Sex-related differences in response to lomitapide in HoFH: A subanalysis of the Pan-European Lomitapide retrospective observational study.

Background And Aims: Homozygous familial hypercholesterolemia (HoFH) is a hereditary lipid metabolism disorder characterized by severe elevation of low-density lipoprotein cholesterol (LDL-C) and heightened risk of premature atherosclerotic cardiovascular disease (ASCVD). Lomitapide, an inhibitor of microsomal triglyceride transfer protein, has shown promise in reducing LDL-C levels, albeit with variable response in real-world settings. Sex-based differences in treatment efficacy and safety remain unclear.

Tip efficiency of a customized lingual appliance: Performance of wires with two different ligatures.

Tip control in lingual orthodontics may be challenging because of the presence of a vertical slot and the particular configuration of the customized appliances. The aim of this in vitro experimental study was to investigate the role of the ligature-wire-slot system in achieving better tip control. A set of customized lingual brackets was obtained for a dedicated typodont made of extracted human teeth.

Effects of Removable Functional Appliances on the Dentoalveolar Unit in Growing Patients.

The objective of this retrospective controlled study is to compare class II growing patients who underwent treatment with two different functional appliances: the Fraenkel regulator (FR-2), utilized as the control group, and the elastodontic device "Cranium Occluded Postural Multifunctional Harmonizers" (AMCOP), utilized as the test group. The study sample consisted of 52 patients with class II division I malocclusion (30 males, 22 females, mean age 8.6 ± 1.

Role of triglyceride-glucose index in metabolic assessment of sarcoidosis patients.

Sarcoidosis is a rare granulomatous disease that can affect any organ. It leads to an increased risk of metabolic syndrome and insulin resistance, due to biochemical pathways involved in low-grade inflammation in both diseases. The aim of our retrospective case-control study was to evaluate the utility of triglyceride-glucose (TyG) index, a surrogate of insulin resistance, for metabolic assessment of sarcoidosis patients.

Minimal Compositionality versus Bird Implicatures: two theories of ABC-D sequences in Japanese tits.

It was argued in a series of experimental studies that Japanese tits (Parus minor) have an ABC call that has an alert function, a D call that has a recruitment function, and an ABC-D call that is compositionally derived from ABC and D, and has a mobbing function. A key conclusion was that ABC-D differs from the combination of separate utterances of ABC and of D (e.g.

Cardiovascular risk in rare diseases: a prognostic stratification model in a cohort of sarcoidosis patients.

Sarcoidosis is a rare granulomatous disease that can affect any organ; as other chronic diseases, it leads to increased risk of atherosclerosis and cardiovascular (CV) disease. The aim of our observational study was to define a prognostic stratification model of sarcoidosis patients based on the evaluation of CV risk through common carotid Doppler ultrasound and cardiovascular risk scores assessment; for this reason, a clinical phenotyping of sarcoidosis patients in four subgroups was done, based on the different organ involvement. A cohort of 53 sarcoidosis patients and a cohort of 48 healthy volunteers were enrolled.

Sarcoidosis and autoimmunity: In the depth of a complex relationship.

Sarcoidosis is a chronic granulomatous disease that can virtually affect any organ. Its etiology is unknown, although it has been proposed that environmental or biological agents can act as triggers, ultimately leading to chronic inflammation in genetically predisposed individuals. The main component of sarcoid inflammation is represented by an exaggerated T- lymphocytic cellular response to a putative antigen that could not be efficiently cleared in the patient.

Clinical phenotyping in sarcoidosis management.

Unlabelled: Sarcoidosis is a heterogeneous granulomatous disease. Biological markers and clinical features could allow specific phenotypes to be associated with different prognosis, severity and treatment responses. This retrospective multicentre study aims to analyse the clinical and immunological features of sarcoidosis and to identify a routine non-invasive biomarker useful in clinical practice.

The Layered Syntactic Structure of the Complementizer System: Functional Heads and Multiple Movements in the Early Left-Periphery. A Corpus Study on Italian.

In this paper we document the developmental trajectory of the complementizer system (CP-system) in Italian by looking at the earliest spontaneous production of eleven young children, whose transcriptions are available on CHILDES. We conducted a novel corpus analysis, tracking down a number of constructions in which the clausal left-periphery is activated. First, we considered the appearance of the different complementizer particles in the CP-system, which overtly realize the three distinct functional projections ForceP, IntP, and FinP.

Do infants have abstract grammatical knowledge of word order at 17 months? Evidence from Mandarin Chinese.

We test the comprehension of transitive sentences in very young learners of Mandarin Chinese using a combination of the weird word order paradigm with the use of pseudo-verbs and the preferential looking paradigm, replicating the experiment of Franck et al. (2013) on French. Seventeen typically-developing Mandarin infants (mean age: 17.

Treatment of Pyonephritis Complicated by Septic Shock Using Extracorporeal Device Polymyxin B-Hemoperfusion.

Direct hemoperfusion using polymyxin B-immobilized fiber (PMX-DHP) is an established treatment method for septic shock caused by Gram-negative infections. We report one instance in which PMX-DHP therapy has been used successfully in a 33-year-old woman with septic shock from urosepsis. Although there is lack of recommendations in latest Surviving Sepsis Campaign Guidelines, evidence of PMX-DHP efficacy in this subset of patients is growing.

Correction to: Chitotriosidase: a biomarker of activity and severity in patients with sarcoidosis.

After publication of our article [1] the authors have notified us that one of the names has been incorrectly tagged.

Morphosyntactic weaknesses in Developmental Language Disorder: the role of structure and agreement configurations.

Agreement is a morphosyntactic dependency which is sensitive to the hierarchical structure of the clause and is constrained by the structural distance that separates the elements involved in this relation. In this paper we present two experiments, providing new evidence that Italian-speaking children with Developmental Language Disorder (DLD), as well as Typically Developing (TD) children, are sensitive to the same hierarchical and locality factors that characterise agreement in adult grammars. This sensitivity holds even though DLD children show accrued difficulties in more complex agreement configurations.

Chitotriosidase: a biomarker of activity and severity in patients with sarcoidosis.

Background: Serum chitotriosidase is a promising biomarker that has shown high specificity and sensitivity in patients with sarcoidosis. The aim of this study was to investigate correlations between serum chitotriosidase, clinical phenotypes, disease localizations and different radiological lung involvement and to identify clinical features associated with over-expression of chitotriosidase in a large cohort of sarcoidosis patients.

Methods: Chitotriosidase activity was evaluated in a population of 694 consecutive patients (males 39%, age 55.

Open pelvic fracture associated with lumbosacral dislocation and extensive perineal injury.

Open pelvic fractures are caused by high-energy trauma. Injuries to other organs are common and the mortality rate can be as high as 50%. Perineal injury is reported in 5% of open pelvic fractures.

Corrigendum: Task-dependency and structure-dependency in number interference effects in sentence comprehension.

[This corrects the article on p. 349 in vol. 6, PMID: 25914652.

Task-dependency and structure-dependency in number interference effects in sentence comprehension.

We report three experiments on French that explore number mismatch effects in intervention configurations in the comprehension of object A'-dependencies, relative clauses and questions. The study capitalizes on the finding of object attraction in sentence production, in which speakers sometimes erroneously produce a verb that agrees in number with a plural object in object relative clauses. Evidence points to the role of three critical constructs from formal syntax: intervention, intermediate traces and c-command (Franck et al.

Short stems in total hip replacement: current status and future.

Short stem hip implants have been introduced as a bone preserving surgery for younger and more active people undergoing hip arthroplasty. Although many short stems are now available, clinical results and long-term survival are controversial. The aim of this paper is to describe the features of the short stems and to analyse their clinical results and long-term survival.

Management of hemodynamically unstable pelvic trauma: results of the first Italian consensus conference (cooperative guidelines of the Italian Society of Surgery, the Italian Association of Hospital Surgeons, the Multi-specialist Italian Society of Young Surgeons, the Italian Society of Emergency Surgery and Trauma, the Italian Society of Anesthesia, Analgesia, Resuscitation and Intensive Care, the Italian Society of Orthopaedics and Traumatology, the Italian Society of Emergency Medicine, the Italian Society of Medical Radiology -Section of Vascular and Interventional Radiology- and the World Society of Emergency Surgery).

Hemodynamically Unstable Pelvic Trauma is a major problem in blunt traumatic injury. No cosensus has been reached in literature on the optimal treatment of this condition. We present the results of the First Italian Consensus Conference on Pelvic Trauma which took place in Bergamo on April 13 2013.

A person is not a number: discourse involvement in subject-verb agreement computation.

Agreement is a very important mechanism for language processing. Mainstream psycholinguistic research on subject-verb agreement processing has emphasized the purely formal and encapsulated nature of this phenomenon, positing an equivalent access to person and number features. However, person and number are intrinsically different, because person conveys extra-syntactic information concerning the participants in the speech act.

When persons disagree: an ERP study of Unagreement in Spanish.

Agreement is one of the main devices used by languages to signal grammatical relations. In this study, we investigated the neurophysiological processing correlates of subject-verb agreement in Spanish using Unagreement, a phenomenon characterized by a person mismatch between subject and verb that nonetheless produces a grammatical pattern. Unagreement was compared to well-formed sentences with full agreement, and ill-formed sentences with a person mismatch.

Sentence syntax and content in the human temporal lobe: an fMRI adaptation study in auditory and visual modalities.

Priming effects have been well documented in behavioral psycholinguistics experiments: The processing of a word or a sentence is typically facilitated when it shares lexico-semantic or syntactic features with a previously encountered stimulus. Here, we used fMRI priming to investigate which brain areas show adaptation to the repetition of a sentence's content or syntax. Participants read or listened to sentences organized in series which could or not share similar syntactic constructions and/or lexico-semantic content.