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1.

Background

Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated disease manifesting in thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. It has a higher incidence of extrarenal manifestations, including central nervous system findings like seizure or stroke, pancreatitis, and cardiac manifestations.

Case Report

We present a case of an unimmunized 14-month-old girl presenting with generalized seizure and ultimately diagnosed with aHUS.

Why Should an Emergency Physician Be Aware of This?

These atypical neurological symptoms can cause the diagnosis to be commonly missed in the emergency department. The etiology of approximately 60% of patients with aHUS can be attributed to genetic mutations in complement regulators including factor H, membrane cofactor protein, factor I, activator factor B, or C3. Although previously treated with plasma transfusion and immunosuppressants, eculizumab is a newer treatment that has been changing prognosis and management of aHUS, but it should be administered within 48 h of symptom onset for best efficacy.  相似文献   
2.

Background

Spontaneous spinal and intracranial subdural hematomas are rarely reported, especially occurring simultaneously. Anticoagulation use has been associated with spontaneous hemorrhages. Prompt diagnosis is required to prevent permanent neurological sequelae. In this case report, we describe a spontaneous spinal and intracranial subdural hematoma in a woman taking warfarin and initially presenting with severe vaginal pain.

Case Report

A 42-year-old woman who had a history of mechanical valve replacement and was therefore taking warfarin, came to an emergency department for relief of severe vaginal pain. Mild concurrent lumbar pain increased concern about spinal pathology, so magnetic resonance imaging of her spine was performed. It revealed a subdural hematoma extending from L1–S1 with arachnoiditis, which suggested intracranial pathology, though the patient had no complaint of a headache. Computed tomography of her brain demonstrated a large right subdural hemorrhage with midline shift. Subsequent imaging revealed no aneurysm or source of the intracranial bleeding. We concluded that the patient experienced spontaneous anticoagulation-related intracranial hemorrhage resulting in lumbar subdural hematoma and arachnoiditis with referred vaginal pain.

Why Should an Emergency Physician Be Aware of This?

Pelvic, vaginal, or perineal pain may be the presenting symptom in patients with lower spinal pathology. It is important to consider causes other than gynecological ones in the differential diagnosis of these patients, as well as to be cognizant of the relationship between spinal and intracranial subdural hemorrhages. In patients with back pain or radiating lumbar pain, especially coupled with neurological effects, clinicians should consider spinal subdural hemorrhage and arachnoiditis to expedite imaging studies and treatment of these rare entities.  相似文献   
3.

Introduction

There are limited randomized data comparing radical cystectomy (RC) with bladder-sparing tri-modality therapy (TMT) in the treatment of muscle-invasive bladder cancer (MIBC). Both strategies are thought to have similar survival outcomes with different morbidity profiles. We compare the effectiveness of TMT and RC using decision-analytic modeling and the endpoint of quality-adjusted life years (QALYs).

Patients and Methods

Using a Markov model, we simulated the lifetime outcomes after TMT versus RC ± neoadjuvant chemotherapy for 67-year-old patients with clinical stage T2-T4aN0M0 MIBC. Model probabilities and utilities were extracted from the literature. The incremental effectiveness was reported in QALYs and sensitivity analyses were performed.

Results

For all patients with MIBC, although the model showed identical survival, TMT was the most effective strategy with an incremental gain of 0.59 QALYs over RC (7.83 vs. 7.24 QALYs, respectively). When limiting the model to favorable, contemporary cohorts in both the TMT and RC strategies, TMT remained more effective with an incremental gain of 1.61 QALYs (9.37 vs. 7.76 QALYs, respectively). One-way sensitivity analyses demonstrated the model was sensitive to the quality of life parameters (ie, the utilities) for RC and TMT. When testing the 95% confidence interval of the RC utility parameter the model demonstrated an incremental gain with TMT from ?0.54 to 4.23 QALYs. Probabilistic sensitivity analysis demonstrated that TMT was more effective than RC for 63% of model iterations.

Conclusions

This modeling study found that treatment of MIBC with organ-sparing TMT in appropriately-selected patients may result in a gain of QALYs relative to RC.  相似文献   
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A recently published framework for the diagnosis of Alzheimer’s disease (AD) in research studies would allow diagnosis on the sole basis of two biomarkers (β-amyloid and pathologic tau), even in people with no objective or subjective memory or cognitive changes. This revision will have substantial implications for future Alzheimer’s research, and the changes should be rigorously evaluated before widespread adoption. We propose three principles for evaluating any revision to diagnostic frameworks for AD: (1) does the revision improve the validity of the diagnosis; (2) does the revision improve the reliability or reduce the expense of the diagnosis; and (3) will the revision foster innovative and rigorous research across populations. The new diagnostic framework is unlikely to achieve any of these goals. Instead, it has the potential to handicap future researchers, and slow progress towards identifying effective strategies to prevent or treat AD.  相似文献   
9.
PurposeThoracic aortic aneurysm (TAA) is a cardiovascular disease characterized by increased aortic diameter, treated with surgery and endovascular therapy in order to avoid aortic dissection or rupture. The mechanism of TAA formation has not been thoroughly studied and many factors have been proposed to drive its progression; however strong focus is attributed to modification of smooth muscle cells (SMCs). Latest research indicates, that microRNAs (miRNAs) may play a significant role in TAA development – these are multifunctional molecules consisting of 19–24 nucleotides involved in regulation of the gene expression level related to many biological processes, i.e. cardiovascular disease pathophysiology, immunity or inflammation.Materials and methodsPrimary SMCs were isolated from aortic scraps of TAA patients and age- and sex-matched healthy controls. Purity of isolated SMCs was determined by flow cytometry using specific markers: α-SMA, CALP, MHC and VIM. Real-time polymerase chain reaction (RT-PCR) was conducted for miRNA analysis.ResultsWe established an isolation protocol and investigated the miRNA expression level in SMCs isolated from aneurysmal and non-aneurysmal aortic samples. We identified that let-7 g (0.71-fold, p = 0.01), miR-130a (0.40-fold, p = 0.04), and miR-221 (0.49-fold, p = 0.05) significantly differed between TAA patients and healthy controls.ConclusionsFurther studies are required to improve our understanding of the pathophysiology underlying TAA, which may aid the development of novel, targeted therapies. The pivotal role of miRNAs in the cardiovascular system provides a new perspective on the pathophysiology of thoracic aortic aneurysms.  相似文献   
10.
Previous behavioral reports of the African lorisid, Perodicticus potto, have speculated that these animals have an extraordinary grip strength. This ability is hypothesized to be facilitated by a range of anatomical features within the forelimb, ranging from the presence of a retia mirabilia in its wrist to the hyper‐abduction of its pollex. Despite numerous behavioral reports, however, this claim of extraordinary grip strength has not been empirically substantiated. This study quantifies the physiological cross‐sectional area of the digital flexor muscles within P. potto. These data are compared with a broad primate sample, including several similarly sized strepsirrhines. Contrary to expectation, we found that P. potto actually has relatively below‐average digital flexor PCSA. However, we identified other myological characteristics in the upper limb of P. potto that were unexpected, including the largest brachioradialis muscle (an elbow flexor) among our primate sample, and – despite P. potto having only a vestigial second digit – an independent digital extensor indicis that is absent in almost a quarter of our primate sample.  相似文献   
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