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Anaesthesiology Intensive Therapy 2019Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are increasingly recognized as aetiologies of organ failure and mortality among a wide...
BACKGROUND
Intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) are increasingly recognized as aetiologies of organ failure and mortality among a wide variety of patient populations. Since the first global survey in 2007, several surveys have been conducted. However, it remains unclear to what extent healthcare professionals in clinical practice are aware of the widely accepted definitions and recommendations proposed in the World Society of the Abdominal Compartment Syndrome (WSACS) guidelines and whether these recommendations are being applied clinically.
METHODS
We conducted an international cross-sectional survey to determine the impact of the 2013 WSACS IAH/ACS Consensus Definitions and Clinical Management Guidelines on IAH/ACS clinical awareness and management. We also aimed to compare the results to the findings of the global survey conducted in 2007.
RESULTS
The survey had 559 respondents with most respondents being physicians from Europe, and who worked in mixed intensive care units (87.3%; n = 448). The majority of respondents (73.2%) were aware of WSACS (the Abdominal Compartment Society), with a greater percentage being aware of the WSACS guidelines compared to the 2007 survey (60.2% vs. 28.4%). A considerable proportion of respondents (18%) never measure intra-abdominal pressure (IAP), with the most common reason for not measuring IAP being reliance on physical examination (39%; n = 38). Analysis of the 11 questions related to knowledge and clinical practice of IAH, ACS and WSACS consensus definitions showed an improvement from the first survey (42.7% of questions answered correctly in comparison to 48.0% in this survey, P = 0.0001). The responses to how physicians managed IAH and ACS were different to the previous survey, with diuretics being used "usually" or "frequently" (49.2%), more than inotropes (38.6%), decompressive laparotomy (37.0%), paracentesis (36.5%), and fluids/blood products (24.2%). Most respondents would perform/request a decompressive laparotomy in cases of ACS. Polycompartment syndrome was something considered by 39% (n = 218) in their daily practice. Almost two thirds of respondents (63.5%; n = 355) thought that gastrointestinal injury should be added to the Sequential Organ Failure Assessment (SOFA) score.
CONCLUSIONS
This survey shows an improvement in general awareness and knowledge regarding IAP, IAH and ACS, although the level of understanding and awareness of WSACS guidelines remains low. There appear to be some practice changes and greater awareness of the need to monitor abdominal pressures. Future initiatives should focus on education, identifying which patients should receive routine monitoring, and incorporation of IAH and ACS care into ICU bundles and protocols already in existence.
Topics: Cross-Sectional Studies; Health Knowledge, Attitudes, Practice; Humans; Intra-Abdominal Hypertension; Physicians; Practice Guidelines as Topic; Practice Patterns, Physicians'; Surveys and Questionnaires
PubMed: 31493332
DOI: 10.5114/ait.2019.87648 -
Gland Surgery Feb 2020Among many recognized sequelae of primary hyperparathyroidism (PHPT), cardiovascular disease remains incompletely understood as a consequence of disordered calcium and... (Review)
Review
Among many recognized sequelae of primary hyperparathyroidism (PHPT), cardiovascular disease remains incompletely understood as a consequence of disordered calcium and parathyroid hormone (PTH) metabolism. While population studies have identified trends that associate PHPT with hypertension, metabolic syndrome, and vascular system calcifications, the fundamental pathophysiology, natural history, and opportunity to reverse or cure the cardiovascular effects with parathyroidectomy are not well established. This chapter reviews the current knowledge of this field of interest within PHPT and summarizes key findings from dedicated investigations that have addressed the impact of parathyroid surgery on the cardiovascular system.
PubMed: 32206605
DOI: 10.21037/gs.2019.12.12 -
Frontiers in Integrative Neuroscience 2020Rett syndrome is a debilitating neurodevelopmental disorder for which no disease-modifying treatment is available. Fortunately, advances in our understanding of the...
Rett syndrome is a debilitating neurodevelopmental disorder for which no disease-modifying treatment is available. Fortunately, advances in our understanding of the genetics and pathophysiology of Rett syndrome has led to the development of promising new therapeutics for the condition. Several of these therapeutics are currently being tested in clinical trials with others likely to progress to clinical trials in the coming years. The failure of recent clinical trials for Rett syndrome and other neurodevelopmental disorders has highlighted the need for electrophysiological or other objective biological markers of treatment response to support the success of clinical trials moving forward. The purpose of this review is to describe the existing studies of electroencephalography (EEG) and evoked potentials (EPs) in Rett syndrome and discuss the open questions that must be addressed before the field can adopt these measures as surrogate endpoints in clinical trials. In addition to summarizing the human work on Rett syndrome, we also describe relevant studies with animal models and the limited research that has been carried out on Rett-related disorders, particularly methyl-CpG binding protein 2 (MECP2) duplication syndrome, CDKL5 deficiency disorder, and FOXG1 disorder.
PubMed: 32547374
DOI: 10.3389/fnint.2020.00030 -
Seminars in Reproductive Medicine Jul 2011Ovarian autoimmunity is increasingly implicated in the etiology of primary ovarian insufficiency (POI), previously termed PREMATURE OVARIAN FAILURE or PREMATURE... (Review)
Review
Ovarian autoimmunity is increasingly implicated in the etiology of primary ovarian insufficiency (POI), previously termed PREMATURE OVARIAN FAILURE or PREMATURE MENOPAUSE. Links to autoimmunity in human POI have long been noted due to the close association of POI with several autoimmune diseases and syndromes such as Addison's disease and Autoimmune polyglandular syndrome 1. However, diagnosis of autoimmune-mediated POI (aPOI) remains challenging because of the lack of sensitive or specific markers of disease. Autoimmunity can arise from the breakdown of immunological tolerance in several ways. How then may we discern what constitutes a relevant target and what represents a downstream phenomenon? The answer lies in the study of pathogenic mechanisms in translational models of disease. From examples in humans and mice, we see that ovarian autoimmunity likely arises from a limited number of antigens targeted in the ovary that are organ specific. These antigens may be conserved but not limited to those seen in animal models of autoimmune ovarian disease. Recent advances in these areas have begun to define the relevant antigens and mechanisms of immune tolerance breakdown in the ovary. Work in translational models continues to provide insight into mechanisms of disease pathogenesis that will allow more accurate diagnosis and, ultimately, improved interventions for women with aPOI.
Topics: Animals; Autoantigens; Autoimmunity; Disease Models, Animal; Female; Humans; Immune Tolerance; Mice; Mice, Inbred C57BL; Mice, Knockout; Ovary; Primary Ovarian Insufficiency
PubMed: 21969265
DOI: 10.1055/s-0031-1280916 -
Fertility and Sterility Mar 2007To update clinicians on the reproductive implications of premutations in FMR1 (fragile X mental retardation 1). Fragile X syndrome, a cause of mental retardation and... (Review)
Review
OBJECTIVE
To update clinicians on the reproductive implications of premutations in FMR1 (fragile X mental retardation 1). Fragile X syndrome, a cause of mental retardation and autism, is due to a full mutation (>200 CGG repeats). Initially, individuals who carried the premutation (defined as more than 55 but less than 200 CGG repeats) were not considered at risk for any clinical disorders. It is now recognized that this was incorrect, specifically with respect to female reproduction.
DESIGN AND SETTING
Literature review and consensus building at two multidisciplinary scientific workshops.
CONCLUSION(S)
Convincing evidence now relates the FMR1 premutation to altered ovarian function and loss of fertility. An FMR1 mRNA gain-of-function toxicity may underlie this altered ovarian function. There are major gaps in knowledge regarding the natural history of the altered ovarian function in women who carry the FMR1 premutation, making counseling about reproductive plans a challenge. Women with premature ovarian failure are at increased risk of having an FMR1 premutation and should be informed of the availability of fragile X testing. Specialists in reproductive medicine can provide a supportive environment in which to explain the implications of FMR1 premutation testing, facilitate access to testing, and make appropriate referral to genetic counselors.
Topics: Adolescent; Adult; Female; Fragile X Mental Retardation Protein; Fragile X Syndrome; Genetic Counseling; Genetic Testing; Humans; Male; Mutation; Pregnancy; Preimplantation Diagnosis; Primary Ovarian Insufficiency; Trinucleotide Repeat Expansion
PubMed: 17074338
DOI: 10.1016/j.fertnstert.2006.09.004 -
Pituitary Oct 2021Bilateral adrenalectomy (BA) still plays an important role in the management of Cushing's disease (CD). Nelson's syndrome (NS) is a severe complication of BA, but... (Meta-Analysis)
Meta-Analysis
PURPOSE
Bilateral adrenalectomy (BA) still plays an important role in the management of Cushing's disease (CD). Nelson's syndrome (NS) is a severe complication of BA, but conflicting data on its prevalence and predicting factors have been reported. The aim of this study was to determine the prevalence of NS, and identify factors associated with its development.
DATA SOURCES
Systematic literature search in four databases.
STUDY SELECTION
Observational studies reporting the prevalence of NS after BA in adult patients with CD.
DATA EXTRACTION
Data extraction and risk of bias assessment were performed by three independent investigators.
DATA SYNTHESIS
Thirty-six studies, with a total of 1316 CD patients treated with BA, were included for the primary outcome. Pooled prevalence of NS was 26% (95% CI 22-31%), with moderate to high heterogeneity (I 67%, P < 0.01). The time from BA to NS varied from 2 months to 39 years. The prevalence of NS in the most recently published studies, where magnet resonance imaging was used, was 38% (95% CI 27-50%). The prevalence of treatment for NS was 21% (95% CI 18-26%). Relative risk for NS was not significantly affected by prior pituitary radiotherapy [0.9 (95% CI 0.5-1.6)] or pituitary surgery [0.6 (95% CI 0.4-1.0)].
CONCLUSIONS
Every fourth patient with CD treated with BA develops NS, and every fifth patient requires pituitary-specific treatment. The risk of NS may persist for up to four decades after BA. Life-long follow-up is essential for early detection and adequate treatment of NS.
Topics: Adrenalectomy; Adult; Humans; Nelson Syndrome; Pituitary ACTH Hypersecretion; Pituitary Gland; Prevalence
PubMed: 34036460
DOI: 10.1007/s11102-021-01158-z -
American Journal of Epidemiology Feb 2023Recombinant zoster vaccine (RZV) (Shingrix; GlaxoSmithKline, Brentford, United Kingdom) is an adjuvanted glycoprotein vaccine that was licensed in 2017 to prevent herpes...
Recombinant zoster vaccine (RZV) (Shingrix; GlaxoSmithKline, Brentford, United Kingdom) is an adjuvanted glycoprotein vaccine that was licensed in 2017 to prevent herpes zoster (shingles) and its complications in older adults. In this prospective, postlicensure Vaccine Safety Datalink study using electronic health records, we sequentially monitored a real-world population of adults aged ≥50 years who received care in multiple US Vaccine Safety Datalink health systems to identify potentially increased risks of 10 prespecified health outcomes, including stroke, anaphylaxis, and Guillain-Barré syndrome (GBS). Among 647,833 RZV doses administered from January 2018 through December 2019, we did not detect a sustained increased risk of any monitored outcome for RZV recipients relative to either historical (2013-2017) recipients of zoster vaccine live, a live attenuated virus vaccine (Zostavax; Merck & Co., Inc., Kenilworth, New Jersey), or contemporary non-RZV vaccine recipients who had an annual well-person visit during the 2018-2019 study period. We confirmed prelicensure trial findings of increased risks of systemic and local reactions following RZV. Our study provides additional reassurance about the overall safety of RZV. Despite a large sample, uncertainty remains regarding potential associations with GBS due to the limited number of confirmed GBS cases that were observed.
Topics: Humans; Aged; Herpes Zoster Vaccine; Electronic Health Records; Prospective Studies; Herpes Zoster; Herpesvirus 3, Human; Vaccines, Attenuated
PubMed: 36193854
DOI: 10.1093/aje/kwac170 -
The Journal of Clinical Endocrinology... Dec 2022
Topics: Humans; COVID-19; SARS-CoV-2; Obesity; Causality; Systemic Inflammatory Response Syndrome
PubMed: 36268884
DOI: 10.1210/clinem/dgac619 -
Endokrynologia Polska 2015Nelson's syndrome (NS) is a rare clinical syndrome caused by an enlarging, aggressive corticotroph pituitary adenoma that can occur following bilateral adrenalectomy...
INTRODUCTION
Nelson's syndrome (NS) is a rare clinical syndrome caused by an enlarging, aggressive corticotroph pituitary adenoma that can occur following bilateral adrenalectomy performed in the treatment of refractory Cushing's disease (CD). Such tumours respond poorly to currently available therapeutic options, which include surgery, radiotherapy, pharmacotherapy, and chemotherapy. They are a challenging problem in neurosurgical practice. The aim of this work was to evaluate the early and long-term results of microsurgery in a single surgeon's series of patients with NS.
MATERIAL AND METHODS
During the period from January 2000 to December 2005, 10 patients with NS underwent surgery. The authors analysed surgical outcomes in the NS group of seven women and three men with the mean age of 47.99 years (range 39-66, SD ± 8.47 years). NS was diagnosed based on clinical signs and symptoms, especially hyperpigmentation of the skin, elevated serum ACTH levels, and pituitary tumour growth. Parasellar extension of the adenomas was assessed in both groups according to Knosp's and Hardy-Wilson classifications. Pituitary function and radiographs were evaluated in the early postoperative period, 30 days after the operation, and during follow-up. Histological examination was based on the WHO (2004) criteria.
RESULTS
According to the criteria for Nelson's syndrome remission, five patients (50%) were cured. No perioperative mortality was reported. Three patients developed pituitary insufficiency and two patients developed diabetes insipidus. There was one case of postoperative cerebrospinal fluid leakage. One patient was diagnosed with pituitary carcinoma.
CONCLUSIONS
Transsphenoidal microsurgical removal of pituitary adenomas is a safe and effective treatment of Nelson' syndrome.
Topics: Adult; Aged; Endocrine Surgical Procedures; Female; Humans; Hypopituitarism; Male; Middle Aged; Nelson Syndrome; Pituitary Gland; Pituitary Neoplasms; Treatment Outcome
PubMed: 26662649
DOI: 10.5603/EP.2015.0062 -
Emergency Medicine Clinics of North... Nov 2000An extensive range of herbal and dietary supplements is now available, and use of these products by ED patients is fairly common. Emergency physicians should be familiar... (Review)
Review
An extensive range of herbal and dietary supplements is now available, and use of these products by ED patients is fairly common. Emergency physicians should be familiar with some of the products used more frequently for common complaints. Emergency personnel also should be vigilant for toxic syndromes resulting from ingestion of certain of these products and be wary of possible toxicity from any of these supplements owing to their minimal quality control and absence of FDA regulation.
Topics: Antioxidants; Cholinergic Antagonists; Complementary Therapies; Dietary Supplements; Drug Contamination; Emergencies; Ephedrine; Heart; Humans; Liver; Phytotherapy; Psychotropic Drugs; Sympathomimetics; Syndrome; Weight Loss
PubMed: 11130934
DOI: 10.1016/s0733-8627(05)70154-1