The American journal of the medical sciences
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Treatments of pulmonary hypertension (PH) continue to evolve with approval of new therapies. The currently FDA approved inhaled PH therapies include inhaled iloprost for group 1 pulmonary arterial hypertension (PAH), inhaled treprostinil solution and treprostinil dry powder inhaler for both group 1 PAH and group 3 PH associated with interstitial lung disease (PH-ILD). ⋯ The pipeline for inhaled PH therapies includes several promising molecules that can enrich the current PH therapeutic era and mitigate several systemic side effects by directly delivering the drug to the target organ. In this review article we summarize the evidence for the currently approved inhaled PAH/PH therapies, discuss the available inhalation devices, present a roadmap for successful treatment strategy, and present several inhaled PAH/PH therapies in the pipeline.
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Nuclear protein in testis (NUT) carcinoma is an extremely rare and undifferentiated malignancy characterized by the rearrangement of NUT gene (NUTM1, Nuclear Protein in Testis). NUT carcinoma is a challenging disease which is difficult to diagnose and treat. ⋯ Therefore, NUT carcinoma should be included in differential diagnosis of poorly differentiated/undifferentiated and rapidly progressive malignancy in children and young adults, occurring in the head, neck or thorax. We report a case of NUT carcinoma presented with pleural effusion in adulthood.
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Discharge against medical advice (DAMA) represents an increasingly burdensome public health issue that leads to worse outcomes for patients and high costs to society. While the rate of patients who DAMA is higher within certain institutions and geographic locations, the problem is present across all healthcare systems. DAMAs are often challenging as they occur suddenly and can be unsatisfactory. ⋯ In this review, we discuss the broader impact, associated factors, the most common reasons, the consequences, and the prevention strategies for DAMA. Further research is needed to create tools for stratifying patients most likely to DAMA. Early identification and appropriate interventions for these patients will allow for safe discharges.
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Apical hypertrophic cardiomyopathy (ApHCM) is thought to be an uncommon variant of hypertrophic cardiomyopathy (HCM). This article is a literature review focusing on the characteristic electrocardiogram (EKG) and 2D echocardiogram findings as currently there are no specific ACC/AHA/ESC guidelines set as diagnostic criteria for ApHCM.
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Patients with persistent fevers of undetermined etiology often undergo extensive evaluation without a diagnosis. Autoinflammatory syndromes may not always be considered in the differential, as these are rare entities, there are no consensus clinical criteria and genetic testing can only capture a few of these diseases. We aimed to describe the experience and value of an undiagnosed diseases program in the evaluation and management of patients who present with persistent fevers. ⋯ This is a pilot study suggesting the role for empiric treatment trials of biologics for patients with suspected autoinflammatory diseases. As the differential diagnosis of patients with persistent fevers is broad, and the diagnosis of autoinflammatory diseases often comes with some degree of uncertainty, evaluation by a center with expertise in diagnosing these conditions can help determine which patients should have empiric trials of biologics.