The burden of Alagille syndrome: uncovering the potential of emerging therapeutics - a comprehensive systematic literature review.

Alagille syndrome (ALGS) is a rare, cholestatic multiorgan disease associated with bile duct paucity, leading to cholestasis. Clinical symptoms of cholestasis include debilitating pruritus, xanthomas, fat-soluble vitamin deficiencies, growth failure, renal disease and impaired health-related quality of life (HRQoL). The main objective was to review the current literature on the epidemiological, clinical, psychosocial and economic burden of ALGS in view of the development of ileal bile acid transporter (IBAT) inhibitors.

The Relationship Between Novel Inflammatory Markers and Serum 25-Hydroxyvitamin D Among US Adults.

Background: Vitamin D is the focus of extensive medical research globally. Recent studies have investigated the correlation between serum 25-hydroxyvitamin D (25(OH)D) and common inflammatory markers. However, few studies have incorporated novel inflammatory markers such as the platelet-to-lymphocyte ratio (PLR), platelet-to-high density lipoprotein cholesterol ratio (PHR), systemic inflammatory index (SII), neutrophil-to-lymphocyte ratio (NLR), systemic inflammatory response (SIRI), and neutrophil-to-high-density lipoprotein cholesterol ratio (NHR).

Beyond the binary: Inferential challenges and solutions in cognitive archaeology.

We welcome Stibbard-Hawkes's empirical contributions and discussion of interpretive challenges for archaeology, but question some of his characterizations and conclusions. Moving beyond critique, it is time to develop new research methods that eschew simplistic modern/premodern binaries. We advocate an inductive, probabilistic approach using multiple lines of evidence to infer the causes and consequences of behavioral variability across time and space.

Shared intentionality may have been favored by persistence hunting in .

Shared intentionality is the derived hominin motivation and skills to align mental states. Research on the role of interdependence in the phylogeny of shared intentionality has only considered the archeological record of . But ethnographic and fossil data must be considered, too.

Don't ignore cognitive evolution during the three million years that preceded the archaeological record of material culture!

The target article rightly questions whether the archaeological record is useful for identifying sea changes in hominin cognitive abilities. This commentary suggests an alternative approach of synthesizing findings from primatology, evolutionary developmental biology, and paleoanthropology to formulate hypotheses about cognitive evolution in hominins that lived during the three million years that preceded the record of material culture (the Botanic Age).

Cultural innovation is not only a product of cognition but also of cultural context.

Innovations, such as symbolic artifacts, are a product of cognitive abilities but also of cultural context. Factors that may determine the emergence and retention of an innovation include the population's pre-existing cultural repertoire, exposure to relevant ways of thinking, and the invention's utility. Thus, we suggest that the production of symbolic artifacts is not guaranteed even in cognitively advanced societies.

Advancing paleoanthropology beyond default nulls.

While we are sympathetic with Stibbard-Hawkes' approach, we disagree with the proposal to switch to a "cognitively modern" null for all species. We argue in favor of a more evidence-driven approach, inspired by recent debates in comparative cognition. Ultimately, parsing the contributions of different genetic and extra-genetic factors in human evolution is more promising than setting a priori nulls.

Sports, team games, and physical skill competitions as an important source of symbolic material culture with low preservation probability.

Sports, team games, and physical skill competitions appear to be a human universal and may have been prevalent throughout the hominin lineage. These activities are cognitively complex and can be associated with a distinctive and symbolic material culture. Yet, many of the artifacts used by foraging groups for sports, team games, and athletic competitions often have a low preservation probability.

Are we jingling modern hunter-gatherers and early ?

Using modern hunter-gatherers to infer about early only works if at least (a) modern hunter-gatherers represent an unbiased sample of humanity, and (b) modern hunter-gatherers act in ways similar to the behavior of early . Both of these are false, leading to the problem of whether we can draw conclusions about early from modern hunter-gatherers.

Behavioural modernity is dead: Long live behavioural modernity.

Using Neanderthal symbolism, I extend on Stibbard-Hawkes to show that reconsidering the link between cognitive capacity and material culture extends beyond matters of preservation. A reconceptualization of behavioural modernity inclusive of both extant and extinct populations must begin with an honest theoretical separation of biological and behavioural modernity, which requires to critically engage with how we frame the underlying questions.

What would be pre-modern human cognition?

Stibbard-Hawkes's detailed demonstration that in the case of hunter-gatherer artifacts, absence of evidence is not evidence of absence must never be forgotten. The belief that there is a single coherent "human cognitive capacity" difference between modern humans and some unspecified earlier form should be rigorously re-examined.

Lyme neuroborreliosis in Japan: Borrelia burgdorferi sensu lato as a cause of meningitis of previously undetermined etiology in hospitalized patients outside of the island of Hokkaido, 2010-2021.

Background And Purpose: Clinical manifestations of Lyme borreliosis (LB), caused by Borrelia burgdorferi sensu lato (Bbsl), include erythema migrans, Lyme neuroborreliosis (LNB), carditis, and arthritis. LB is a notifiable disease in Japan with <30 surveillance-reported LB cases annually, predominately from Hokkaido Prefecture. However, LB, including LNB, may be under-diagnosed in Japan since diagnostic tests are not readily available.

Blended phenotype of TECPR2-associated hereditary sensory-autonomic neuropathy and Temple syndrome.

Uniparental isodisomy (UPiD) can cause mixed phenotypes of imprinting disorders and autosomal-recessive diseases. We present the case of a 3-year-old male with a blended phenotype of TECPR2-related hereditary sensory and autonomic neuropathy (HSAN9) and Temple syndrome (TS14) due to maternal UPiD of chromosome 14, which includes a loss-of-function founder variant in the TECPR2 gene [NM_014844.5: c.

Clinical criteria for limbic-predominant age-related TDP-43 encephalopathy.

Limbic predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC) is highly prevalent in late life and a common co-pathology with Alzheimer's disease neuropathologic change (ADNC). LATE-NC is a slowly progressive, amnestic clinical syndrome. Alternatively, when present with ADNC, LATE-NC is associated with a more rapid course.

Pre-emptive treatment for cytomegalovirus viraemia to prevent cytomegalovirus disease in solid organ transplant recipients.

Background: Cytomegalovirus (CMV) is a significant cause of morbidity and death in solid organ transplant recipients. Pre-emptive treatment of patients with CMV viraemia using antiviral agents has been suggested as an alternative to routine prophylaxis to prevent CMV disease. This is an update of a Cochrane review first published in 2006 and updated in 2013.

A universal framework for design and manufacture of deterministic lateral displacement chips.

Despite being a high-resolution separation technique, deterministic lateral displacement (DLD) technology is facing multiple challenges with regard to design, manufacture, and operation of pertinent devices. This work specifically aims at alleviating difficulties associated with design and manufacture of DLD chips. The process of design and production of computer-aided design (CAD) mask layout files that are typically required for computational modeling analysis, optimization, as well as for manufacturing DLD-based micro/nanofluidic chips is complex, time-consuming, and often necessitates a high level of expertise in the field.

Clinical utility of plasma p-tau217 in identifying abnormal brain amyloid burden in an Asian cohort with high prevalence of concomitant cerebrovascular disease.

Introduction: Using an Asian cohort with high prevalence of concomitant cerebrovascular disease (CeVD), we evaluated the performance of a plasma immunoassay for tau phosphorylated at threonine 217 (p-tau217) in detecting amyloid beta positivity (Aβ+) on positron emission tomography and cognitive decline, based on a three-range reference, which stratified patients into low-, intermediate-, and high-risk groups for Aβ+.

Methods: Brain amyloid status (Aβ- [n = 142] vs Aβ+ [n = 73]) on amyloid PET scans was assessed along with the plasma ALZpath p-tau217 assay to derive three-range reference points for PET Aβ+ based on 90% sensitivity (lower threshold) and 90% specificity (upper threshold).

Results: Plasma p-tau217 (area under the curve [AUC] = 0.

AAPM Truth-based CT (TrueCT) reconstruction grand challenge.

Background: This Special Report summarizes the 2022, AAPM grand challenge on Truth-based CT image reconstruction.

Purpose: To provide an objective framework for evaluating CT reconstruction methods using virtual imaging resources consisting of a library of simulated CT projection images of a population of human models with various diseases.

Methods: Two hundred unique anthropomorphic, computational models were created with varied diseases consisting of 67 emphysema, 67 lung lesions, and 66 liver lesions.

Towards a unified approach in managing resistance to vaccines, drugs, and pesticides.

Everywhere, pests and pathogens evolve resistance to our control efforts, impairing human health and welfare. Developing sustainable solutions to this problem requires working with evolved immune and ecological systems, rather than against these evolutionary forces. We advocate a transdisciplinary approach to resistance based on an evolutionary foundation informed by the concepts of integrated pest management and One Health.

Cognitive aging outcomes are related to both tau pathology and maintenance of cingulate cortex structure.

Introduction: Successful cognitive aging is related to both maintaining brain structure and avoiding Alzheimer's disease (AD) pathology, but how these factors interplay is unclear.

Methods: A total of 109 cognitively normal older adults (70+ years old) underwent amyloid beta (Aβ) and tau positron emission tomography (PET) imaging, structural magnetic resonance imaging (MRI), and cognitive testing. Cognitive aging was quantified using the cognitive age gap (CAG), subtracting chronological age from predicted cognitive age.

Simple and safe redo sternotomy technique using the ultrasonic bone scalpel for patients with an embedded calcified conduit.

With the increase of patients with adult congenital heart disease, the number of high-risk multiple redo sternotomies is increasing. Calcified conduit embedded in the sternum or large vessels attached to the sternum presents an especially challenging case. This video tutorial presents a simple safe redo sternotomy technique using an ultrasonic bone scalpel in such high-risk patients.