2q33 Deletions Underlying Syndromic and Non-syndromic CTLA4 Deficiency.

Purpose: CTLA4 deficiency is an inborn error of immunity (IEI) due to heterozygosity for germline loss-of-function variants of the CTLA4 gene located on chromosome 2q33.2. CTLA4 deficiency underlies pleiotropic immune and lymphoproliferation-mediated features with incomplete penetrance.

The oculomotor cistern and pituitary adenomas: anatomical and clinical study.

Objective: The oculomotor cistern (OMC) is a meningeal cuff filled with CSF that contains the oculomotor nerve (cranial nerve [CN] III) at the level of the lateral wall of the cavernous sinus. Only a few studies have investigated the involvement of the OMC by pituitary adenomas (pituitary neuroendocrine tumors [PitNETs]), mainly with relatively small case series. The aim of this study was to perform a histomorphological description of the OMC and systematically analyze its involvement by PitNETs from radiological, clinical, and surgical perspectives.

Incidence, Risk Factors, and Long-Term Outcomes for Extubation Failure in ICU in Patients With Obesity: A Retrospective Analysis of a Multicenter Prospective Observational Study.

Background: To our knowledge, no large observational study has compared the incidence and risk factors for extubation failure within 48 h and during ICU stay in the same cohort of unselected critically ill patients with and without obesity.

Research Question: What are the incidence and risk factors of extubation failure in patients with and without obesity?

Study Design And Methods: In the prospective multicenter observational FREE-REA study in 26 ICUs, the primary objective was to compare the incidence of extubation failure within 48 h in patients with and without obesity. Secondary objectives were to describe and to identify the independent specific risk factors for extubation failure using first a logistic regression model and second a decision tree analysis.

Beneath HMGA2 alterations in pleomorphic adenomas: Pathological, immunohistochemical, and molecular insights.

Aims: Most salivary gland neoplasms are distinguished by specific recurrent gene fusions. Recently, a subset of pleomorphic adenomas (PAs) originated from the parotid gland harboring the HMGA2:WIF1 fusion was described with a canalicular adenoma-like morphology and a greater propensity for recurrence and carcinomatous transformation.

Methods And Results: This study delineates the clinicopathological attributes of 54 cases of PAs exhibiting HMGA2 alterations, predominantly characterized by the HMGA2:WIF1 fusion, alongside a comparative analysis of their morphological and immunohistochemical profiles.

Reference values for biochemical tests during pregnancy: analysis of the PRECAREbio Cohort and correlation with precarious status.

Objective: To establish normal current values of various biochemical parameters during pregnancy in the northern area of Paris (France) in a subgroup from the PreCARE cohort and to study their changes according to the term of pregnancy, and to determine the influence of maternal precarity.

Design: The PreCAREbio cohort of pregnant women was defined in the PreCARE study, a multicenter cohort study.

Setting: Participants completed detailed questionnaires at enrolment and immediately postpartum.

Femoro-axillary versus femoro-femoral veno-arterial extracorporeal membrane oxygenation for refractory cardiogenic shock: A monocentric retrospective study.

Rationale: For veno-arterial extracorporeal membrane oxygenation (ECMO), the femoral artery is the preferred cannulation site (femoro-femoral: Vf-Af). This results in retrograde aortic flow, which increases the left ventricular afterload and can lead to severe pulmonary edema and thrombosis of the cardiac chambers. Right axillary artery cannulation (femoral-axillary: Vf-Aa) provides partial anterograde aortic flow, which may prevent some complications.

Impact of sentinel lymph node mapping on survival in patients with high-risk endometrial cancer in the early stage: A matched cohort study.

Objective: The aim of this study was to compare patient survival using sentinel lymph node (SLN) procedure and pelvic lymphadenectomy for stating early-stage high risk endometrial cancer.

Methods: Patients who underwent surgery for early-stage high risk endometrial cancer between 2010 and 2017 were extracted from the incidence registry of the SEER program. We identified patients who underwent SLN mapping.

Understanding the management of pediatric spondylodiscitis based on existing literature; a systematic review.

Background: Spondylodiscitis (SD), a rare disease in children, poses diagnostic challenges due to non-specific presenting symptoms, scarcity in incidence, and difficulty expressing pain in non-verbal children.

Method: A comprehensive search was conducted on three databases, including PubMed/Medline, Web of Science, and Scopus until March 2023. The inclusion criteria were studies that investigated the clinical characteristics, treatment, and complications of children's spondylodiscitis.

Adipose tissue macrophage dysfunction is associated with a breach of vascular integrity in NASH.

Background & Aims: In non-alcoholic fatty liver disease (NAFLD), monocytes infiltrate visceral adipose tissue promoting local and hepatic inflammation. However, it remains unclear what drives inflammation and how the immune landscape in adipose tissue differs across the NAFLD severity spectrum. We aimed to assess adipose tissue macrophage (ATM) heterogeneity in a NAFLD cohort.

Micro- and macrocirculatory effects of norepinephrine on anaesthesia-induced hypotension: a prospective preliminary study.

Background: Intraoperative arterial hypotension (IOH) leads to increased postoperative morbidity. Norepinephrine is often use to treat IOH. The question regarding the mode of administration in either a bolus or continuous infusion remains unanswered.

Management of Adult Patients With Drug Reaction With Eosinophilia and Systemic Symptoms: A Delphi-Based International Consensus.

Importance: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international consensus on its severity assessment and treatment.

Objective: To reach an international, Delphi-based multinational expert consensus on the diagnostic workup, severity assessment, and treatment of patients with DRESS.

STAT expression and TFH1 cells in CVID granulomatosis and sarcoidosis: immunological and histopathological comparisons.

Granulomatous disease is a serious complication of common variable immunodeficiency (CVID-GD) that occurs in 8-22% of these patients and can mimic sarcoidosis, with which it shares certain clinical, biological, and radiological features. However, few studies to date have compared the two pathologies immunologically and histologically. Therefore, we analyzed the immunological-histological findings for different tissue samples from ten patients with CVID-GD and compared them to those of biopsy-proven sarcoidosis.

Practical diagnosis of cirrhosis in non-alcoholic fatty liver disease using currently available non-invasive fibrosis tests.

Unlike for advanced liver fibrosis, the practical rules for the early non-invasive diagnosis of cirrhosis in NAFLD remain not well defined. Here, we report the derivation and validation of a stepwise diagnostic algorithm in 1568 patients with NAFLD and liver biopsy coming from four independent cohorts. The study algorithm, using first the elastography-based tests Agile3+ and Agile4 and then the specialized blood tests FibroMeter and CirrhoMeter, provides stratification in four groups, the last of which is enriched in cirrhosis (71% prevalence in the validation set).

The ethics of induction of labor at 39 weeks in low-risk nulliparas in research and clinical practice.

The "A Randomized Trial of Induction Versus Expectant Management" trial (ARRIVE trial) published in 2018 suggested that induction of labor can be considered a "reasonable option" for low-risk nulliparous women at ≥39 weeks of gestation. The study results led some professional societies to endorse the option for elective induction of labor at 39 weeks of gestation in low-risk nulliparas, and this has begun to change obstetrical practice. The ARRIVE trial provided valuable information supporting the benefits of induction of labor; however, the trial is insufficient to serve as the primary justification for widespread elective induction of labor at 39 weeks of gestation in low-risk nulliparas because of concerns about external validity.

Cross-Comparison of Inflammatory Skin Disease Transcriptomics Identifies PTEN as a Pathogenic Disease Classifier in Cutaneous Lupus Erythematosus.

Tissue transcriptomics is used to uncover molecular dysregulations underlying diseases. However, the majority of transcriptomics studies focus on single diseases with limited relevance for understanding the molecular relationship between diseases or for identifying disease-specific markers. In this study, we used a normalization approach to compare gene expression across nine inflammatory skin diseases.

Prognostic value of complement serum C3 level and glomerular C3 deposits in anti-glomerular basement membrane disease.

Background And Objectives: Activation of the complement system is involved in the pathogenesis of anti-glomerular basement membrane (anti-GBM) disease. Glomerular deposits of complement 3 (C3) are often detected on kidney biopsies. The primary objective of this study was to analyze the prognostic value of the serum C3 level and the presence of C3 glomerular deposits in patients with anti-GBM disease.