Ecosystem-based assessment of a widespread Mediterranean marine habitat: The Coastal Detrital Bottoms, with a special focus on epibenthic assemblages

IntroductionCoastal detrital bottoms (CDB) are one of the most extensive habitats of the continental shelf worldwide, in the upper levels of the circalittoral zone. Hosting a diverse community structured by sediment grain size, trophic interactions and calcified organisms, CDB exhibit important ecological functions. In the Mediterranean Sea, CDB are constituted by recent elements partly provided by adjacent infralittoral and circalittoral ecosystems. Since the 2010s, the offshore extension of many Marine Protected Areas (MPAs) has resulted in the incorporation of vast areas of CDB, raising the issue of their management. The Marine Strategy Framework Directive (MSFD) has embraced the concept of an ecosystem-based approach involving taking into account the functioning of marine habitats and their related ecosystem services. The purpose of this paper is to propose an ecosystem-based quality index (EBQI) tested on CDB from the north-western Mediterranean Sea, focusing mainly on epibenthic assemblages.MethodsThe first step has been to define a conceptual model of the CDB functioning, including the main trophic compartments and their relative weighting, then to identify appropriate assessment methods and potential descriptors. Twenty-nine sites were sampled along the coast of Provence and French Riviera (Southern France). Study sites were chosen with a view to encompassing a wide range of hydrological conditions and human pressures.ResultsVery well-preserved sites were found in Provence in areas without trawling and terrigenous inputs, while impacted and low-ES sites were located in the vicinity of urbanized areas. The cover of rhodoliths characterizes the seascape and might be an indicator of the good ES of CDB and reduced human pressure. However, the absence of rhodoliths may also be induced by natural phenomena.DiscussionThe EBQI designed for CDB proved representative and useful for a functional assessment based on epibenthic assemblages. However, some descriptors have shown their limitations and should be further explored. We highlight here the priority of establishing an index corresponding to a societal demand (e.g., European Directives, Barcelona convention) as a basis for a broad and large-scale assessment, for practical reasons. We stress the need to better apprehend the role of the macro-infauna and to extend this index over a wider geographical scale

Bone Marrow Transplant

Mucopolysaccharidosis type I-H (MPS I-H) is a rare lysosomal storage disorder caused by α-L-Iduronidase deficiency. Early haematopoietic stem cell transplantation (HSCT) is the sole available therapeutic option to preserve neurocognitive functions. We report long-term follow-up (median 9 years, interquartile range 8-16.5) for 51 MPS I-H patients who underwent HSCT between 1986 and 2018 in France. 4 patients died from complications of HSCT and one from disease progression. Complete chimerism and normal α-L-Iduronidase activity were obtained in 84% and 71% of patients respectively. No difference of outcomes was observed between bone marrow and cord blood stem cell sources. All patients acquired independent walking and 91% and 78% acquired intelligible language or reading and writing. Intelligence Quotient evaluation (n = 23) showed that 69% had IQ ≥ 70 at last follow-up. 58% of patients had normal or remedial schooling and 62% of the 13 adults had good socio-professional insertion. Skeletal dysplasia as well as vision and hearing impairments progressed despite HSCT, with significant disability. These results provide a long-term assessment of HSCT efficacy in MPS I-H and could be useful in the evaluation of novel promising treatments such as gene therapy

Réinventer les facteurs humains aéronautiques en chirurgie cervico-faciale

International audienc

Respiratory polygraphy in subjects with bronchopulmonary dysplasia: a retrospective study

Periodic assessment of the need for oxygen supplementation and/or mechanical ventilation in children with severe bronchopulmonary dysplasia (BPD) is crucial. The aim of the study was to analyze the indications and results of respiratory polygraphies (RP) performed in preterm infants with BPD followed at a tertiary university hospital

Noninvasive Respiratory Support as an Alternative to Tracheostomy in Severe Laryngomalacia.

International audienceObjectives/hypothesis: To analyze the role of noninvasive respiratory support (NRS) as an alternative to tracheostomy in the management of severe laryngomalacia.Study design: We conducted a monocentric retrospective study in a tertiary pediatric care center.Methods: All children under the age of 3 years with severe laryngomalacia, treated between January 2014 and December 2019, were included. Patient demographics, medical history, nutrition, surgery, NRS, and outcome were reviewed. Predictors for NRS were analyzed.Results: One hundred and eighty-eight patients were included. Mean age was 4 ± 5 months and mean weight was 4,925 ± 1,933 g. An endoscopic bilateral supraglottoplasty was performed in 183 (97%) patients and successful in 159 (87%). NRS was initiated in 29 (15%) patients at a mean age of 3 ± 2 months (1-11 months): 15 (52%) patients were treated with NRS after surgical failure, 9 (31%) were treated with NRS initiated prior to surgery because of abnormal overnight gas exchange, and 5 (17%) were treated exclusively with NRS due to comorbidities contraindicating an endoscopic procedure. NRS was successfully performed in all patients with a mean duration of 6 ± 11 months. No patient required a tracheostomy. Univariate analysis identified the following predictors of NRS: neonatal respiratory distress (P = .003), neurological comorbidity (P < .001), associated laryngeal abnormality (P < .001), cardiac surgery (P = .039), surgical endoscopic revision (P = .007), and nutritional support (P < .001).Conclusion: NRS is a safe procedure, which may avoid a tracheostomy in severe laryngomalacia, in particular, in case of endoscopic surgery failure, respiratory failure before surgery, and/or severe co-morbidity.Level of evidence: 3 Laryngoscope, 2021

Laryngotracheoesophageal cleft, a rare differential diagnosis of esophageal atresia

International audienc

Endothelial CD34 expression and regulation of immune cell response in-vitro

Abstract Endothelial cells cover the lining of different blood vessels and lymph nodes, and have major functions including the transport of blood, vessel homeostasis, inflammatory responses, control of transendothelial migration of circulating cells into the tissues, and formation of new blood vessels. Therefore, understanding these cells is of major interest. The morphological features, phenotype and function of endothelial cells varies according to the vascular bed examined. The sialomucin, CD34, is widely used as an endothelial marker. However, CD34 is differentially expressed on endothelial cells in different organs and in pathological conditions. Little is known about regulation of endothelial CD34 expression or function. Expression of CD34 is also strongly regulated in-vitro in endothelial cell models, including human umbilical vein endothelial cells (HUVEC) and endothelial colony forming cells (ECFC). We have therefore analysed the expression and function of CD34 by comparing CD34high and CD34low endothelial cell subpopulations. Transcriptomic analysis showed that CD34 gene and protein expressions are highly correlated, that CD34high cells proliferate less but express higher levels of IL-33 and Angiopoietin 2, compared with CD34low cells. Higher secretion levels of IL-33 and Angiopoietin 2 by CD34high HUVECs was confirmed by ELISA. Finally, when endothelial cells were allowed to interact with peripheral blood mononuclear cells, CD34high endothelial cells activated stronger proliferation of regulatory T lymphocytes (Tregs) compared to CD34low cells whereas expansion of other CD4+-T cell subsets was equivalent. These results suggest that CD34 expression by endothelial cells in-vitro associates with their ability to proliferate and with an immunogenic ability that favours the tolerogenic response

Natural history of Myhre syndrome

International audienceAbstract Background Myhre syndrome (MS) is a rare genetic disease characterized by skeletal disorders, facial features and joint limitation, caused by a gain of function mutation in SMAD4 gene. The natural history of MS remains incompletely understood. Methods We recruited in a longitudinal retrospective study patients with molecular confirmed MS from the French reference center for rare skeletal dysplasia. We described natural history by chaining data from medical reports, clinical data warehouse, medical imaging and photographies. Results We included 12 patients. The median age was 22 years old (y/o). Intrauterine and postnatal growth retardation were consistently reported. In preschool age, neurodevelopment disorders were reported in 80% of children. Specifics facial and skeletal features, thickened skin and joint limitation occured mainly in school age children. The adolescence was marked by the occurrence of pulmonary arterial hypertension (PAH) and vascular stenosis. We reported for the first time recurrent strokes from the age of 26 y/o, caused by a moyamoya syndrome in one patient. Two patients died at late adolescence and in their 20 s respectively from PAH crises and mesenteric ischemia. Conclusion Myhre syndrome is a progressive disease with severe multisystemic impairement and life-threathning complication requiring multidisciplinary monitoring

COVID-19 and ENT Pediatric otolaryngology during the COVID-19 pandemic. Guidelines of the French Association of Pediatric Otorhinolaryngology (AFOP) and French Society of Otorhinolaryngology (SFORL)

Objective: Joint guidelines of the French Pediatric Otolaryngology Society (AFOP) and of the French Society of otorhinolaryngology–head and neck surgery (SFORL) on the management of paediatric otolaryngology patients in the context of the COVID-19 pandemic. Methods: A nation-wide workgroup drew guidelines based on clinical experience, national and local recommendations and scientific literature. Proposals may have to be updated on a day-to-day basis. Results: In children, incidence of symptomatic COVID-19 (1–5%) is low and of good prognosis. The indications for nasal flexible endoscopy should be drastically limited. If undertaken, full Personal Protective Equipment (PPE) including FFP2 masks are required, as well as use of a sheath. Saline nose wash done by caregivers other than parents at home should require PPE. Unless foreign body tracheobronchial aspiration is clinically obvious, CT-scan should be performed to confirm indication of endoscopy. Surgical indications should be limited to emergencies and to cases that cannot be delayed beyond 2 months (especially endonasal, endopharyngeal laryngo-tracheobronchial procedures). Postponement should ideally be a group decision and recorded as such in the medical file. Surgical techniques should be adapted to limit the risk of viral dissemination in the air, avoiding the use of drills, microdebriders, monopolar cautery or lasers. Continuous suction should be placed near the operating field. In case of confirmed Covid-19 cases, or suspected cases (or in some centres systematically), PPE with FFP2 mask should be worn by all staff members present in the operating room.SCOPUS: ar.jDecretOANoAutActifinfo:eu-repo/semantics/publishe