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Ugeskrift For Laeger Jan 2024This is a case report of two men aged 39 and 43 years with dissection of the coeliac trunk involving the splenic arteries causing splenic infarction. One case was...
This is a case report of two men aged 39 and 43 years with dissection of the coeliac trunk involving the splenic arteries causing splenic infarction. One case was associated with an increase in abdominal pressure during defaecation and the other occurred during treatment with methylphenidate. Based on the published 43 cases, risk factors include male sex, increased intraabdominal pressure or increased vascular pressure. Methylphenidate most likely increased the blood pressure, and dissections of other arteries have been described during treatment with this and the similar drug amphetamine.
Topics: Humans; Male; Amphetamine; Blood Pressure; Celiac Artery; Methylphenidate; Splenic Artery; Adult
PubMed: 38235724
DOI: 10.61409/V07230468 -
American Journal of Hypertension Oct 2023Fibromuscular dysplasia (FMD) is an idiopathic and systemic non-inflammatory and non-atherosclerotic arterial disease. Fifteen to 25% of patients with FMD present with...
Fibromuscular dysplasia (FMD) is an idiopathic and systemic non-inflammatory and non-atherosclerotic arterial disease. Fifteen to 25% of patients with FMD present with arterial dissection in at least one arterial bed. Conversely, a substantial number of patients with renal, carotid, and visceral dissection have underlying FMD. Also, while few patients with FMD develop coronary artery dissection, lesions suggestive of multifocal FMD have been reported in 30-80% of patients with spontaneous coronary artery dissection (SCAD), and the relation between these two entities remains controversial. The frequent association of FMD with arterial dissection, both in coronary and extra-coronary arteries raises a number of practical and theoretical questions: (i) Are FMD and arterial dissections two different facets of the same disease or distinct though related entities? (ii) Is SCAD just a manifestation of coronary FMD or a different disease? (iii) What is the risk and which are predictive factors of developing arterial dissection in a patient with FMD? (iv) What proportion of patients who experienced an arterial dissection have underlying FMD, and does this finding influence the risk of subsequent arterial complications? In this review we will address these different questions using fragmentary, mostly cross-sectional evidence derived from large registries and studies from Europe and the United States, as well as arguments derived from demographics, clinical presentation, imaging, and when available histology and genetics. From there we will derive practical consequences for nosology, screening and follow-up.
PubMed: 37379454
DOI: 10.1093/ajh/hpad056 -
European Review For Medical and... Sep 2023Marfan syndrome (MFS) is a systemic connective tissue disease that commonly and most severely affects the ocular, skeletal, and cardiovascular systems. The aim of the... (Review)
Review
Marfan syndrome (MFS) is a systemic connective tissue disease that commonly and most severely affects the ocular, skeletal, and cardiovascular systems. The aim of the manuscript is to review the aortic involvement and complications in MFS, including aortal dissection, thoracic aortic aneurysm, abdominal aortic aneurysm, and acute aortic syndrome. Dissecting thoracic aortic aneurysm and progressing aortic root enlargement are the major causes of MFS morbidity and mortality. Guidelines on aortic disease endorsed by the American College of Cardiology, and the American Heart Association recommend the measurement of the external and internal aortic diameters perpendicular to the axis of blood flow when Computed Tomography, or Magnetic Resonance Imaging, or Cardiac Echography are performed. The pathophysiology, diagnosis, prevention, and medical and surgical treatments of MFS associated with aortic complications are reported in this narrative review. Development and strengthening of centers specialized in cardiovascular diseases and MFS, together with an improvement in the knowledge of its pathogenesis through genetics and proteomics investigations, can ameliorate the prognosis of this disease.
Topics: United States; Humans; Marfan Syndrome; Aorta; Aortic Dissection; Aortic Aneurysm, Abdominal; Acute Aortic Syndrome; Aortic Aneurysm, Thoracic
PubMed: 37750650
DOI: 10.26355/eurrev_202309_33582 -
Hepatology Communications Sep 2023Primary liver cancers (PLCs) are a broad class of malignancies that include HCC, intrahepatic cholangiocarcinoma, and combined hepatocellular and intrahepatic... (Review)
Review
Primary liver cancers (PLCs) are a broad class of malignancies that include HCC, intrahepatic cholangiocarcinoma, and combined hepatocellular and intrahepatic cholangiocarcinoma. PLCs are often associated with a poor prognosis due to their high relapse and low therapeutic response rates. Importantly, PLCs exist within a dynamic and complex tumor ecosystem, which includes malignant, immune, and stromal cells. It is critical to dissect the PLC tumor ecosystem to uncover the underlying mechanisms associated with tumorigenesis, relapse, and treatment resistance to facilitate the discovery of novel therapeutic targets. Single-cell and spatial multi-omics sequencing techniques offer an unprecedented opportunity to elucidate spatiotemporal interactions among heterogeneous cell types within the complex tumor ecosystem. In this review, we describe the latest advances in single-cell and spatial technologies and review their applications with respect to dissecting liver cancer tumor ecosystems.
Topics: Humans; Liver Neoplasms; Ecosystem; Carcinoma, Hepatocellular; Cholangiocarcinoma; Bile Duct Neoplasms; Bile Ducts, Intrahepatic
PubMed: 37639704
DOI: 10.1097/HC9.0000000000000248 -
Medicine Dec 2023Connective tissue disorders, including Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS), are characterized by genetic mutations affecting connective tissue... (Review)
Review
Connective tissue disorders, including Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS), are characterized by genetic mutations affecting connective tissue structural integrity. These disorders significantly elevate the risk of aortic dissection, a life-threatening condition. This comprehensive review delves into the intricate interplay between connective tissue disorders and aortic dissection, shedding light on the clinical features, pathophysiology, genetic underpinnings, diagnostic approaches, clinical management, associated comorbidities, and prognosis, mainly focusing on MS and EDS, while also exploring rare connective tissue disorders and forms of cutis laxa contributing to aortic pathology.
Topics: Humans; Connective Tissue Diseases; Marfan Syndrome; Ehlers-Danlos Syndrome; Aortic Aneurysm; Aortic Dissection; Connective Tissue
PubMed: 38050214
DOI: 10.1097/MD.0000000000036499 -
Journal of the Intensive Care Society Nov 2023Aortic dissections are associated with significant mortality and morbidity, with rapid treatment paramount. They are caused by a tear in the intimal lining of the aorta... (Review)
Review
Aortic dissections are associated with significant mortality and morbidity, with rapid treatment paramount. They are caused by a tear in the intimal lining of the aorta that extends into the media of the wall. Blood flow through this tear leads to the formation of a false passage bordered by the inner and outer layers of the media. Their diagnosis is challenging, with most deaths caused by aortic dissection diagnosed at post-mortem. Aortic dissections are classified by location and chronicity, with management strategies depending on the nature of the dissection. The Stanford method splits aortic dissections into type A and B, with type A dissections involving the ascending aorta. De Bakey classifies dissections into I, II or III depending on their origin and involvement and degree of extension. The key to diagnosis is early suspicion, appropriate imaging and rapid initiation of treatment. Treatment focuses on initial resuscitation, transfer (if possible and required) to a suitable specialist centre, strict blood pressure and heart rate control and potentially surgical intervention depending on the type and complexity of the dissection. Effective post-operative care is extremely important, with awareness of potential post-operative complications and a multi-disciplinary rehabilitation approach required. In this review article we will discuss the aetiology and classifications of aortic dissection, their diagnosis and treatment principles relevant to critical care. Critical care clinicians play a key part in all these steps, from diagnosis through to post-operative care, and thus a thorough understanding is vital.
PubMed: 37841293
DOI: 10.1177/17511437231162219 -
Nature Communications Dec 2023G protein-coupled receptors (GPCRs) mediate responses to various extracellular and intracellular cues. However, the large number of GPCR genes and their substantial...
G protein-coupled receptors (GPCRs) mediate responses to various extracellular and intracellular cues. However, the large number of GPCR genes and their substantial functional redundancy make it challenging to systematically dissect GPCR functions in vivo. Here, we employ a CRISPR/Cas9-based approach, disrupting 1654 GPCR-encoding genes in 284 strains and mutating 152 neuropeptide-encoding genes in 38 strains in C. elegans. These two mutant libraries enable effective deorphanization of chemoreceptors, and characterization of receptors for neuropeptides in various cellular processes. Mutating a set of closely related GPCRs in a single strain permits the assignment of functions to GPCRs with functional redundancy. Our analyses identify a neuropeptide that interacts with three receptors in hypoxia-evoked locomotory responses, unveil a collection of regulators in pathogen-induced immune responses, and define receptors for the volatile food-related odorants. These results establish our GPCR and neuropeptide mutant libraries as valuable resources for the C. elegans community to expedite studies of GPCR signaling in multiple contexts.
Topics: Animals; Caenorhabditis elegans; Receptors, G-Protein-Coupled; Neuropeptides; Chemoreceptor Cells; Phylogeny
PubMed: 38110404
DOI: 10.1038/s41467-023-44177-z