Annals of medicine
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Review
Refractory systemic onset juvenile idiopathic arthritis: current challenges and future perspectives.
Systemic juvenile idiopathic arthritis (SJIA) is a rare disease with distinct features not seen in other categories of juvenile idiopathic arthritis. In recent years, advances in the understanding of disease immunopathogenesis have led to improved targeted therapies with significant improvement in patient outcomes. Despite these advances, there remain subsets of SJIA with refractory disease and severe disease-associated complications. ⋯ There is no current agreed upon definition for refractory SJIA and we propose in this review that refractory SJIA is presence of active systemic or arthritic features despite treatment with anti-IL-1 or anti-IL-6 therapy or disease requiring glucocorticoids for control beyond 6 months. SJIA disease associated complications include presence of associated macrophage activation syndrome (MAS), interstitial lung disease (ILD) or amyloidosis and management of each differs. Refractory SJIA treatment options currently include additional conventional synthetic disease modifying anti-rheumatic drugs (csDMARDS), biologic (bDMARDS), combination biologic therapy, targeted synthetic (tsDMARDS) or other immunomodulatory therapies.
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Psychotropic medications are commonly prescribed among adults with intellectual disability, often in the absence of a psychiatric diagnosis. The aim of this scoping review is to provide an overview of the extent, range, and nature of the available research on medication use and practices and medication management in people with intellectual disability taking psychotropic medications for behaviours that challenge. ⋯ These findings can inform prescribing interventions and highlight the need for timely and comprehensive patient outcome data, especially on long-term use of high doses of psychotropics and what happens when reduce or stop prescribing these doses.KEY MESSAGESPsychotropic medications are frequently prescribed for people with intellectual disabilities, often at high doses and these medications are associated with both positive and negative patient outcomes.Work to rationalize psychotropic use has been reported with interventions aiming to reduce polypharmacy or deprescribe a single psychotropic medicine. These interventions had mixed success and risk of relapse was documented in some studies.Limitations in sample size and heterogenous patient cohorts make it challenging to understand the risks and benefits associated with reducing or stopping psychotropic medicines.Patient, carer, and clinician partnerships are critical to advance medication management.
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To assess in vivo confocal microscopy features of corneal sub-basal nerve plexus in patients with congenital or aponeurogenic blepharoptosis using a fully automated software (ACCMetrics). ⋯ These data suggested that corneal confocal microscopy demonstrated no significant changes in patients with blepharoptosis as compared with normal controls, except for relatively wider CNFW in congenital affected eyes. However, in some children and young adults with congenital blepharoptosis, the density of corneal sub-basal nerve plexus was evidently decreased, which needs to be cautioned when ones with congenital blepharoptosis want to take corneal surgeries or wear contact lens.Key messagesWhen compared with normal controls, no significant effect was found in the influence of blepharoptosis on the most of corneal nerve parameters, except for corneal nerve fibre width (CNFW) in the group of congenital blepharoptosis.The age of onset of blepharoptosis may influence corneal nerve fibres, so timely surgical treatment of congenital blepharoptosis is not only conducive to the development of normal vision, but also beneficial to the reduction of corneal nerve lesions to some extent.We noted that some young blepharoptosis patients revealed sparse corneal nerve, which should be taken precaution when ones with congenital blepharoptosis who want to take corneal surgeries or wear contact lens.
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Percutaneous catheter drainage (PCD) has been viewed as first-line treatment for pyogenic liver abscess (PLA), yet detailed guidance is lacking for best practice of PCD. This study investigated characteristics of patients with PLA who had received PCD, identified factors associated with prolonged fever, and aimed to evaluate the relationship between timing of PCD and clinical improvement. ⋯ Large abscesses with loculation could be successfully treated with PCD, and early PCD protected patients with PLA from prolonged fever. Our findings suggest that early intervention should be provided if PCD is indicated in clinical practice.KEY MESSAGESLarge abscesses and multiloculated abscesses can be treated with percutaneous catheter drainage.Early percutaneous catheter drainage is identified as a protective factor of prolonged fever among patients with pyogenic liver abscesses.Early intervention should be provided if percutaneous catheter drainage is indicated for pyogenic liver abscesses.
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Observational Study
Clinical implications of cardiac troponin-I in patients with hypertensive crisis visiting the emergency department.
Cardiac troponin-I (cTnI) is a representative marker of myocardial injury. Elevation of cTnI is frequently observed in patients with hypertensive crisis, but few studies have examined its prognostic significance in hypertensive crisis. We aimed to determine whether cTnI could predict all-cause mortality in patients with hypertensive crisis visiting the emergency department (ED). ⋯ In patients with hypertensive crisis, elevated cTnI levels provide useful prognostic information and permit the early identification of patients with an increased risk of death. Moreover, putatively normal but detectable cTnI levels also significantly correlated with a higher risk of all-cause mortality. Intensive treatment and follow-up strategies are needed for patients with hypertensive crisis with elevated and detectable cTnI levels.Key messagesCardiac troponin-I level was an independent prognostic factor for all-cause mortality in patients with hypertensive crisis.Detectable but normal range cardiac troponin-I, which was considered clinically insignificant, also had a prognostic impact on all-cause mortality comparable to elevated cardiac troponin-I levels.