IFN-γ licenses normal and pathogenic ALPK1/TIFA pathway in human monocytes.

Alpha-kinase 1 (ALPK1) is an immune receptor sensing the bacterial nucleotide sugar ADP-heptose. ALPK1 phosphorylates TIFA leading to its oligomerization and downstream NF-κB activation. Specific mutations in are associated with an autoinflammatory syndrome termed ROSAH and with spiradenoma (skin cancers with sweat gland differentiation).

Aborted pancreatoduodenectomy after extensive vascular dissection in patients with pancreatic cancer: a word of caution.

Background: Difficulties in distinguishing fibrosis from tumor in borderline and locally advanced pancreatic adenocarcinoma (PDAC) justify surgical exploration to assess resectability. This issue is especially relevant after neoadjuvant treatments (NAT) prior to pancreaticoduodenectomy (PD) although outcomes of aborted PD are unknown.

Objectives: This study aimed to evaluate early outcomes after aborted PD in patients with PDAC.

Identification of protease-sensitive but not misfolding PNLIP variants in familial and hereditary pancreatitis.

Mutations in the PNLIP gene have recently been implicated in chronic pancreatitis. Several PNLIP missense variants have been reported to cause protein misfolding and endoplasmic reticulum stress although genetic evidence supporting their association with chronic pancreatitis is currently lacking. Protease-sensitive PNLIP missense variants have also been associated with early-onset chronic pancreatitis although the underlying pathological mechanism remains enigmatic.

Daily practices in chemotherapy for advanced gastric or gastroesophageal junction adenocarcinoma: METESTOMAC French prospective cohort.

Background: Around 50% of gastric cancers are diagnosed at an advanced stage. Several chemotherapy regimens are now internationally validated. Few data are available on the routine daily management of advanced gastric or gastroesophageal junction cancers.

Steroid hormone catabolites activate the pyrin inflammasome through a non-canonical mechanism.

The pyrin inflammasome acts as a guard of RhoA GTPases and is central to immune defenses against RhoA-manipulating pathogens. Pyrin activation proceeds in two steps. Yet, the second step is still poorly understood.

Extreme Hyperferritinemia: Causes and Prognosis.

The significance of extreme hyperferritinemia and its association with certain diagnoses and prognoses are not well characterized. We performed a retrospective analysis of adult patients with at least one total serum ferritin (TSF) measurement ≥ 5000 µg/L over 2 years, in three university hospitals. Conditions associated with hyperferritinemia were collected, and patients were classified into 10 etiological groups.

Evaluation of Glycosylated Ferritin in Adult-Onset Still's Disease and Differential Diagnoses.

Glycosylated ferritin (GF) has been reported as a good diagnostic biomarker for adult-onset Still’s disease (AOSD), but only a few studies have validated its performance. We performed a retrospective study of all adult patients with at least one GF measurement over a 2-year period in one hospital laboratory. The diagnosis of AOSD was based on the expert opinion of the treating physician and validated by two independent investigators.

The Prevalence of Social Withdrawal in Infants With Cleft Lip and Palate: The Feasibility of the Full and the Modified Versions of the Alarm Distress Baby Scale.

Background: Social withdrawal is a risk indicator for infant development with both organic and non-organic causes. Cleft lip and palate (CLP) impose a higher risk of physical and emotional distress in infants and alters parent-infant relationships. The ADBB scale is a screening tool to identify social withdrawal as a sign of distress in infants.

Infant Social Withdrawal Behavior: A Key for Adaptation in the Face of Relational Adversity.

As a result of evolution, human babies are born with outstanding abilities for human communication and cooperation. The other side of the coin is their great sensitivity to any clear and durable violation in their relationship with caregivers. Infant sustained social withdrawal behavior (ISSWB) was first described in infants who had been separated from their caregivers, as in Spitz's description of "hospitalism" and "anaclitic depression.

Functional Assessment of Disease-Associated Pyrin Variants.

The pyrin inflammasome detects effectors and toxins that inhibit RhoA GTPases and triggers inflammatory cytokines release and a fast cell death termed pyroptosis. Ancient plague pandemics in the Mediterranean basin have selected in the human population pyrin variants that can trigger an autoinflammatory disease termed familial Mediterranean fever (FMF). In addition, distinct mutations in MEFV, the gene encoding pyrin, cause a different rare autoinflammatory disease termed pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND).

The Effects of Prenatal Diagnosis on the Interaction of the Mother-Infant Dyad: A Longitudinal Study of Prenatal Care in the First Year of Life.

Introduction: Mother-child interactions during the first years of life have a significant impact on the emotional and cognitive development of the child. In this work, we study how a prenatal diagnosis of malformation may affect maternal representations and the quality of these early interactions. To this end, we conducted a longitudinal observational study of mother-child interactions from the gestational stage until the baby completed 12 months of age.

Longitudinal Human Milk miRNA Composition over the First 3 mo of Lactation in a Cohort of Healthy Mothers Delivering Term Infants.

Background: MicroRNAs (miRNAs) are small noncoding RNAs involved in posttranscriptional regulation. miRNAs can be secreted and found in many body fluids, and although they are particularly abundant in breastmilk, their functions remain elusive. Human milk (HM) miRNAs start to raise considerable interest, but a comprehensive understanding of the repertoire and expression profiles along lactation has not been well characterized.

Are colorectal cancer patients at risk for COVID-19 infection during the postoperative period? The Covid-GRECCAR study.

Introduction: During the COVID-19 pandemic, cancer patients have been regarded as having a high risk of severe events if they are infected with SARS-CoV-2, particularly those under medical or surgical treatment. The aim of this study was to assess the posttreatment risk of infection by SARS-CoV-2 in a population of patients operated on for colorectal cancer 3 months before the COVID-19 outbreak and who after hospitalization returned to an environment where the virus was circulating.

Materials And Methods: This French, multicenter cohort study included consecutive patients undergoing elective surgery for colorectal cancer between January 1 and March 31, 2020, at 19 GRECCAR hospitals.

Fast diagnostic test for familial Mediterranean fever based on a kinase inhibitor.

Background And Objective: Familial Mediterranean fever (FMF) is the most frequent hereditary autoinflammatory disease. Its diagnosis relies on a set of clinical criteria and a genetic confirmation on identification of biallelic pathogenic variants. encodes pyrin, an inflammasome sensor.

Delivery of hepato-pancreato-biliary surgery during the COVID-19 pandemic: an European-African Hepato-Pancreato-Biliary Association (E-AHPBA) cross-sectional survey.

Background: The extent of the COVID-19 pandemic and the resulting response has varied globally. The European and African Hepato-Pancreato-Biliary Association (E-AHPBA), the premier representative body for practicing HPB surgeons in Europe and Africa, conducted this survey to assess the impact of COVID-19 on HPB surgery.

Methods: An online survey was disseminated to all E-AHPBA members to assess the effects of the pandemic on unit capacity, management of HPB cancers, use of COVID-19 screening and other aspects of service delivery.

Strategies for liver transplantation during the SARS-CoV-2 outbreak: Preliminary experience from a single center in France.

Liver transplantation (LT) during the ongoing severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic is challenging given the urgent need to reallocate resources to other areas of patient care. Available guidelines recommend reorganizing transplant care, but data on clinical experience in the context of SARS-CoV-2 pandemic are scarce. Thus, we report strategies and preliminary results in LT during the peak of the SARS-CoV-2 pandemic from a single center in France.