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Introduction: Dental rehabilitation of patients with alveolar-dental cleft sequelae is a crucial issue in the final stages of functional and aesthetic management of these patients. The objectives of this study are to establish the success of implant-supported rehabilitations for patients with alveolar dental cleft sequelae followed in the Department of Maxillofacial Surgery and Stomatology of Lille University Hospital and the reasons for not using this type of rehabilitation for others., Materials and Method: Patients treated between January 2009 and December 2018 with implant-supported prostheses at an alveolar dental cleft site were included. Dental implants were placed after a complementary alveolar bone graft. Clinical and radiological criteria regarding periodontal, occlusal and prosthetic status were studied., Results: A total of 12 implants in 8 patients were placed. One of these implants was lost, resulting in an implant survival rate of 91.7%. All patients were able to benefit from functional rehabilitation., Discussion: The present results demonstrate the interest and reliability of implant rehabilitation in these patients. They furthermore highlight financial factors as a barrier to using this solution among the majority of patients in care., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Global health priority setting increasingly focuses on understanding the functioning of health systems and on how they can be strengthened. Beyond vertical programs, health systems research should examine system-wide delivery platforms (e.g. health facilities) and operational elements (e.g. supply chains) as primary units of study and evaluation., Methods: We use dynamical system methods to develop a simple analytical model for the supply chain of a low-income country's health system. In doing so, we emphasize the dynamic links that integrate the supply chain within other elements of the health system; and we examine how the evolution over time of such connections would affect drug delivery, following the implementation of selected interventions (e.g. enhancing road networks, expanding workforce). We also test feedback loops and forecasts to study the potential impact of setting up a digital system for tracking drug delivery to prevent drug stockout and expiration., Results: Numerical simulations that capture a range of supply chain scenarios demonstrate the impact of different health system strengthening interventions on drug stock levels within health facilities. Our mathematical modeling also points to how implementing a digital drug tracking system could help anticipate and prevent drug stockout and expiration., Conclusion: Our mathematical model of drug supply chain delivery represents an important component toward the development of comprehensive quantitative frameworks that aim at describing health systems as complex dynamical systems. Such models can help predict how investments in system-wide interventions, like strengthening drug supply chains in low-income settings, may improve population health outcomes.

Noonan syndrome (NS) is a relatively common congenital multiple-anomaly syndrome, resembling Turner syndrome, but without chromosomal anomaly. Besides the unusual facies, the maxillofacial and dental features of patients with NS are not well-summarized in the literature. The aim of this study was to describe these features and propose specific treatment guidelines for practitioners involved in oral and maxillofacial care. A retrospective multicentric study was conducted of 14 patients who were referred for NS screening. In total, 10 patients were found to carry a mutation involved in NS or NS-related disorders. Fifty percent of the mutations affected PTPN11. All patients presented with the typical extraoral features, such as macrocephaly, hypertelorism, ptosis, triangular face shape and ear dystrophy. Intraoral manifestations, including malocclusion (maxillary transversal deficiency, crossbite, anterior open-bite and class II malocclusion), dental anomalies (delayed eruption, agenesis and dystrophy, odontoma) and radiologic jaw lesions were identified in five out of 10 patients. These findings were searched in a review of the literature to obtain a comprehensive description of oral and maxillofacial features in patients with NS. The proposed treatment guidelines emphasize frequent coagulation anomalies that need to be considered prior to surgery. Early dental assessment and yearly follow-up with oral prophylaxis are recommended. Orthodontics and orthognathic surgery are also of primary importance in the management of NS patients., (Copyright © 2020 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Unilateral condylar hyperplasia (UCH) is characterized by an overgrowth of the mandibular condyle responsible for a facial and dental asymmetry associated with temporomandibular joint function and maxillary growth consequences. The diagnosis is based on a body of clinical, radiological and histological arguments. A 38 years old woman with a reactivation of UCH after a latency period of 16 years following an orthognathic surgery performed for facial asymmetry normalization is presented. She was addressed to our department for a facial progressive asymmetry relapse and dental prosthetic consequences.The radiological images and the planar scintigraphy combined with single-photon emission computed tomography scans showed an active left unilateral condylar hyperplasia. A left proportional condylectomy was performed.The case presented highlights the possibility for the UCH to be reactivated after a long period of latency, leading to a relapse of the occlusal and facial disorders and so advocates the need for first condylectomy or at least a long-term follow-up if condylectomy is not performed as a first-line treatment.

One of the most common causes of short stature is a defect of the short stature homeobox-containing (SHOX) gene, which is located in pseudoautosomal region 1 on the distal end of the short arm of chromosomes Xp22.33 and Yp11.32. More than 300 different mutations in the SHOX gene responsible for short stature syndrome have been described. The phenotypic expression of SHOX haploinsufficiency is remarkably varied. The 3 typical clinical presentations, from least to most severe, are idiopathic short stature without skeletal malformations, Leri-Weill dyschondrosteosis (LWD), and Langer mesomelic dysplasia, which is believed to represent the homozygous form of LWD. Despite a higher prevalence in women, suggesting the potentiating action of high estrogen levels on the effects of SHOX deficiency, the syndrome was initially believed to have an autosomal pattern of inheritance. In reality, heterozygous SHOX mutations can be transferred from the Y to the X chromosome and vice versa. This phenomenon is called "the jumping SHOX gene" and corresponds to a pseudoautosomal dominant inheritance. LWD is characterized by mesomelic short stature and Madelung deformity defined by an upward and medial displacement of the radial joint surface, which restricts range of motion. Less specific dysmorphic signs associated with LWD, such as short hands and feet, scoliosis, or muscular hypertrophy, have been described. When reviewing the dental and maxillofacial signs, only limited and summary data (micrognathia and high arched palate) have been published in the literature. This report presents a case of LWD that highlights many other noteworthy dental and maxillofacial signs that are important to clearly identify and appropriately treat., (Copyright © 2018 American Association of Oral and Maxillofacial Surgeons. All rights reserved.)

This study investigates morphological changes in pulp chambers of living and archaeological individuals with past vitamin D deficiency. Living individuals (n=29), four with detailed medical and dental records and three groups of archaeological individuals (n=25) were radiographed; selected individuals were further evaluated histologically for the presence of incremental interglobular dentin (IIGD), indicative of deficiency (28 living; 17 archaeological). Measurements of pulp horns/chambers from radiographs were conducted to quantify morphological observations. One group had clear skeletal evidence of rickets from St. Matthew, Quebec (n=1) and St. Jacques, France (n=4); a second group had slight skeletal indicators from Bastion des Ursulines, Quebec (n=6); and a third group lacked both skeletal and radiological evidence of deficiency from St. Antoine (n=6) and Pointe-aux-Trembles (n=4). Results showed archaeological individuals with clear and slight skeletal evidence of past deficiency displayed constricted or chair shaped pulp horns. Living individuals with deficiency exhibited similar pulp chamber morphology. Radiographic pulp horn/chamber measurements corroborated morphological findings and significant differences were found in pulp horn/chamber measurements between those with and without deficiency. Results suggest that radiograph assessment of teeth can be used as a screening technique to elucidate patterns of deficiency and select individuals for microCT or histological assessment., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: Dagfinn Aarksog first described faciodigitogenital syndrome in 1970. Its inheritance is X linked and autosomally recessive. Currently, the diagnosis of Aarskog-Scott syndrome (ASS) is based on clinical dysmorphologic findings and can be supported by genetic examination., Report of Cases: This report describes 3 brothers already diagnosed with ASS who were referred for examination of oral and maxillofacial malformations associated with ASS. They presented classic features of ASS, such as digital and genital (shawl scrotum) anomalies. More specifically, in terms of orbitopalpebral malformations, they showed marked ptosis with hypertelorism and antimongoloid palpebral fissure that gave them the characteristic facies. Concerning their oral and maxillofacial malformations, they had dental and skeletal major discrepancies and some dental agenesia., Discussion and Conclusion: ASS is a rare X-linked syndrome composed of numerous morphologic facial, digital, and genital anomalies. The diagnosis is established genetically with the FGD1 mutation but there is no phenotypic and genotypic correlation with FGD1 mutations. Concerning maxillofacial malformations, maxillary and mandibular hypoplasia with jaw discrepancies can be found, as can teeth anomalies. It seems that these anomalies are widely underestimated., (Copyright © 2018 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Trauma remains a leading cause of death worldwide. Hemorrhagic shock and direct injury to vital organs are responsible for early mortality whereas most delayed deaths are secondary to complex pathophysiological processes. These processes result from imbalanced systemic reactions to the multiple aggressions associated with trauma. Trauma results in the uncontrolled local and systemic release of endogenous mediators acting as danger signals [damage-associated molecular patterns (DAMPs)]. Their recognition by the innate immune system triggers a pro-inflammatory immune response paradoxically associated with concomitant immunosuppression. These responses, ranging in intensity from inappropriate to overwhelming, promote the propagation of injuries to remote organs, leading to multiple organ failure and death. Some of the numerous DAMPs released after trauma trigger the assembly of intracellular multiprotein complexes named inflammasomes. Once activated by a ligand, inflammasomes lead to the activation of a caspase. Activated caspases allow the release of mature forms of interleukin-1β and interleukin-18 and trigger a specific pro-inflammatory cell death termed pyroptosis. Accumulating data suggest that inflammasomes, mainly NLRP3, NLRP1, and AIM2, are involved in the generation of tissue damage and immune dysfunction after trauma. Following trauma-induced DAMP(s) recognition, inflammasomes participate in multiple ways in the development of exaggerated systemic and organ-specific inflammatory response, contributing to organ damage. Inflammasomes are involved in the innate responses to traumatic brain injury and contribute to the development of acute respiratory distress syndrome. Inflammasomes may also play a role in post-trauma immunosuppression mediated by dysregulated monocyte functions. Characterizing the involvement of inflammasomes in the pathogenesis of post-trauma syndrome is a key issue as they may be potential therapeutic targets. This review summarizes the current knowledge on the roles of inflammasomes in trauma.

Background: There is increasing evidence that traces of hazardous drugs occur in the urine of health care professionals who are exposed to these drugs., Objective: To review the scientific literature regarding urinary monitoring of health care professionals exposed to antineoplastic drugs through their work., Data Sources: A search of PubMed using the Medical Subject Headings 'occupational exposure' and 'antineoplastic agents' and of Google Scholar using the terms 'antineoplastic', 'urine', and 'occupational exposure'., Study Selection and Data Extraction: The analysis covered all articles in English or French pertaining to health care professionals exposed to hazardous drugs in the workplace, published from January 1, 2010, to December 31, 2015. Articles that did not discuss the results of urine tests and those concerning veterinarians, as well as literature reviews, editorials, letters to the editor, and conference abstracts, were excluded., Data Synthesis: Twenty-four articles were retained. The studies were conducted in 52 health care institutions in 7 countries. They included 826 workers exposed to hazardous drugs and 175 controls, specifically nurses ( n = 16 studies), pharmacists ( n = 10), pharmacy technicians ( n = 8), physicians ( n = 7), health care aides ( n = 2), and others ( n = 8). Various analytical methods were used to quantify the presence of 13 hazardous drugs, primarily cyclophosphamide ( n = 16 studies), platinum-based drugs ( n = 7), and alpha-fluoro-beta-alanine, a urine metabolite derived from 5-fluorouracil ( n = 3). The proportion of workers with positive results ranged from 0% ( n = 10 studies) to 100% ( n = 4). Considering only those studies that allowed calculation of the rate of workers with at least one positive urine sample ( n = 23), the total proportion was 21% (173/809 workers, for all methods and drugs combined)., Conclusion: Twenty-four studies on urine monitoring were conducted in 7 countries between 2010 and 2015. In several studies, no traces of drugs were detected in urine.

Purpose: For a century, autologous bone grafts have been used in maxillofacial reconstruction. The ideal bone harvest site and grafting procedure remains a point of contention in regards to obtaining optimal long-term results with sufficient bone quantity and density without serious complications. More recently, confronted with growing patient requests and biomaterials development, maxillofacial surgeons and dentists have been considering these issues as they relate to pre-implant surgery. This study sought to evaluate implant success rate and complications following pre-implant surgery with parietal bone grafting., Materials and Methods: A retrospective study was carried out on patients who underwent maxillofacial reconstruction of different sites (symphysis, mandibular corpus, maxillary sinus and premaxilla) for the purpose of implant insertion., Results: 311 procedures in 211 patients were included. The implant osseointegration rate was around 95%. Clinical follow-up ranged from 10 months to 11 years. A secondary procedure was performed in 6.1% of cases and we noted no serious complications at the harvest site., Discussion: With good revascularization and osseointegration of the graft, the use of parietal bone leads to an implant success rate similar to that seen in the literature. Moreover, the use of this material results in few infections and low bone resorption provided there is strict immobilization of the graft and no tension on the soft tissue sutures., Conclusion: Parietal bone grafts technique possess the required qualities for the success of implant surgery, offering results at least as interesting as others using autogenous bone and with no serious complications on donor site., (Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Introduction: Long standing maxillary edentulism leads to alveolar ridge resorption which prevent implant placement and causes prosthetic malocclusion. The aim of the study was to assess vertical and transversal bone increase following Le Fort 1 osteotomy associated with calvarial bone grafting., Materials and Methods: 66 patients who presented severely atrophic maxillae were treated with Le Fort 1 osteotomy with bone grafting from 2003 to 2014. Vertical and transversal bone level was measured preoperatively and 6 months post-operatively to calculate the alveolar ridge augmentation. Follow up ranged from 10 months to 11 years., Results: The mean increase of bone height was 9.3 mm and the mean increase of bone width was 6 mm 417 endosseous implants were placed in the grafted maxilla. Mean endosseous implant length was of 10.7 mm at the first molar site (range: 8-16 mm). A total of 25 implants failed, the overall implant survival rate is of 94%. The definitive prosthetis was fixed in 65% of the patients and removable in 35% of the patients., Discussion: Le Fort 1 osteotomy associated with calvarial bone grafting is the main treatment option able to offer fixed bridge and perfect class 1 occlusion in cases of severe maxillary atrophy., (Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

The gray mouse lemur (Microcebus murinus) is considered a useful primate model for translational research. In the framework of IMI PharmaCog project (Grant Agreement n°115009, www.pharmacog.org), we tested the hypothesis that spectral electroencephalographic (EEG) markers of motor and locomotor activity in gray mouse lemurs reflect typical movement-related desynchronization of alpha rhythms (about 8-12 Hz) in humans. To this aim, EEG (bipolar electrodes in frontal cortex) and electromyographic (EMG; bipolar electrodes sutured in neck muscles) data were recorded in 13 male adult (about 3 years) lemurs. Artifact-free EEG segments during active state (gross movements, exploratory movements or locomotor activity) and awake passive state (no sleep) were selected on the basis of instrumental measures of animal behavior, and were used as an input for EEG power density analysis. Results showed a clear peak of EEG power density at alpha range (7-9 Hz) during passive state. During active state, there was a reduction in alpha power density (8-12 Hz) and an increase of power density at slow frequencies (1-4 Hz). Relative EMG activity was related to EEG power density at 2-4 Hz (positive correlation) and at 8-12 Hz (negative correlation). These results suggest for the first time that the primate gray mouse lemurs and humans may share basic neurophysiologic mechanisms of synchronization of frontal alpha rhythms in awake passive state and their desynchronization during motor and locomotor activity. These EEG markers may be an ideal experimental model for translational basic (motor science) and applied (pharmacological and non-pharmacological interventions) research in Neurophysiology.

Pyogenic sacroiliitis is exceptional in very young children. Diagnosis is difficult because clinical examination is misleading. FABER test is rarely helpful in very young children. Inflammatory syndrome is frequent. Bone scintigraphy and MRI are very sensitive for the diagnosis. Joint fluid aspiration and blood cultures are useful to identify the pathogen. Appropriate antibiotic therapy provides rapid regression of symptoms and healing. We report the case of pyogenic sacroiliitis in a 13-month-old child.Clinical, biological, and imaging data of this case were reviewed and reported retrospectively.A 13-month-old girl consulted for decreased weight bearing without fever or trauma. Clinical examination was not helpful. There was an inflammatory syndrome. Bone scintigraphy found a sacroiliitis, confirmed on MRI. Aspiration of the sacroiliac joint was performed. Empiric intravenous biantibiotic therapy was started. Patient rapidly recovered full weight bearing. On the 5th day, clinical examination and biological analysis returned to normal. Intravenous antibiotic therapy was switched for oral. One month later, clinical examination and biological analysis were normal and antibiotic therapy was stopped.Hematogenous osteoarticular infections are common in children but pyogenic sacroiliitis is rare and mainly affects older children. Diagnosis can be difficult because clinical examination is poor. Moreover, limping and decreased weight bearing are very common reasons for consultation. This may delay the diagnosis or refer misdiagnosis. Bone scintigraphy is useful to locate a bone or joint disease responsible for limping. In this observation, bone scintigraphy located the infection at the sacroiliac joint. Given the young age, MRI was performed to confirm the diagnosis. Despite the very young age of the patient, symptoms rapidly disappeared with appropriate antibiotic therapy.We report the case of pyogenic sacroiliitis in a 13-month-old child. It reminds the risk of misdiagnosing pyogenic sacroiliitis in children because it is exceptional and clinical examination is rarely helpful. It also highlights the usefulness of bone scintigraphy and MRI in osteoarticular infections in children.

A structured Web-based search was performed to identify online educational programs on the optimal use of antibiotics in children. Identified Web sites of interest were classified using predefined quality criteria for educational Web resources. Of 10 Web sites identified, only 2 met all the quality criteria: APUA and Getsmart., (© The Author 2013. Published by Oxford University Press on behalf of the Pediatric Infectious Diseases Society. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.)

The endoplasmic reticulum (ER) is a multifunctional organelle critical for the proper folding and assembly of secreted and transmembrane proteins. Perturbations of ER functions cause ER stress, which activates a coordinated system of transcriptional and translational controls called the unfolded protein response (UPR), to cope with accumulation of misfolded proteins and proteotoxicity. It results in ER homeostasis restoration or in cell death. Senescence is a complex cell phenotype induced by several stresses such as telomere attrition, DNA damage, oxidative stress, and activation of some oncogenes. It is mainly characterized by a cell enlargement, a permanent cell-cycle arrest, and the production of a secretome enriched in proinflammatory cytokines and components of the extracellular matrix. Senescent cells accumulate with age in tissues and are suspected to play a role in age-associated diseases. Since senescence is a stress response, the question arises of whether an ER stress could occur concomitantly with senescence and participate in the onset or maintenance of the senescent features. Here, we described the interconnections between the UPR signaling and the different aspects of the cellular senescence programs and discuss the implication of UPR modulations in this context., (Copyright © 2015 the American Physiological Society.)

The intestinal epithelium is a particularly attractive biological adult model to study epigenetic mechanisms driving adult stem cell renewal and cell differentiation. Since epigenetic modifications are dynamic, we have developed an original ex vivo approach to study the expression and epigenetic profiles of key genes associated with either intestinal cell pluripotency or differentiation by isolating cryosections of the intestinal crypt-villus axis. Gene expression, DNA methylation and histone modifications were studied by qRT-PCR, methylation-specific PCR and micro-chromatin immunoprecipitation, respectively. Using this approach, it was possible to identify segment-specific methylation and chromatin profiles. We show that (i) expression of intestinal stem cell markers (Lgr5, Ascl2) exclusively in the crypt is associated with active histone marks, (ii) promoters of all pluripotency genes studied and transcription factors involved in intestinal cell fate (Cdx2) harbour a bivalent chromatin pattern in the crypts and (iii) expression of differentiation markers (Muc2, Sox9) along the crypt-villus axis is associated with DNA methylation. Hence, using an original model of cryosectioning along the crypt-villus axis that allows in situ detection of dynamic epigenetic modifications, we demonstrate that regulation of pluripotency and differentiation markers in healthy intestinal mucosa involves different and specific epigenetic mechanisms., (Copyright © 2015. Published by Elsevier B.V.)

The prevention of fertilization through self-pollination (or pollination by a close relative) in the Brassicaceae plant family is determined by the genotype of the plant at the self-incompatibility locus (S locus). The many alleles at this locus exhibit a dominance hierarchy that determines which of the two allelic specificities of a heterozygous genotype is expressed at the phenotypic level. Here, we uncover the evolution of how at least 17 small RNA (sRNA)-producing loci and their multiple target sites collectively control the dominance hierarchy among alleles within the gene controlling the pollen S-locus phenotype in a self-incompatible Arabidopsis species. Selection has created a dynamic repertoire of sRNA-target interactions by jointly acting on sRNA genes and their target sites, which has resulted in a complex system of regulation among alleles., (Copyright © 2014, American Association for the Advancement of Science.)

The prevalence of breastfeeding in France is one of the lowest in Europe: 65% of infants born in France in 2010 were breastfed when leaving the maternity ward. Exclusive breastfeeding allows normal growth until at least 6 months of age, and can be prolonged until the age of 2 years or more, provided that complementary feeding is started after 6 months. Breast milk contains hormones, growth factors, cytokines, immunocompetent cells, etc., and has many biological properties. The composition of breast milk is influenced by gestational and postnatal age, as well as by the moment of the feed. Breastfeeding is associated with slightly enhanced performance on tests of cognitive development. Exclusive breastfeeding for at least 3 months is associated with a lower incidence and severity of diarrhoea, otitis media and respiratory infection. Exclusive breastfeeding for at least 4 months is associated with a lower incidence of allergic disease (asthma, atopic dermatitis) during the first 2 to 3 years of life in at-risk infants (infants with at least one first-degree relative presenting with allergy). Breastfeeding is also associated with a lower incidence of obesity during childhood and adolescence, as well as with a lower blood pressure and cholesterolemia in adulthood. However, no beneficial effect of breastfeeding on cardiovascular morbidity and mortality has been shown. Maternal infection with hepatitis B and C virus is not a contraindication to breastfeeding, as opposed to HIV infection and galactosemia. A supplementation with vitamin D and K is necessary in the breastfed infant. Very few medications contraindicate breastfeeding. Premature babies can be breastfed and/or receive mother's milk and/or bank milk, provided they receive energy, protein and mineral supplements. Return to prepregnancy weight is earlier in breastfeeding mothers during the 6 months following delivery. Breastfeeding is also associated with a decreased risk of breast and ovarian cancer in the premenopausal period, and of osteoporosis in the postmenopausal period., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

The aim of this study was to determine and validate the new thresholds for various intensities of physical activity in adolescents using the Actigraph accelerometer. Sixty healthy participants aged 10-16 years were recruited. Forty participants participated in the calibration study whereas the others participated in the validation study. Accelerometer data, heart rate, and oxygen consumption were measured at nine levels of physical activity which varied in intensity: sedentary, light, moderate and vigorous. The activity categories and accelerometer counts were sedentary activity, 0-400 counts/min; light activity, 401-1900 counts/min; moderate activity, 1901-3918 counts/min; and vigorous activity, greater than 3918 counts/min, respectively. This study establishes new threshold values for various physical activities that can be used for population-based studies in adolescents.

Background and Aims: Repeated intravenous antibiotic therapy (IVAT) against chronic pulmonary infection with Pseudomonas aeruginosa is often necessary in cystic fibrosis patients (CF). The aim of this study was to monitor kinetics of degradation of pulmonary and nutritional status after IVAT in CF patients., Methods: Lung function, nutritional status and physical activity (PA) were measured for 21 CF patients (mean +/- SD age, 16 +/- 1.9 years; 9 boys) who were chronically colonized by P. aeruginosa. Each parameter was measured every 15 days during 3-6 months., Results: Nutritional status as well as PA did not change in the interval of 2 IVAT. In contrary, lung function worsened with a decrease of -14.6% of the FEV(1) (P < 0.05)., Conclusion: In CF, there is a progressive decrease in lung function without any evident deterioration of nutritional status and PA between two IVAT., ((c) 2009 Wiley-Liss, Inc.)