Notes

Spatially fractionated stereotactic system radiotherapy (Lattice) for big cancers.
As a result, survival is improving, and we are no longer faced with a dualistic choice between hospice or organ transplant. The future goal is to develop anti-fibril therapies that will be safe and effective at removing deposited amyloid fibrils and restoring organs to their pre-amyloid state. (S)-JQ-35 For the millions of carriers of variant ATTR, enhanced testing followed by genetic editing may allow a cure even before patients develop clinical signs of the disease.Cardiac amyloidosis is increasingly recognized as an underlying cause of left ventricular wall thickening, heart failure, and arrhythmia with variable clinical presentation. Due to the subtle cardiac findings in early transthyretin cardiac amyloidosis and the availability of therapies that can modify but not reverse the disease progression, early recognition is vital. In light chain amyloidosis, timely diagnosis and treatment can significantly improve survival. In this manuscript, we review the clinical, imaging, and electrocardiographic clues that should raise suspicion for cardiac amyloidosis and provide a simplified diagnostic workup algorithm that ensures an accurate diagnosis. The evolution of the noninvasive diagnosis of cardiac amyloidosis has significantly influenced our understanding of disease prevalence, presentations, and outcomes. However, clinical recognition of clues and red flags remains the most important factor in advancing the care of patients with cardiac amyloidosis.Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed disease and an underestimated cause of both heart failure and conduction abnormalities. It is characterized by pathologic accumulation of extracellular protein arising from unstable transthyretin (TTR) tetramers, which dissociate into monomers that misfold, aggregate, and form insoluble fibrils that are resistant to proteolysis. Cardiac amyloidosis appears in two distinct forms hereditary and wild-type. There is considerable heterogeneity in the clinical presentation of ATTR, ranging from primarily cardiac, primarily neuropathic, or mixed cardiac and neuropathic disease. Pathogenic variants in the TTR gene that predominantly involve the heart include Val122Ile, Leu111Met, and Ile68Leu. The wild-type form of ATTR is also predominantly cardiac. Phenotypic heterogeneity is linked to differences among specific pathogenic TTR variants, geography, and the subtype of endemic versus nonendemic disease. Factors contributing to wild-type ATTR are largely unknown, but similar factors likely influence the penetrance of hereditary ATTR. Recognition of ATTR-CM is improving due to the increased use of cardiac scintigraphy as a noninvasive diagnostic tool, and early recognition of cardiac infiltration is crucial to optimize long-term prognosis.Cardiac amyloidosis (CA) is the buildup and infiltration of amyloid plaque in cardiac muscle. An underdiagnosed form of restrictive cardiomyopathy, CA can rapidly progress into heart failure. CA is evaluated using a multimodality approach that includes echocardiography, cardiac magnetic imaging, and nuclear imaging. Echocardiography remains an essential first-line modality that raises suspicion for CA and establishes functional baselines. Cardiac magnetic imaging provides additional incremental value via high-resolution imaging, robust functional assessment, and superior tissue characterization, all of which enable a more comprehensive investigation of CA. Cardiac scintigraphy has eliminated the need for invasive diagnostic approaches and helps differentiate CA subtypes. Positron emission tomography is the first modality introducing targeted amyloid binding tracers that allow for precise burden quantification, early detection, and disease monitoring. In this review, we highlight the role of several cardiac imaging techniques in the evaluation of CA.Philip Alexander, MD, is a native Texan, retired physician, and accomplished musician and artist. After 41 years as an internal medicine physician, Dr. Phil retired from his practice in College Station in 2016. A lifelong musician and former music professor, he often performs as an oboe soloist for the Brazos Valley Symphony Orchestra. He began exploring visual art in 1980, evolving from pencil sketches-including an official White House portrait of President Ronald Reagan-to the computer-generated drawings featured in this journal. His images, which first appeared in this journal in the spring of 2012, are his own original creations. If you would like to see your art published in the Methodist DeBakey Cardiovascular Journal, submit your creation online at journal.houstonmethodist.org as a "Humanities" entry.Cardiac amyloidosis (CA) is a restrictive cardiomyopathy with a traditionally poor prognosis. Until recently, CA treatment options were limited and consisted predominantly of managing symptoms and disease-related complications. However, the last decade has seen significant advances in disease-modifying therapies, increased awareness of CA, and improved diagnostic methods resulting in earlier diagnoses. In this review, we provide an overview of current and experimental treatments for the predominant types of CA transthyretin cardiac amyloidosis (ATTR-CA) and immunoglobulin light chain (AL)-mediated CA (AL-CA). The mainstay of AL-CA treatment is proteasome inhibitor-based chemotherapy with daratumumab and, when feasible, autologous stem cell transplantation. For ATTR-CA, the stabilizer tafamidis is the only US Food and Drug Administration (FDA)-approved treatment. However, promising novel therapies on the horizon target various points in the ATTR-CA amyloidogenic cascade. These include transthyretin gene (TTR) silencing agents to prevent TTR formation, TTR tetramer stabilization and inhibition of oligomer aggregation to prevent fibril formation, anti-TTR fiber antibodies, and amyloid degradation. For end-stage CA, advanced interventions may need to be considered, including heart, heart-kidney, and, for hereditary ATTR-CA, heart-liver transplantation. Despite the evolution of treatment options, CA management remains complex due to patient frailty and therapeutic side effects or intolerance with advanced cardiac disease. This is particularly relevant for those with AL-CA, when active teamwork between the hematologist-oncologist and the cardiologist is critical for treatment success. Often, referral to an expert center is necessary for timely diagnosis, initiation of treatment, and participation in clinical trials.Cardiac sarcoidosis (CS) is a widely underdiagnosed yet clinically significant form of granulomatous myocarditis associated with significant morbidity and mortality. Clinical presentation ranges from silent cardiac involvement detected on imaging to cardiomyopathy or sudden cardiac death. Diagnosis of CS remains challenging due to the lack of sensitivity and specificity of any single diagnostic method, underscoring the importance of elevated clinical suspicion and the use of multimodality imaging to guide diagnosis and treatment. In this review, we discuss the epidemiology, pathogenesis, clinical features, and diagnosis of this clinically evading and enigmatic disease.A 52-year-old female was admitted to our hospital in April 2021 with dyspnea. She was discharged from the hospital 3 weeks ago due to the diagnosis of pneumonia caused by coronavirus disease 2019 (COVID-19). Physical examination revealed an oxygen desaturation of 82%. The patient underwent computed tomography angiography (CTA) that showed a ground-glass pattern and a giant left atrial appendage ( Figure 1A ). Film array respiratory panel was negative, and pulmonary aspergillosis was diagnosed after bronchoscopy. Cardiac magnetic resonance corroborated the huge left atrial appendage ( Figure 1B ). No other structural or functional heart abnormalities were diagnosed. A giant left atrial appendage is a rare cardiac anomaly that can be congenital or acquired. In the literature, it is called a left atrial appendage aneurysm. The dilatation can be generalized or focused. Although it can occur in all age groups, it is predominant in patients in their 30s to 50s and most common in females.1 Patients can be asymptomatic or present with symptoms such as palpitations, chest pain, or dyspnea. A number of recent cases in the literature have highlighted the diagnostic utility of CTA.2 While there is no standard treatment for this condition, surgical resection is the most frequent therapy. Another option reported in the literature is anticoagulant treatment for select cases.3 Closure of the left atrial appendage is a more recent and emerging intervention that can be considered. In our patient, we initiated anticoagulant therapy to reduce the risk of thromboembolic events; however, we recommended left atrial appendage occlusion or surgical resection after completing the treatment for pulmonary aspergillosis.Do not go gentle into that good night,Old age should burn and rave at close of day;Rage, rage against the dying of the light. Though wise men at their end know dark is right,Because their works had forked no lightning theyDo not go gentle into that good night. Good men, the last wave by, crying how brightTheir frail deeds might have danced in a green bay,Rage, rage against the dying of the light. Wild men who caught and sang the sun in flight,And learn, too late, they grieved it on its way,Do not go gentle into that good night. Grave men, near death, who see with blinding sightBlind eyes could blaze like meteors and be gay,Rage, rage against the dying of the light. And you, my father, there on the sad height,Curse, bless, me now with your fierce tears, I pray.Do not go gentle into that good night.Rage, rage against the dying of the light. Dylan ThomasThe Poems of Dylan Thomas, New Directions Publishing 1971. This poem is in the public domain.Amyloidosis is a disorder of protein misfolding and metabolism in which insoluble fibrils are deposited in various tissues, causing organ dysfunction and eventually death. Out of the 60-plus heterogeneous amyloidogenic proteins that have been identified, approximately 30 are associated with human disease. The unifying feature of these proteins is their tendency to form beta-pleated sheets aligned in an antiparallel fashion. These sheets then form rigid, nonbranching fibrils that resist proteolysis, causing mechanical disruption and local oxidative stress in affected organs such as the heart, liver, kidneys, nervous system, and gastrointestinal tract. Here we review the epidemiology of light chain amyloidosis, the staging, and the concomitant prognostication that is critical in determining the appropriate treatment.The column in this issue is provided by Salil Kumar, MD, and Joe Aoun, MD, chief cardiology fellows in the Houston Methodist Cardiology Department, and Arvind Bhimaraj, MD, associate professor of Clinical Cardiology at the Houston Methodist Academic Institute. Dr. Bhimaraj specializes in cardiovascular disease and advanced heart failure and transplantation.
To describe changes in hospital-based care for children with neurologic diagnoses during the initial 6weeks following regional Coronavirus 2019 Shelter-in-Place orders.

This retrospective cross-sectional study of 7 US and Canadian pediatric tertiary care institutions included emergency and inpatient encounters with a neurologic primary discharge diagnosis code in the initial 6weeks of Shelter-in-Place (COVID-SiP), compared to the same period during the prior 3years (Pre-COVID). Patient demographics, encounter length, and neuroimaging and electroencephalography use were extracted from the medical record.

27,900 encounters over 4years were included. Compared to Pre-COVID, there was a 54% reduction in encounters during Shelter-in-Place. COVID-SiP patients were younger (median 5years vs 7years). The incidence of encounters for migraine fell by 72%, and encounters for acute diagnoses of status epilepticus, infantile spasms, and traumatic brain injury dropped by 53%, 55%, and 56%, respectively. There was an increase in hospital length of stay, relative utilization of intensive care, and diagnostic testing (long-term electroencephalography, brain MRI, and head CT (all
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