Preserved emotional awareness of pain in a patient with extensive bilateral damage to the insula, anterior cingulate, and amygdala

Functional neuroimaging investigations of pain have discovered a reliable pattern of activation within limbic regions of a putative "pain matrix" that has been theorized to reflect the affective dimension of pain. To test this theory, we evaluated the experience of pain in a rare neurological patient with extensive bilateral lesions encompassing core limbic structures of the pain matrix, including the insula, anterior cingulate, and amygdala. Despite widespread damage to these regions, the patient's expression and experience of pain was intact, and at times excessive in nature. This finding was consistent across multiple pain measures including self-report, facial expression, vocalization, withdrawal reaction, and autonomic response. These results challenge the notion of a "pain matrix" and provide direct evidence that the insula, anterior cingulate, and amygdala are not necessary for feeling the suffering inherent to pain. The patient's heightened degree of pain affect further suggests that these regions may be more important for the regulation of pain rather than providing the decisive substrate for pain's conscious experience

Clinical and laboratory risk factors for sickle cell retinopathy and maculopathy: a scoping review of the current evidence.

Sickle cell retinopathy (SCR) is a complication of sickle cell disease (SCD) and can drastically impair visual acuity. Screening for SCR is, therefore, recommended, but evidence for optimal screening frequency on an individual level is lacking. This scoping review mapped the current evidence on risk factors for SCR and sickle cell maculopathy (SCM). A literature search (in Medline [Ovid]), Embase [Ovid]), and Scopus) resulted in 67 included articles which covered demographic risk factors, genetic risk factors, systemic therapy, correlations with other forms of SCD-related organ damage, and hematologic risk factors. SCR risk factors include older age, male sex, HbSC genotype, hemolysis, and HbF% <15% (in HbSS) and increased blood viscosity (in HbSC). For SCM, risk factors are older age, HbSS genotype, and higher degree of hemolysis. The pathophysiology of SCR and SCM appears multifactorial, but distinct patterns emerge suggesting that vaso-occlusion and hemolysis cause SCM and NPSCR in HbSS, while hyperviscosity in HbSC leads to peripheral retinopathy. We recommend yearly screening for high-risk patients (older HbSC males) and triennial screening for low-risk patients (young females HbSS with HbF>15%) to ensure comprehensive yet proportionate ophthalmic care. However, future studies are needed on the role of interventions for SCR and the long-term consequences of SCM in order to evaluate and define appropriate screening schedules

Increasing risks of extreme salt intrusion events across European estuaries in a warming climate

Over the last decade, many estuaries worldwide have faced increased salt intrusion as a result of human activities and a changing climate. Despite its socio-economic importance, our current projections on the statistics of future salt intrusion are limited to case studies in certain regions. Here, we show that, compared to present-day conditions, river discharge in the summer months is projected to be reduced by 10–60% in 17 out of 22 investigated major European river basins at the end of the 21st century under the high CO2 emission scenario (Shared Socioeconomic Pathways, SSP 3-7.0). We find that the reduced future river discharge in the summer months, in turn, increases salt intrusion lengths by 10–30% in 9 representative European estuaries at low and mid latitudes. Our analysis further indicates that the European estuaries are projected to experience more than five times more frequent extreme salt intrusion events

Explaining the Statistical Properties of Salt Intrusion in Estuaries Using a Stochastic Dynamical Modeling Approach

Determining the statistical properties of salt intrusion in estuaries on sub-tidal time scales is a substantial challenge in environmental modeling. To study these properties, we here extend an idealized deterministic salt intrusion model to a stochastic one by including a stochastic model of the river discharge. In the river discharge model, two types of stochastic forcing are used: one independent (additive noise) and one dependent (multiplicative noise) on the river discharge state. Each type of forcing results in a non-Gaussian response in the salt intrusion length, which we consider here as the distance of the 2 psu isohaline contour to the estuary mouth. The salt intrusion model including both types of stochastic forcing in the river discharge provides a satisfactory explanation of the multi-year statistics of observed salt intrusion lengths in the San Francisco Bay estuary, in particular for the skewness of its probability density function

Withholding transfusion therapy in children with sickle cell disease with abnormal transcranial doppler and normal magnetic resonance angiography: a retrospective analysis

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