent 11 beta-HSD-1, has an affinity in the micromolar range and bidirectional activity. The NAD(+)-dependent 11 beta-HSD-2 has a higher affinity, in the nanomolar range, and exhibits only oxidase activity. 11 beta-HSD-2, because of its affinity and co-localization with the mineralocorticoid receptor, is likely to serve as the "gatekeeper" for the mineralocorticoid receptor in the kidney. Although the rat kidney expresses both isoforms, only the high-affinity, NAD(+)-dependent 11 beta-HSD-2 has been reported in the sheep kidney. We found both 11 beta-HSD NAD(+)- and NADP(+)-dependent activities in sheep kidney to be present. The NAD(+)-dependent activity exhibited a Km similar to that reported in the literature, 3.85 +/- 1.28 nM for corticosterone and 21.3 +/- 5.8 for cortisol, was distributed in approximately equal amounts between microsomes and nuclei, and was unidirectional, converting corticosterone to 11-dehydrocorticosterone. The enzyme exhibited prominent substrate inhibition. The NADP(+)-dependent activity had a Km for corticosterone of 4 +/- 1.3 nM for a Km for cortisol of 35.2 +/- 2 nM, 100-fold lower than that described for the 11 beta-HSD-1 in the liver of sheep and other species, and was more prevalent in the microsomes than the nuclei. This enzyme was not inhibited by its substrate. The NAD(+)-dependent activity was approximately 3-10 times greater than the NADP(+)-dependent activity when incubated with 5 nM corticosterone substrate, but had similar activity when incubated with 100 nM substrate concentrations. CHOP cells (a modified Chinese hamster ovary cell line) transiently transfected with the sheep 11 beta-HSD-2 plasmid exhibited a marked preference for NAD+ as co-factor. Oxidation of corticosterone by transfected cells in the presence of NADP+ was present, but minimal; NADP+ did not support the metabolism of cortisol, the primary glucocorticoid of sheep. These data suggest the existence of another NADP(+)-dependent enzyme, 11 beta-HSD-3, which, because of its high affinity and unidirectional oxidase activity, may play a physiological role in the modulation of glucocorticoid binding to both the mineralocorticoid and glucocorticoid receptors.

11-beta-Hydroxysteroid Dehydrogenases↗

PapD and superfamily of periplasmic immunoglobulin-like pilus chaperones.

The formation of a P pilus requires a molecular chaperone in the periplasm and a molecular usher in the outer membrane. Each pilus is composed of six different types of proteins that are assembled into a composite fiber in a defined order. The correct folding of subunits into domains that can serve as assembly modules requires an association with the periplasmic chaperone. PapD is the prototype member of the family of bacterial pilus chaperones that have a three-dimensional structure consistent with an immunoglobulin fold. In general, proteins with an immunoglobulin fold structure have molecular recognition functions in eukaryotic cells that are often integrated with effector functions. PapD has also a recognition function, binding nascently translocated pilus subunits and maintaining them in assembly-competent conformations. The association of the chaperone with the subunit triggers the targeting of the latter to an outer membrane usher. The usher serves as a molecular gatekeeper, allowing the ordered incorporation of the pilus subunits into the pilus structure from the periplasmic chaperone complexes. The two immunoglobulin-like domains of PapD are oriented to form a cleft that contains the subunit binding site. This is a different binding paradigm from that used by either antibodies or the growth hormone receptor. The blend of genetics, biochemistry, X-ray crystallography, and carbohydrate chemistry in the study of pili biogenesis will continue to give insight into some of the most basic intellectual challenges in molecular biology concerning how proteins fold into domains that serve as modules for the formation of larger assemblies, and relating these processes to microbial pathogenesis.

Adhesins, Escherichia coli↗

The role of neurotransmission and the Chopper domain in p75 neurotrophin receptor death signaling.

The role of p75 neurotrophin receptor (p75NTR) in mediating cell death is now well characterized, however, it is only recently that details of the death signaling pathway have become clearer. This review focuses on the importance of the juxtamembrane Chopper domain region of p75NTR in this process. Evidence supporting the involvement of K+ efflux, the apoptosome (caspase-9, apoptosis activating factor-1, APAF-1, and Bcl-xL), caspase-3, c-jun kinase, and p53 in the p75NTR cell death pathway is discussed and regulatory roles for the p75NTR ectodomain and death domain are proposed. The role of synaptic activity is also discussed, in particular the importance of neutrotransmitter-activated K+ channels acting as the gatekeepers of cell survival decisions during development and in neurodegenerative conditions.

Animals↗

Beyond a relay nucleus: neuroimaging views on the human LGN.

The lateral geniculate nucleus (LGN) is the thalamic station in the retinocortical projection and has traditionally been viewed as the gateway for sensory information to enter the cortex. Here, we review recent studies of the human LGN that have investigated the retinotopic organization, physiologic response properties, and modulation of neural activity by selective attention and by visual awareness in a binocular rivalry paradigm. In the retinotopy studies, we found that the contralateral visual field was represented with the lower field in the medial-superior portion and the upper field in the lateral-inferior portion of each LGN. The fovea was represented in posterior and superior portions, with increasing eccentricities represented more anteriorly. Functional MRI responses increased monotonically with stimulus contrast in the LGN and in visual cortical areas. In the LGN, the dynamic response range of the contrast function was larger and contrast gain was lower than in the cortex. In our attention studies, we found that directed attention to a spatial location modulated neural activity in the LGN in several ways: it enhanced neural responses to attended stimuli, attenuated responses to ignored stimuli, and increased baseline activity in the absence of visual stimulation. Furthermore, we showed in a binocular rivalry paradigm that neural activity in the LGN correlated strongly with the subjects' reported percepts. The overall view that emerges from these studies is that the human LGN plays a role in perception and cognition far beyond that of a relay nucleus and, rather, needs to be considered as an early gatekeeper in the control of visual attention and awareness.

Attention↗

Managed care and its impact on American urology.

America's health care is undergoing a revolution. A previous private, fee-for-service, delivery system chiefly centered around hospital specialty care is rapidly being replaced by a commercialized system of managed care, controlled by businessmen whose prime motive is profit. Increasing emphasis of these managed care organizations is upon primary physicians who function as gatekeepers. While this new commercialized method of health care has been attended with reductions in the previous omnipresent health care inflation our country has experienced for the past several decades, its impact on quality of care and patient choice of physician remain a great concern. Especially vulnerable in this new system are our nation's academic centers, which, burdened with responsibility for education and research, are at a disadvantage in the competitive cost-based bidding for managed care contracts. Urology work force issues and the number of urologists in our nation remain another concern for urologists as they compete for access to patients in this new highly competitive environment. In a 1995 survey of a cohort of urologists in seven states, the respondents reported 35.8% of gross income came from managed care contracts, 86% reported the need for preservice approval for many diagnostic and therapeutic undertakings, 87% reported an inability to refer complex cases outside the Managed Care Organization (MCO) network, and 23% reported they were required to retain patients for treatment who they would have otherwise referred to a more qualified urologist. The majority of American urologists are reporting dropping gross revenues and increasing overhead in their dealings with managed care contracts. The advent of managed care is being attended with dropping gross revenues, increasing overhead costs and interference with the practice patterns of American urologists.

Academic Medical Centers↗

Roundabout controls axon crossing of the CNS midline and defines a novel subfamily of evolutionarily conserved guidance receptors.

The robo gene in Drosophila was identified in a large-scale mutant screen for genes that control the decision by axons to cross the CNS midline. In robo mutants, too many axons cross and recross the midline. Here we show that robo encodes an axon guidance receptor that defines a novel subfamily of immunoglobulin superfamily proteins that is highly conserved from fruit flies to mammals. For those axons that never cross the midline, Robo is expressed on their growth cones from the outset; for the majority of axons that do cross the midline, Robo is expressed at high levels on their growth cones only after they cross the midline. Transgenic rescue experiments reveal that Robo can function in a cell-autonomous fashion. Robo appears to function as the gatekeeper controlling midline crossing.

Amino Acid Sequence↗

Seven deaths in Darwin: case studies under the Rights of the Terminally Ill Act, Northern Territory, Australia.

BACKGROUND: During the 9 months between July, 1996, and March, 1997, the provision of euthanasia for the terminally ill was legal in the Northern Territory of Australia. Seven patients made formal use of the Rights of the Terminally Ill (ROTI) Act; four died under the Act. We report their clinical details and the decision-making process required by the Act. METHODS: We taped in-depth interviews with the general practitioner who provided euthanasia. Further information was available from public texts created by patients, the media, and the coroner. FINDINGS: All seven patients had cancer, most at advanced stages. Three were socially isolated. Symptoms of depression were common. Having met criteria of the Act, some patients deferred their decision for a time before proceeding with euthanasia. Medical opinions about the terminal nature of illness differed. INTERPRETATION: Provision of opinions about the terminal nature of illness and the mental health of the patient, as required by the ROTI Act, created problematic gatekeeping roles for the doctors involved.

Aged↗

Ethical and legal implications of managed care.

This article addresses several ethical, regulatory, and legal issues in managed care with attention to recent court cases that focus on physicians' responsibility, fiduciary duty, and the impact that these legal decisions have on physicians practicing in a managed care environment. Discussion of the impact of changes in the control of decision-making processes for physicians, the use of managed care protocols, restriction of resources, and gatekeeping systems are addressed as are the specific duties and obligations of physicians to their patients.

Cost Control↗

Mucosal mast cells and the allergic response against nematode parasites.

IgE-mediated Type-I allergic reactions at nematode-infected mucosal surfaces are considered to have a direct protective function. The contribution of mucosal mast cells (MMC) to these mucosal allergic responses is reviewed. In addition to the T helper 2 cell-mediated regulation of MMC hyperplasia during nematode infection the kit ligand, stem cell factor (SCF), plays a key role in the early development of the MMC response. Studies in the mouse suggest that MMC protect against certain nematodes which enter the mucosa but not against lumen dwelling nematodes. The protective roles of MMC in other species, including sheep, are less certain and there is some evidence that MMC might enhance parasite fecundity. The measurement of MMC-specific granule chymases released systemically, and into the gut lumen, permits precise monitoring of mast cell activation and suggests that the secreted chymases may target epithelial junctional complex proteins, thereby causing increased mucosal permeability. The abundant intraepithelial MMC found in parasitised mucosa may, therefore, serve as epithelial gatekeepers permitting the translocation of plasma proteins onto the mucosal surface.

Animals↗

The role of SHIP in mast cell degranulation and IgE-induced mast cell survival.

Atopic disorders are on the increase in the Western world and are due, at least in part, to an overactive mast cell response. A better understanding of the intracellular signalling pathways that regulate both mast cell degranulation and the secretion of arachidonic acid metabolites and inflammatory cytokines could help in the treatment of these disorders. The src homology 2-containing inositol-polyphosphate 5'-phosphatase, SHIP, has been shown to be a key 'gatekeeper' of mast cell degranulation. SHIP prevents degranulation from occuring when IgE alone binds to the high-affinity receptor for IgE (FcvarepsilonR1), SHIP restrains it when IgE-bound FcvarepsilonR1 are engaged by multivalent allergens, and SHIP inhibits it when an IgG against the same allergen co-clusters the inhibitory low-affinity receptor for IgG (FcgammaRIIB) with the IgE receptor. SHIP acts as a negative regulator of degranulation by hydrolyzing phosphatidylinositol-3,4,5-trisphosphate, a second messenger generated in activated cells by phosphatidylinositol 3-kinase. Our finding that binding of only IgE to the FcvarepsilonR1 of SHIP-deficient mast cells results in massive degranulation, led us to investigate the signalling pathways that are triggered in normal murine bone marrow-derived mast cells by monomeric IgE. We report here that monomeric IgE activates signalling pathways resulting in mast cell survival, without stimulating degranulation or proliferation. These studies demonstrate that mast cell sensitization by IgE is an active rather than a passive process.

Animals↗

Primary care reform: a three country comparison of 'budget holding'.

Governments in most developed nations have been looking to organisational and financial reform of health systems over the last decade. Although the structure and problems of the health care sector in each country may differ, with countries correspondingly adopting different reform agendas, there has been some element of commonality in reforms: that of (managed) competition. Of particular importance in such reforms has been the strengthening of primary care. General practitioners and primary care physicians, as 'gatekeepers' to the health system, are increasingly being called upon to be accountable; not only for their patients' health but also for the wider resource implications of any treatments prescribed. In some countries this role has been formalised through establishing 'budget holding' for general practitioners and primary care physicians, for example, through general practice 'fund holding' in the UK, Health Maintenance Organisations in the USA, and Independent Practice Associations in New Zealand. This paper examines: (i) what such budget holding seeks to achieve; (ii) the effectiveness of the budget holding experience to date in achieving these objectives; and (iii) factors which appear to determine the success of budget holding in achieving its objectives.

Budgets↗

General practice care and patients' priorities in Europe: an international comparison.

Insight into patients' priorities with respect to health care should complement the views of professionals and policy makers on what is thought to be appropriate health care. To determine the strengths and weaknesses of general practice care from patients' perspectives written surveys were performed among patients in Denmark, Germany, Israel, Netherlands, Norway, Portugal, Sweden and United Kingdom (n = 3540). The potential quality problems identified were spread over the different countries: the low involvement of general practitioners in out-of-hours services in Portugal; the low provision of routine screening in Sweden, Norway and The Netherlands; the lack of a defined patient population in Germany; the lack of a formal gatekeeper role to secondary care in general practice in Germany and Sweden; and the low number of home visits in Sweden.

Europe↗