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• Abetalipoproteinemia (ABL) is a rare monogenic familial hypobetalipoproteinemia caused by bi-allelic variants in MTTP gene. • We identified four French-Canadian patients homozygous for the same MTTP variant. • The ABL carrier frequency in Saguenay-Lac-Saint-Jean is estimated at 1:203. • This represents the 2nd highest worldwide carrier estimates for ABL. • Screening for ABL should be considered in this population. Abetalipoproteinemia (ABL) is a rare recessive genetic disease caused by bi-allelic pathogenic variants in the microsomal triglyceride transfer protein (MTTP) gene. This disease is characterized by a deficiency in the secretion of apolipoprotein B-containing lipoproteins. Patients with ABL present with neurological, hematological, and gastrointestinal symptoms due to fat malabsorption and a deficiency in liposoluble vitamins. In this report, we present a total of four ABL cases, including three new cases, all originating from the same French-Canadian founder population in Saguenay-Lac-Saint-Jean, Québec, Canada. These individuals are homozygous for the same pathogenic variant in the MTTP gene (c.419dup, p.Asn140Lysfs*2). We found that this variant is more common than anticipated in this population, with an estimated carrier frequency of 1:203. Early diagnosis is essential to initiate treatment known to prevent complications associated with ABL. Population carrier screening or newborn screening for ABL should be considered in this French-Canadian founder population. [ABSTRACT FROM AUTHOR]

This article examines the architectural dimensions of the remise, a courtly ceremony that marked the moment when a royal bride departed her homeland to be given into her new husband's possession. Staged in frontier zones, this ritual was often facilitated by ephemeral structures such as bridges and pavilions that, as part of their ritual function, marked out firm boundaries where none existed before. This study focuses on the remise staged in 1745 on a disputed island, the Isle of Pheasants, for the marriage of Princess Maria Teresa Rafaela of Spain to the French dauphin Louis-Ferdinand. Using state papers, contracts, and ceremonial texts, the article reconstructs the ephemeral spaces in which the handover was transacted. In doing so, this study reveals both the architectural stage management of the Franco-Spanish relationship and, more broadly, the spatial and ritual practices that undergirded early modern European diplomacy. [ABSTRACT FROM AUTHOR]

Background Cervical artery dissection (CeAD) is a frequent manifestation of fibromuscular dysplasia (FMD). However, risk factors for CeAD are unknown. We investigated factors associated with CeAD in the ARCADIA (Assessment of Renal and Cervical Artery Dysplasia) registry. Methods and Results The ARCADIA registry includes women or men aged ≥18 years, with a diagnosis of renal, cervical, or intracranial artery FMD, who were prospectively recruited at 16 university hospitals in France and Belgium. Diagnosis of acute or past CeAD at inclusion was established on imaging according to standard diagnostic criteria. Associations between potential determinants and CeAD were assessed by logistic regression analyses. Among 469 patients (75 men) with FMD, 65 (13.9%) had CeAD. Patients with CeAD were younger, more likely to be men, have a history of migraine, and less likely to have a history of hypertension than patients without CeAD. In the multivariable analysis, male sex (odds ratio [OR], 2.66; 95% CI, 1.34-5.25), history of migraine (OR, 1.90; 95% CI, 1.06-3.39), age ≥50 years (OR, 0.41; 95% CI, 0.23-0.73), history of hypertension (OR, 0.35; 95% CI, 0.20-0.64), and involvement of ≥3 vascular beds (OR, 2.49; 95% CI, 1.15-5.40) were significantly associated with CeAD. To validate the association between CeAD and sex, we performed a systematic review. We collected additional data on sex from 2 published studies and unpublished data from the US Registry for Fibromuscular Dysplasia and the European/International FMD Registry. In the pooled analysis (289 CeAD, 1933 patients), male sex was significantly associated with CeAD (OR, 2.04; 95% CI, 1.41-2.95; I 2 =0%). Conclusions In patients with FMD, male sex and multisite involvement are associated with CeAD, in addition to other previously known risk factors. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT02884141.

Since 2016, the French Society of Hypertension has warned about the decline in the management of high blood pressure in France: stagnation, or even decreased number of people who know their blood pressure level, take a treatment and are controlled. These results are lower than those observed in many other countries. Blood pressure is measured with an old method in the doctor's office. Accepted, simple and cost-effective, this method is currently unavoidable for reasons of feasibility and social habit. It has been used in observational and intervention studies that are the basis of the medical reasoning for screening, treatment and drug control of hypertension. In practice, it is too often poorly applied and unpredictable. It is now necessary to measure blood pressure in mmHg using a validated oscillometric automatic device coupled to a specific upper arm cuff adapted to the arm circumferences for the diagnosis and monitoring of high blood pressure in the doctor's office and at home. The auscultatoric measurement is only recommended if any doubt about the reliability of the electronic measurement. Blood pressure measurement is basically performed on both arms to detect asymmetry and then on the arm with the highest blood pressure. It is performed in sitting or lying position after a few minutes of rest without speaking and without having smoked and then in standing position to diagnose orthostatic hypotension, especially in elderly, diabetic and multi-medicated subjects. The blood pressure measurement during the consultation must be repeated and include at least 3 consecutive measurements at one minute intervals. The average of the last 2 measurements determines the blood pressure level. It is recommended to perform BP measurements outside the medical environment for the diagnosis and monitoring of hypertension; Home BP measurement is preferred to ambulatory blood pressure measurement for practical reasons unless otherwise specified. The home blood pressure measurement should include three measurements in the morning at breakfast and three measurements in the evening before bedtime at one minute intervals for at least three days. Prior training must be provided. In treated hypertensive patients, a masked hypertension should be considered as an uncontrolled hypertension and antihypertensive therapy adapted accordingly. The measurement of central BP pressure (aorta) should be limited to clinical research., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Catch crops can effectively decrease nitrate leaching in arable cropping systems but their long-term impacts on nitrogen mineralization are not well known. This study quantified the effects of continuous catch crops on net N mineralization, crop N uptake, crop N use efficiency and N leaching in three long-term (13-17 years) field experiments in northern France. Mustard was grown every year at Boigneville, radish every year at Thibie and ryegrass every 2 years at Kerlavic. The mean N content of catch crop residues at these sites was 33, 36 and 35 kg ha yr and their mean C:N ratio was 13, 17 and 28, respectively. Net mineralization was calculated with a mass balance of soil mineral N using measured inputs and outputs. Catch crop establishment enhanced annual mineralization by on average 26, 18 and 9 kg N ha yr respectively during the 13-17 year period. The difference in mineralization rate between catch crop and control treatments (extra mineralization) was positive from the first year at Boigneville, whereas it was negative or nil during the first 3-5 years at Thibie and Kerlavic. At the latter sites, the extra mineralization rate increased significantly over time at a rate of 2.0 and 2.6 kg N ha yr. At the end of the experiment, cumulative extra mineralization represented 72%, 60% and 23% of the total N added by catch crop residues at Boigneville, Thibie and Kerlavic, respectively. Repeated catch crops significantly increased N uptake and N use efficiency by main crops at Kerlavic and Thibie, but not at Boigneville. The efficiency of catch crops in reducing N leaching persisted over the years at all sites. A model simulating C and N dynamics during catch crop decomposition was able to reproduce the pattern of extra N mineralization kinetics with the various catch crop species, but underestimated the range of variation between sites. Better predictions were obtained when C or N inputs due to catch crops were increased by 10-57%, suggesting that the actual inputs could be markedly greater than those measured in catch crop residues. According to the model, the C:N ratio of catch crop residues largely explained differences in mineralization due to different catch crop species. [ABSTRACT FROM AUTHOR]

While "natural history" is practically synonymous with the name of Buffon, the term itself has been otherwise overlooked by historians of science. This essay attempts to address this omission by investigating the meanings of "physique," "natural philosophy," and "history," among other terms, with the purpose of understanding Buffon's actual objectives. It also shows that Buffon never claimed to be a Newtonian and should not be considered as such; the goal is to provide a historical analysis that resituates Buffon's thought within his own era. This is done, primarily, by eschewing the often-studied question of time in Buffon. Instead, this study examines the nontemporal meanings of the word "history" within the naturalist's theory and method. The title of his Natural History is examined both as an indicator of the kind of science that Buffon was hoping to achieve and as a source of great misinterpretation among his peers. Unlike Buffon, many of his contemporaries actually envisioned the study of nature from a Baconian perspective where history was restricted to the mere collection of facts and where philosophy, which was the implicit and ultimate goal of studying nature, was seen, at least for the present, as unrealizable. Buffon confronts this tendency insofar as his Histoire naturelle claims to be the real physique that, along with describing nature, also sought to identify general laws and provide clear insight into what true knowledge of nature is or should be. According to Buffon, history (both natural and civil) is not analogous to mathematics; it is a nonmathematical method whose scope encompasses both nature and society. This methodological stance gives rise to the "physicization" of certain moral concepts--a gesture that was interpreted by his contemporaries as Epicurean and atheist. In addition, Buffon reduces a number of metaphysically tainted historical concepts (e.g., antediluvian monuments) to objects of physical analysis, thereby confronting the very foundation of natural theology. In Buffon, as this essay makes clear, natural history is paving the way for a new physique (science of natural beings), independent from mathematics and from God, that treats naturalia in a philosophical and "historical" manner that is not necessarily "temporal." [ABSTRACT FROM AUTHOR]

The effect of botanical composition and nutrient availability on the relative allocation of biomass to stems and leaves in a permanent upland pasture in the central Pyrenees was assessed. Six short-term and medium-term fertilizer treatments (nitrogen and phosphorus) were applied to a meadow to create large differences in the proportions of the different species and a wide range of herbage nutrient status. The above-ground herbage dry-matter components (green leaves, sheaths and stems for grasses and dicotyledons, and senescent material) were measured for the first growth cycle. The leaf mass depended mainly on the herbage N status, whereas the stem mass depended both on the botanical composition and on the herbage N and P status. During spring growth, the proportion of leaves in the above-ground dry matter decreased faster in plots that had the highest nutrient status or that were composed of species characteristic of nutrient-rich (Festuca-poor) habitats. Application of fertilizer decreased the proportion of leaves both for grasses and dicotyledons, but to a larger extent for grasses. There was a single relationship between the proportion of leaves in the above-ground dry matter and the total mass of above-ground dry matter, irrespective of the sampling date, the botanical composition or the herbage nutrient status. This statistical relationship resulted from (i) a faster increase in stem mass than leaf mass for a given botanical composition when the herbage nutritional status increased, (ii) a greater stem mass when the sward was composed of species usually found in nutrient-rich habitats. [ABSTRACT FROM AUTHOR]

Objective: An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of tricuspid regurgitation (VTR) and right-sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3-year followup of patients from the same cohort (the ItinérAIR-Sclérodermie Study)., Methods: Patients with SSc and without evidence of PAH underwent evaluation for dyspnea and VTR at study entry and during subsequent visits. Patients in whom PAH was suspected because of a VTR of 2.8-3.0 meters/second and unexplained dyspnea or a VTR of >3.0 meters/second underwent RHC to confirm the diagnosis., Results: A total of 384 patients were followed up for a mean+/-SD of 41.03+/-5.66 months (median 40.92 months). At baseline, 86.7% of the patients were women, and the mean+/-SD age of the patients was 53.1+/-12.0 years. The mean+/-SD duration of SSc at study entry was 8.7+/-7.6 years. After RHC, PAH was diagnosed in 8 patients, postcapillary PH in 8 patients, and PH associated with severe pulmonary fibrosis in 2 patients. The incidence of PAH was estimated to be 0.61 cases per 100 patient-years. Two patients who exhibited a mean pulmonary artery pressure of 20-25 mm Hg at baseline subsequently developed PAH., Conclusion: The estimated incidence of PAH among patients with SSc was 0.61 cases per 100 patient-years. The high incidence of postcapillary PH highlights the value of RHC in investigating suspected PAH.

Objectives: This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline., Methods: At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan-Meier method. Multivariate survival analyses were conducted using the Cox model., Results: In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 +/- 13.0 years and mean duration of SSc of 8.8 +/- 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality., Conclusion: This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.