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Since the original description of adrenal insufficiency by Thomas Addison in 1855, there has been an exponential growth in the understanding of adrenal gland biology and its role in the hypothalamic-pituitary-adrenal axis. Despite this, the mainstay of therapeutic glucocorticoid replacement for most clinicians has remained unchanged for nearly 50 years. More recently, there has been better recognition of the morbidity and mortality associated with current approaches and the challenges to tackle in reducing this and improving clinical outcomes. In this review, we have summarised the history of glucocorticoid replacement therapy from its nascence in the 1930s, through common practice and culminating in more recent glucocorticoid replacement strategies plus the potential of stem cell therapy in the future.

Local governments are in an ideal position to integrate government, private, and citizen data to deliver beneficial new digitally enabled public services. However, progress toward achieving the benefits has stagnated in many cases. This is because managers lack the requisite know-how to lead the implementation and enactment of integrated enterprise systems to improve the processes of public service delivery, part of a journey called ‘digital transformation’. If digital transformations are to progress, we need more nuanced empirical elaboration of the know-how managers require. To that end, this study reports evidence from case studies in 11 local governments in Canada. The findings provide the empirical basis for a theory of the know-how managers require to lead the implementation and enactment of integrated enterprise systems in support of digital transformation. We then propose a new theoretical model of how to transfer that requisite know-how to managers through public-private partnerships, and thereby mitigate barriers to that transfer.

Patients affected by gastroenteropancreatic–neuroendocrine tumors (GEP–NETs) have an increased risk of developing osteopenia and osteoporosis, as several factors impact on bone metabolism in these patients. In fact, besides the direct effect of bone metastasis, bone health can be affected by hormone hypersecretion (including serotonin, cortisol, and parathyroid hormone-related protein), specific microRNAs, nutritional status (which in turn could be affected by medical and surgical treatments), and vitamin D deficiency. In patients with multiple endocrine neoplasia type 1 (MEN1), a hereditary syndrome associated with NET occurrence, bone damage may carry other consequences. Osteoporosis may negatively impact on the quality of life of these patients and can increment the cost of medical care since these patients usually live with their disease for a long time. However, recommendations suggesting screening to assess bone health in GEP–NET patients are missing. The aim of this review is to critically analyze evidence on the mechanisms that could have a potential impact on bone health in patients affected by GEP–NET, focusing on vitamin D and its role in GEP–NET, as well as on factors associated with MEN1 that could have an impact on bone homeostasis.

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence worldwide of 0.7–2.0 cases/million/year. Initial staging is the most important factor in determining prognosis. If diagnosed early, complete surgical resection +/− adjuvant treatment can lead to 5-year survival of up to 80%. However, often it is diagnosed late and in advanced disease, 5-year survival is

Background Hajdu-Cheney syndrome (HCS) (#OMIM 102500) is a rare, autosomal dominant condition that presents in early childhood. It is caused by mutations in the terminal exon of NOTCH2, which encodes the transmembrane NOTCH2 receptor. This pathway is involved in the coupled processes of bone formation and resorption. The skeletal features of HCS include acro-osteolysis of the digits and osteoporosis commonly affecting vertebrae and long bones. Fractures are a prominent feature and are associated with significant morbidity. There is no specific treatment, but with both acro-osteolysis and generalized osteoporosis, it is possible that anti-resorptive treatment might be of benefit. However, to date only a few case reports have evaluated the effectiveness of bisphosphonate treatment. Methods We describe the clinical features, treatment regimens and response to bisphosphonate treatment in 7 newly described patients aged 6–39 with HCS, and pooled the data with that from 8 previously published cases (a total of 17 courses of treatment in 15 individuals). Results The mean lumbar spine bone mineral density (BMD) z-score before treatment was − 2.9 (SD 1.2). In 14 courses of treatment (82%), there was an increase in BMD with bisphosphonate treatment, but the impact (in terms of change in spinal BMD z-score) appeared to be less with advancing age (p = 0.01). There was no evidence that acro-osteolysis was prevented. Conclusions Although individual response is variable and age-related, the data support a role for bisphosphonates in preventing or treating spinal osteoporosis in HCS, but bone loss from the lumbar spine may be rapid after cessation.

We present a 66 year old gentleman with constrictive pericarditis and persistent atrial flutter. Initial management with oral loop diuretics was successful until he developed persistent atrial flutter. Once in atrial flutter the patient developed progressive signs of right heart failure resistant to high dose intravenous loop diuretics. He was referred to a tertiary electrophysiology service where he underwent successful isthmus catheter ablation and reverted to sinus rhythm. His responsiveness to diuretics improved immediately. His symptoms improved and he was discharged 48 h later on oral diuretics. He remains well one month after discharge.This is the first reported case of symptomatic improvement in a patient with constrictive pericarditis and persistent atrial flutter with targeted treatment of the dysrhythmia. This offers a possible short-term palliation option in a group of patients where definitive surgical management carries too high a risk.

The effective sharing of organizational knowledge is particularly relevant for multinational corporations, where firm-specific tacit knowledge (know-how) is considered a source of competitive advantage for subsidiaries participating in a global strategy. To that end, multinational corporations (MNCs) are asking their IT departments to support both the exploitation of existing knowledge and the unit-to-unit transfer of new know-how derived in units from exploration. Nonetheless, new know-how derived from exploratory research, development and experience in one unit can be difficult to transfer to units that can exploit that know-how to commercial ends. The factors that impede the transfer of new know-how have been conceptualized as ''factors of stickiness''. In this paper, we present a theoretical model of organizational factors that can cause (or conversely mitigate) stickiness in the flow of new know-how between MNC units. To test the six hypotheses of the model, we used meta-analytic structural equation modeling (MASEM) of 31 empirical studies, representing 10,432 cases of new know-how transfer between units. The result of MASEM shows that the factors of receiving units' potential absorptive capacity and transmission channel in form of social capital that is enacted through its three dimensions (i.e., embedded social ties between units, institutional shared vision of units, and interorganizational trust of units) affect recipient subsidiaries' capability to exploit new know-how in practice (i.e., realized absorptive capacity), thus effectuating its transfer. Based on our findings, we propose research directions within the context of agile information systems development, distributed software projects, and management of information systems functions in MNCs.

For more than a decade, healthcare reform has emphasized coordinated “patient-centered care”. To that end, policymakers have invested in integration of healthcare providers’ information flows. Research has studied healthcare providers’ information needs but overlooked communicative exchanges among participants in coordinating treatment plan decisions. Consequently, although medical literature asserts that patients should depend on information exchange with healthcare providers to enable participation in treatment plan decisions, the assertion has not been tested. In this paper, the authors conduct an empirical study to elucidate the structure of actors’ communications in support of their information dependencies. The findings illustrate that although patients are well connected through personal contact with healthcare providers, patients are disenfranchised from integrated healthcare information systems (IS) and the potential of IS to support patients’ participation in coordinated “patient-centered care” decisions. Furthermore, knowledge asymmetry between patients and healthcare providers should be considered in the selection and design of healthcare IS.

Medical interventions are often delayed or erroneous when information needed for diagnosing or prescribing is missing or unavailable. In support of increased information flows, the healthcare industry has invested substantially in standards intended to specify, routinize, and make uniform the type and format of medical information in clinical healthcare information systems such as Electronic Medical Record systems (EMRs). However, fewer than one in four Canadian physicians have adopted EMRs. Deeper analysis illustrates that physicians may perceive value in standardized EMRs when they need to exchange information in highly structured situations among like participants and like environments. However, standards present restrictive barriers to practitioners when they face equivocal situations, unforeseen contingencies, or exchange information across different environments. These barriers constitute a compelling explanation for at least part of the observed low EMR adoption rates. Our recommendations to improve the perceived value of standardized clinical information systems espouse re-conceptualizing the role of standards to embrace greater flexibility in some areas.