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.

Collagenous gastritis in children: A national cohort.

Background: Collagen gastritis is a rare disease that manifests in children mainly as isolated gastric involvement associated with martial deficiency anemia. There are no recommendations for the management and follow-up of these patients. We aimed to describe the clinical data, endoscopic findings, and treatments deployed in France's children with collagenous gastritis.

Ethylene plays a dual role in sex determination and fruit shape in cucurbits.

Shapes of vegetables and fruits are the result of adaptive evolution and human selection. Modules controlling organ shape have been identified. However, little is known about signals coordinating organ development and shape.

Health-related quality of life of food-allergic children compared with healthy controls and other diseases.

Background: Food allergy is a potentially life-threatening disease, affecting up to 10% of the pediatric population.

Objective: The aim of our study was to assess the health-related quality of life (HRQL) of food-allergic patients compared with the general population and patients with other chronic diseases with dietary or allergic burden, in a cross-sectional study.

Methods: We recruited patients aged 8-17 years diagnosed with food allergy and matched healthy controls recruited in schools.

Quantification of Chaotic Intrinsic Variability of Sea-Air CO Fluxes at Interannual Timescales.

Chaotic intrinsic variability (CIV) emerges spontaneously from nonlinear ocean dynamics even without any atmospheric variability. Eddy-permitting numerical simulations suggest that CIV is a significant contributor to the interannual to decadal variability of physical properties. Here we show from an ensemble of global ocean eddy-permitting simulations that large-scale interannual CIV propagates from physical properties to sea-air CO fluxes in areas of high mesoscale eddy activity (e.

A comprehensive quantification of global nitrous oxide sources and sinks.

Nitrous oxide (NO), like carbon dioxide, is a long-lived greenhouse gas that accumulates in the atmosphere. Over the past 150 years, increasing atmospheric NO concentrations have contributed to stratospheric ozone depletion and climate change, with the current rate of increase estimated at 2 per cent per decade. Existing national inventories do not provide a full picture of NO emissions, owing to their omission of natural sources and limitations in methodology for attributing anthropogenic sources.

Tracking Improvement in Simulated Marine Biogeochemistry Between CMIP5 and CMIP6.

Purpose Of Review: The changes or updates in ocean biogeochemistry component have been mapped between CMIP5 and CMIP6 model versions, and an assessment made of how far these have led to improvements in the simulated mean state of marine biogeochemical models within the current generation of Earth system models (ESMs).

Recent Findings: The representation of marine biogeochemistry has progressed within the current generation of Earth system models. However, it remains difficult to identify which model updates are responsible for a given improvement.

Decadal trends in the ocean carbon sink.

Measurements show large decadal variability in the rate of [Formula: see text] accumulation in the atmosphere that is not driven by [Formula: see text] emissions. The decade of the 1990s experienced enhanced carbon accumulation in the atmosphere relative to emissions, while in the 2000s, the atmospheric growth rate slowed, even though emissions grew rapidly. These variations are driven by natural sources and sinks of [Formula: see text] due to the ocean and the terrestrial biosphere.

Predictive Model for Gastrostomy Placement in Adolescents With Developmental Disabilities and Cerebral Palsy.

Background: Factors associated with gastrostomy placement in adolescents with developmental disabilities (DDs) and cerebral palsy (CP) are poorly investigated. We aimed to develop and validate a machine learning (ML) model for gastrostomy placement in adolescents with DDs and CP.

Methods: We performed a multinational, double-blinded, case-control study including 130 adolescents with severe DD and CP (72 males, 58 females; mean age 16 ± 2 years).

Arabidopsis thaliana plants challenged with uranium reveal new insights into iron and phosphate homeostasis.

Uranium (U) is a naturally occurring radionuclide that is toxic to plants. It is known to interfere with phosphate nutrition and to modify the expression of iron (Fe)-responsive genes. The transporters involved in the uptake of U from the environment are unknown.

Abnormal movements associated with oropharyngeal dysfunction in a child with Chiari I malformation.

Background: Chiari I malformations (CM I) are rare hindbrain herniations. Dysphagia and other oropharyngeal dysfunctions may be associated with CM I, but to our knowledge, no clinical presentation similar to ours has ever been reported. The purpose of this communication is to draw attention to a unique and atypical clinical presentation of a child with CM I.

Uranium perturbs signaling and iron uptake response in Arabidopsis thaliana roots.

Uranium is a natural element which is mainly redistributed in the environment due to human activity, including accidents and spillages. Plants may be useful in cleaning up after incidents, although little is yet known about the relationship between metal speciation and plant response. Here, J-Chess modeling was used to predict U speciation and exposure conditions affecting U bioavailability for plants.

Expression of SofLAC, a new laccase in sugarcane, restores lignin content but not S:G ratio of Arabidopsis lac17 mutant.

Lignin is a complex phenolic heteropolymer deposited in the secondarily thickened walls of specialized plant cells to provide strength for plants to stand upright and hydrophobicity to conducting cells for long-distance water transport. Although essential for plant growth and development, lignin is the major plant cell-wall component responsible for biomass recalcitrance to industrial processing. Peroxidases and laccases are generally thought to be responsible for lignin polymerization, but, given their broad substrate specificities and large gene families, specific isoforms involved in lignification are difficult to identify.

Drug safety of rosiglitazone and pioglitazone in France: a study using the French PharmacoVigilance database.

Background: Thiazolidinediones (TZDs), rosiglitazone (RGZ) and pioglitazone (PGZ) are widely used as hypoglycemic drugs in patients with type 2 diabetes mellitus. The aim of our study was to investigate the profile of adverse drug reactions (ADRs) related to TZDs and to investigate potential risk factors of these ADRs.

Methods: Type 2 diabetic patients were identified from the French Database of PharmacoVigilance (FPVD) between 2002 and 2006.

Disruption of LACCASE4 and 17 results in tissue-specific alterations to lignification of Arabidopsis thaliana stems.

Peroxidases have been shown to be involved in the polymerization of lignin precursors, but it remains unclear whether laccases (EC 1.10.3.

[Eosinophilic esophagitis. Clinical presentation, allergology and treatment: a series of 22 children].

Background And Aims: Eosinophilic esophagitis (EE) is a disorder characterized by severe eosinophilic infiltration of the esophagus, with dysphagia and gastroesophageal reflux disease (GERD) symptoms unresponsive to acid blockade therapy but responsive to the removal of dietary antigens. We report information relating to children diagnosed with EE in Nice, France, over a 6-year period.

Patients And Methods: We conducted a retrospective study between January 1, 2004, and July 31, 2009, evaluating all children diagnosed with EE.

[Neonatal screening for sickle cell disease at the Nice University Hospital: the last 8 years].

Introduction: Screening for sickle cell disease, the most common of recessive autosomic hemoglobin disorders, allows detection of sickle cell disease SCD (homozygous sickle cell disease, compound heterozygote SC, and S β-thalassemia) in a target population. Our objective was to evaluate its effectiveness at the Nice University Hospital.

Population And Methods: This prospective study was conducted between 1 January 2000 and 31 December 2008.

[Abdominal aortic injury in a child: seat-belt syndrome].

Traumatic injuries of the abdominal aorta are rare, especially in the pediatric population, but they must be sought when the clinical signs are associated with a severe traumatic context. We report the case of a 7-year-old child who presented with a partial transection of the abdominal aorta following a traffic accident. The optimal management is based on the detection of the less striking but suggestive clinical signs, such as a belt hematoma in the abdominal wall or a decreased pulse in the lower limbs, as well as a systematic precise X-ray examination, and an urgent surgical intervention.

Erythromycin as a prokinetic agent: risk factors.

Erythromycin (ER) is an antibiotic with prokinetic activity. This property has led to its clinical use to improve gastric emptying in patients with gastroparesis. Several papers have reported the effectiveness of ER in such patients; however few of these studies have been well-conducted methodologically with evaluation of clinical endpoints and their results are often contradictory.

[Prevalence of diabetes in france and drug use: study based on the French pharmacovigilance database].

Aim: The aim of this study was to investigate the use of the French Pharmacovigilance Database to estimate characteristics of drug utilization in specific diseases.

Materials And Method: We identified diabetic patients from the French Pharmacovigilance Database between 2002 and 2005. In this population, we studied demographic characteristics, and the patterns of drug use, particularly hypoglycemiant drug use and other drug exposure.

Changes in culturability and virulence of Salmonella typhimurium during long-term starvation under desiccating conditions.

The survival of Salmonella typhimurium under desiccation and starvation conditions commonly associated with farm buildings was investigated in a desiccation model system: filtration onto polycarbonate membranes placed in a sealed desiccator with 0.0067 g/m3 absolute humidity. Heterogeneities within bacterial populations in relation to time of desiccation were investigated on a single-cell basis by epifluorescence microscopy coupled with an image analysis system in conjunction with fluorescent dyes Chemchrome V6 and DAPI.