Pathogenesis, manifestations, diagnosis, and management of CNS complications in hereditary ATTR amyloidosis.

The clinical efficacy of transthyretin (TTR) tetramer stabilisers and gene silencers in addition to liver transplantation has been established for hereditary ATTR (ATTRv) amyloidosis. Accordingly, non-central nervous system (CNS) systemic amyloidosis manifestations, such as peripheral neuropathy and cardiomyopathy, are now being overcome. However, emerging disease-modifying therapeutics have limited effects on CNS amyloidosis since they target the blood-circulating TTR produced in the liver, and not the cerebral spinal fluid (CSF) TTR synthesised in the choroid plexus.

[Analysis of the operational conditions to preserve immunobiological products in vaccination rooms in Brazil: a mixed study].

This study aimed to analyze the operational conditions to preserve immunobiological products in Brazil. This mixed-method study with a sequential explanatory design was developed in vaccination rooms in several Brazilian regions from 2021 to 2022. Its quantitative stage developed a descriptive cross-sectional study by applying the Immunobiological Conservation Assessment Scale to nursing professionals.

Brain MRI in patients with V30M hereditary transthyretin amyloidosis.

Background: Central nervous system dysfunction is common in longstanding hereditary transthyretin amyloidosis (ATTRv) caused by the V30M (p.V50M) mutation. Neuropathology studies show leptomeningeal amyloid deposition and cerebral amyloid angiopathy (CAA).

Palinacousis in amyloidosis: exploring the hallucinatory phenomenon in brain pathology-a case report.

Background: Hereditary transthyretin amyloidosis, caused by transthyretin gene mutations, progresses with systemic impact and often presents peripheral neuropathy. Recent research reveals central nervous system involvement, marked by leptomeningeal amyloid accumulation and transient focal neurological episodes displaying cortical dysfunction.

Case Presentation: A 47-year-old Caucasian man with hereditary transthyretin amyloidosis presented with motor aphasia, right hemiparesis, fever, and an altered state of consciousness.

Effects of bottom-up versus top-down digital cognitive training in older adults: A randomized controlled trial.

Introduction: Impairments in bottom-up perceptual processing have been associated to the age-related cognitive decline. Digital cognitive training focusing on bottom-up and/or top-down processes have been studied as a tool to remediate age-related cognitive decline. However, the most effective training type and order of application remain unclear.

Alterations in the pro-resolving lipid mediator machinery within first trimester maternal tissue: Implications in decidualization and miscarriage risk.

A pivotal event in uterine receptivity and human reproduction is the differentiation of endometrial stromal cells into decidual cells, known as decidualization. Decidualization is interlinked with its inflammatory environment. Our study aimed to investigate the presence and role of pro-resolving lipid mediators in first trimester maternal tissue.

Neuropathology of central nervous system involvement in TTR amyloidosis.

Hereditary transthyretin amyloidosis (ATTRv) is a systemic disease caused by the accumulation of misfolded transthyretin (TTR). It usually presents with an adult-onset progressive axonal peripheral neuropathy and cardiomyopathy. In the central nervous system (CNS), variant TTR is produced by the choroid plexus and accumulates in the leptomeninges.

Intermittent versus Continuous Catheterization and Differences in the Evolution of Labor: Systematic Review and Meta-analysis.

Objective:  To evaluate the differences between bladder emptying options (permanent catheterization and intermittent bladder emptying/spontaneous urination) regarding the effects on labor length, need of operative vaginal deliveries, and cesarean section rate.

Data Sources:  The search was conducted in MEDLINE, Scopus, Web of Science, and The Cochrane Central Register of Controlled Trials databases.

Selection Of Studies:  The survey returned 964 studies.

Obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment: a systematic review and meta-analysis of prevalence.

Objective/background: Sleep disorders in cystic fibrosis may be present before daytime clinical manifestations, regardless of lung function impairment, affecting quality of life and disease progression. This study investigated the prevalence of obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment, and evaluated its association with clinical variables.

Methods: A systematic review with meta-analysis of prevalence was conducted, including observational studies with polysomnographies in patients with cystic fibrosis who presented mean lung function values > 60% predicted.

CNS Involvement in Hereditary Transthyretin Amyloidosis.

Hereditary transthyretin amyloidosis (ATTRv amyloidosis) is predominantly a disease of the peripheral nerves, heart, kidney, and eye. CNS involvement has been a marginal issue in research and the clinical setting until recently. Growing evidence shows that leptomeningeal amyloid accumulation is frequent and present from early stages of ATTRv amyloidosis.

Assessment of the Financial Capacity in the Context of Normal and Pathological Cognitive Aging. Preliminary Analysis of the Portuguese Version of the (NADL-F).

Financial capacity (FC) is conceptualized as a dimension that encompasses a wide range of basic aptitudes and the capacity to judge and assess situations and make decisions according to one best interests. The (NADL-F) is an instrument of FC for clinical use developed in Italy. This study aims to perform a preliminary analysis on the psychometric characteristics of the Portuguese version of NADL-F.

The "Script" of a New Urban Layout: Mobility, Environment, and Embellishment in Lisbon's Streets (1850-1910).

This article is at the crossroads of two scholarly traditions: the history of technology studying urban infrastructures and the history of urban planning. Using Actor Network Theory's concept of "script," it analyzes the strategic role played by the street to overcome nineteenth-century urban problems. The street was simultaneously the epicenter of the "urban question" and the key to its solution.

Hyporeninemic hypoaldosteronism and diabetes mellitus: Pathophysiology assumptions, clinical aspects and implications for management.

Patients with diabetes mellitus (DM) frequently develop electrolyte disorders, including hyperkalemia. The most important causal factor of chronic hyperkalemia in patients with diabetes is the syndrome of hyporeninemic hypoaldosteronism (HH), but other conditions may also contribute. Moreover, as hyperkalemia is related to the blockage of the renin-angiotensin-aldosterone system (RAAS) and HH is most common among patients with mild to moderate renal insufficiency due to diabetic nephropathy (DN), the proper evaluation and management of these patients is quite complex.

Computational simulation of carotid stenosis and flow dynamics based on patient ultrasound data - A new tool for risk assessment and surgical planning.

Purpose: There is nowadays extensive experimental and computational investigation on the pathophysiology of atherosclerosis, searching correlations between its focal nature and local hemodynamic environment. The goal of this work is to present a methodology for patient-specific hemodynamics study of the carotid artery bifurcation based on the use of ultrasound (US) morphological and blood flow velocity patient data.

Materials/methods: Subject-specific studies were performed for two patients, using a developed finite element code.

Toward hemodynamic diagnosis of carotid artery stenosis based on ultrasound image data and computational modeling.

The ability of using non-expensive ultrasound (US) image data together with computer fluid simulation to access various severities of carotid stenosis was inquired in this study. Subject-specific hemodynamic conditions were simulated using a developed finite element solver. Individual structured meshing of the common carotid artery (CCA) bifurcation was built from segmented longitudinal and cross-sectional US images; imposed boundary velocities were based on Doppler US measurements.

Blood flow simulation and vascular reconstruction.

In medical practice, bypass grafts are commonly used as an alternative route around strongly stenosed or occluded arteries. In contrast to arterial bifurcations, surgically created anastomosis can be modified with the objective of enabling optimal graft geometry to yield a flow environment that improves its longevity. This paper presents a three dimensional numerical study of blood flow through bypass systems with different geometries.