Practical Recommendations for the Diagnosis and Management of Lysosomal Acid Lipase Deficiency with a Focus on Wolman Disease.

Lysosomal acid lipase deficiency (LAL-D) is an ultra-rare lysosomal storage disease with two distinct phenotypes, an infantile-onset form (formerly Wolman disease) and a later-onset form (formerly cholesteryl ester storage disease). The objective of this narrative review is to examine the most important aspects of the diagnosis and treatment of LAL-D and to provide practical expert recommendations. The infantile-onset form occurs in the first weeks of life and is characterized by malnourishment and failure to thrive due to gastrointestinal impairment (vomiting, diarrhea, malabsorption), as well as systemic inflammation, hepatosplenomegaly, and adrenal calcifications.

Stroke severity shapes extracellular vesicle profiles and their impact on the cerebral endothelial cells.

Ischaemic stroke is a leading cause of death and disability. Circulating extracellular vesicles (EVs) post-stroke may help brain endothelial cells (BECs) counter ischaemic injury. However data on how EVs from ischaemic stroke patients, considering injury severity, affect these cells are limited.

Dynamic reconfiguration of aperiodic brain activity supports cognitive functioning in epilepsy: A neural fingerprint identification.

Temporal lobe epilepsy (TLE) is characterized by alterations of brain dynamic on a large-scale associated with altered cognitive functioning. Here, we aimed at analyzing dynamic reconfiguration of brain activity, using the neural fingerprint approach, to delineate subject-specific characteristics and their cognitive correlates in TLE. We collected 10 min of resting-state scalp-electroencephalography (EEG, 128 channels), free from epileptiform activity, from 68 TLE patients and 34 controls.

Recent Advances in the Study of Conflict-Related Sexual Violence.

Purpose Of Review: Conflict-related sexual violence (CRSV) is a frequent but not ubiquitous part of warfare, affecting not only survivors but also their families and wider communities. This thematic review describes advances in research on CRSV, reviews new developments in the literature, and proposes recommendations for future study.

Recent Findings: An increasing number of studies consider how to address methodological and ethical research challenges, how understudied victim/survivor groups as well as families and communities are affected by CRSV, how survivor-centered justice mechanisms can be developed to reduce impunity, and how CRSV is perceived by different actors.

The Predict4All open-source word prediction and word correction engine in French language: a feasibility study.

Purpose: Information and communication technologies are crucial for social and professional integration, but access to technology can be difficult for people with physical impairments. Text entry can be slow and tiring. We developed a free and open-source module called for use with AAC (augmentative/alternative communication) software in French language.