Mutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans.

IKKα, encoded by CHUK, is crucial in the non-canonical NF-κB pathway and part of the IKK complex activating the canonical pathway alongside IKKβ. The absence of IKKα causes fetal encasement syndrome in humans, fatal in utero, while an impaired IKKα-NIK interaction was reported in a single patient and causes combined immunodeficiency. Here, we describe compound heterozygous variants in the kinase domain of IKKα in a female patient with hypogammaglobulinemia, recurrent lung infections, and Hay-Wells syndrome-like features.

Ultrasound-guided needle positioning confirmation with injection of saline solution for nodal dynamic contrast-enhanced MR-lymphangiography in pediatric patients.

Purpose: To assess the success rate of confirmation of ultrasound-guided intranodal needle positioning by saline injection for dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) in pediatric patients.

Material And Methods: Data from children undergoing nodal DCMRL after ultrasound-guided needle positioning into inguinal lymph nodes and validation of the needle position by injection of plain saline solution between 05/2020 and 12/2022 were reviewed. On injection of saline solution, adequate needle position was confirmed by lymph node distension without leakage.

sp. nov., a novel endophytic bacterium with plant growth-promoting potential, isolated from root nodules of in Northwestern Algeria.

A thorough polyphasic taxonomic study, integrating genome-based taxonomic approaches, was carried out to characterize the RB5 strain isolated from root nodules of growing on the coastal dunes of Bousfer Beach (Oran, Algeria). The 16S rRNA gene sequence analysis revealed that strain RB5 had the highest similarity to LMG27940 (98.94%) and IzPS32d (98.

A noncanonical-GPRC5A signaling regulates keratinocyte adhesion and migration by nuclear translocation.

G-Protein Coupled Receptor, Class C, Group 5, Member A (GPRC5A) has been extensively studied in lung and various epithelial cancers. Nevertheless, its role in the skin remains to be elucidated. In this study, we sought to investigate the function of this receptor in skin biology.

Literature review of pediatric robotic surgery in Spain.

Introduction: Robotic surgery has revolutionized surgical practice globally since the first Da Vinci device was launched in 2000. Adoption in Spain has been more moderate than in other countries, especially in the field of pediatric surgery, which represents less than 0.5% of all robotic procedures.

The role of intramolecular hydrogen bonding in photoelectron circular dichroism: the diastereoisomers of 1-amino-2-indanol.

(1,2)--Aminoindanol and (1,2)--aminoindanol, denoted as -AI and -AI, are diastereoisomer aromatic aminoalcohols differing by the presence of a weak intramolecular hydrogen bond in -AI, which is absent in -AI. They also differ by the number of conformers under supersonic jet conditions, one for -AI and two for -AI. One-photon and resonance-enhanced two-photon photoelectron circular dichroism (PECD) spectra are obtained for the two molecules.

Modeling plant phenotypic plasticity and its underlying genetic architecture: a comparative study.

Phenotypic plasticity can contribute to crop adaptation to challenging environments. Plasticity indices are potentially useful to identify the genetic basis of crop phenotypic plasticity. Numerous methods exist to measure phenotypic plasticity.

Impact of fluorination on membrane-protein stabilization and extraction by lactobionamide detergents.

We report the synthesis of a series of detergents with a lactobionamide polar head group and a tail containing four to seven perfluorinated carbon atoms. Critical micellar concentrations (CMCs) were determined using isothermal titration calorimetry (ITC) and surface tension (SFT) measurements, showing a progressive decrease from 27 mM to about 0.2 mM across the series.

Physical Prehabilitation for Older Patients with Cancer before Complex Medical-Surgical Interventions: An Umbrella Review.

Prehabilitation has become a field of increasing interest over recent decades. However, few studies specifically investigated prehabilitation for older patients with cancer. The objective of this umbrella review was to summarize evidence on prehabilitation programs to identify the physical interventions that may be applied with benefit to older cancer patients who will undergo complex medical-surgical procedures.

Clinical Trial: Study to Investigate the Efficacy and Safety of the Alpha-2-Delta Ligand PD-217,014 in Patients With Irritable Bowel Syndrome.

Introduction: Despite the emergence of drugs to treat irritable bowel syndrome (IBS), improving abdominal pain can still be challenging. αδ ligands, such as gabapentin and pregabalin, are sometimes used off-label to tackle this problem. However, evidence for efficacy is limited, and no large-scale studies have been published.

Transmastoid superior semicircular canal dehiscence plugging: VHIT findings.

Background: Superior canal dehiscence syndrome (Minor's syndrome) is a condition characterized by a bony defect in the superior semicircular canal (SSCC), with treatment primarily being surgical, notably through plugging of SSCC.

Aims/objectives: To examine the clinical outcome and postoperative VHIT findings after transmastoid plugging of the SSCC.

Materials And Methods: Patients having a superior semicircular canal dehiscence (SSCCD) syndrome with debilitating symptoms who underwent a plugging of the SSCC a transmastoid approach were included.

Tackling Hominin Tickling: Bonobos Share the Social Features and Developmental Dynamics of Play Tickling With Humans.

It is under debate whether intersubjectivity-the capacity to experience a sense of togetherness around an action-is unique to humans. In humans, heavy tickling-a repeated body probing play that causes an automatic response including uncontrollable laughter (gargalesis)-has been linked to the emergence of intersubjectivity as it is aimed at making others laugh (self-generated responses are inhibited), it is often asymmetrical (older to younger subjects), and it elicits agent-dependent responses (pleasant/unpleasant depending on social bond). Intraspecific tickling and the related gargalesis response have been reported in humans, chimpanzees, and anecdotally in other great apes, potentially setting the line between hominids and other anthropoids.

Primary Effusion Lymphoma Prognostic Score (PEL-PS): A Validated International Prognostic Score in HIV-Associated Primary Effusion Lymphoma.

Primary effusion lymphoma (PEL) is an HIV-associated B-cell non-Hodgkin lymphoma (NHL) caused by Kaposi sarcoma herpesvirus (KSHV). There is no validated prognostic model in PEL, and prognosis is thought to be poor compared to other HIV-associated NHL. We derived the PEL-Prognostic score (PEL-PS) from an international real-world training set of 59 patients with HIV-associated PEL who received first-line anthracycline-containing chemotherapy from the HIV and AIDS Malignancy Branch at the National Cancer Institute (NCI) in the United States and the National Center for HIV Malignancy at the Chelsea and Westminster Hospital (CWH) in England from 2000 to 2022.

Evaluation of the predictive value of CSF-restricted oligoclonal bands on residual disability and risk of relapse in adult patients with MOGAD: MOGADOC study.

Background: The clinical course of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is variable. However, robust markers of poor outcome and/or relapse risk are still missing.

Objective: To evaluate the frequency of cerebrospinal fluid-restricted oligoclonal bands (CSF-OCB) in a national cohort of adult MOGAD patients and to assess their prognostic value for the risk of relapse and severity.

Radioactive contamination of southeast Abai oblast, Kazakhstan, from the Chinese nuclear weapons testing program at Lop Nor: an analytical review.

Between 1949 and 1962 the Soviet Union performed atmospheric tests of nuclear weapons at the Semipalatinsk nuclear test site (SNTS) in Kazakhstan, resulting in widespread contamination of the surrounding region with radioactive fallout. Settlements in the southeast Abai oblast of Kazakhstan, close to the border with China, are not thought to have received significant fallout from the SNTS. There is, however, evidence that the study area, including Makanchi, Urdzhar and Taskesken villages, was contaminated by atmospheric nuclear tests performed by China at the Lop Nor NTS between 1964 and 1980.

Polarization resolved second harmonic scattering of neat water in the right angle and forward scattering geometries.

Liquid water still remains an ubiquitous liquid whose molecular organization requires careful investigation. In this work, we present a study of the second harmonic scattering (SHS) intensity for two different scattering angles, namely, the forward and the right angle geometries. This method performed at optical wavelengths is indeed selective toward long correlation lengths.

Exploring the equilibrium and non-equilibrium properties of a cooperative trinuclear spin-crossover chain: The role of elastic frustration.

Among the large family of spin-crossover (SCO) solids, recent investigations focused on polynuclear SCO materials, whose specific molecular configurations allow the presence of multi-step transitions and elastic frustration. In this contribution, we develop the first elastic modeling of thermal and dynamical properties of trinuclear SCO solids. For that, we study a finite SCO open chain constituted of successive elastically coupled trinuclear (A=B=C) blocks, in which each site (A, B, and C) may occupy two electronic configurations, namely, low-spin (LS) and high-spin (HS) states, accompanied with structural changes.

Cardiovascular diseases, prevention and management of complications in older adults and frail patients treated for elective or post-traumatic hip orthopaedic interventions.

Due to the aging population, focusing on healthy aging has become a global priority. Cardiovascular diseases (CVDs) and frailty, characterized by increased vulnerability to adverse stress and health events, interact synergistically in advanced age. In older adults, hip fractures are a frequent dramatic "life-transition" event.

The Predictive Value of Criminogenic Needs on Institutional Misconduct in a Dutch Forensic Hospital.

According to the Risk-Need-Responsivity model, criminogenic needs are important in predicting violent behavior. Eight criminogenic needs are considered strong predictors: history of antisocial behavior, antisocial personality traits, criminal attitudes, criminal associates, substance abuse, family problems, poor work performance, and lack of involvement in prosocial leisure/recreation activities. The purpose of the current study was to examine whether seven criminogenic needs predict institutional misconduct in the first year of admission of Dutch patients who were admitted to a forensic hospital.

Prevalence of pathogenic variants of inborn errors of immunity in critically ill children admitted to the pediatric intensive care unit for sepsis: A Moroccan cohort study.

Introduction: Pediatric sepsis remains a leading cause of morbidity and mortality in Africa. Nearly half of pediatric sepsis deaths occur in previously healthy children. The role of inborn errors of immunity (IEI) in susceptibility to sepsis is yet to be identified and their prevalence amongst previously healthy children admitted to the pediatric intensive care unit (PICU) is unclear.

High performance COVID-19 screening using machine learning.

Since the World Health Organization declared the Coronavirus Disease 2019 (COVID-19) pandemic as an international concern of public health emergency in the early 2020, several strategies have been initiated in many countries to prevent healthcare services breakdown and collapse of healthcare structures. The most important strategy was the increased testing, diagnosis, isolation, contact tracing to identify, quarantine and test close contacts. In this context, finding a rapid, reliable and affordable tool for COVID-19 screening was the main challenge to address the pandemic.

The role of multi-modality imaging for the assessment of left atrium and left atrial appendage. A clinical consensus statement of the European Association of Cardiovascular Imaging (EACVI), European Heart Rhythm Association (EHRA) of the European Society of Cardiology (ESC).

Structural, architectural, contractile or electrophysiological alterations may occur in the left atrium (LA). The concept of LA cardiopathy is supported by accumulating scientific evidence demonstrating that LA remodeling has become a cornerstone diagnostic and prognostic marker. The structure and the function of LA and left atrial appendage (LAA) which is an integral part of the LA, are key elements for a better understanding of multiple clinical conditions, most notably atrial fibrillation (AF), cardioembolism, heart failure and mitral valve diseases.

How tailor-made copolymers can control the structure and properties of hybrid nanomaterials: the case of polyionic complexes.

Hybrid polyionic complexes (HPICs) are colloidal structures with a charged core rich in metal ions and a neutral hydrophilic corona. Their properties, whether as reservoirs or catalysts, depend on the accessibility and environment of the metal ions. This study demonstrates that modifying the coordination sphere of these ions can tune the properties of HPICs by altering the composition of the complexing block or varying formulation conditions.