Can procalcitonin measurement help the diagnosis of osteomyelitis and septic arthritis? A prospective trial

<p>Abstract</p> <p>Objectives</p> <p>Procalcitonin (PCT) is an accurate marker for differentiating bacterial infection from non-infective causes of inflammation or viral infection. However, there is only one study in children which tested procalcitonin as a diagnostic aid in skeletal infections. With this study we sought to evaluate the sensitivity, specificity and predictive values of procalcitonin for identifying bone and joint infection in children evaluated in the emergency department for non traumatic decreased active motion of a skeletal segment.</p> <p>Methods</p> <p>Patients aged 1 month to 14 years were prospectively included in the emergency department when suspected for osteomyelitis or septic arthritis. Procalcitonin levels, C reactiv protein, white blood cell count were measured and bacteriological samples were collected before initiation of antibiotic treatment. Patients were assigned to 3 groups according to the degree of suspected infection: group 1 confirmed infection, group 2 presumed infection and group 3 non infected patients.</p> <p>Results</p> <p>Three hundred thirty nine patients were included (118 girls and 221 boys). Group 1 comprised 8 patients (2 had PCT levels > 0.5 ng/ml). Two had osteomyelitis and 6 septic arthritis. Forty children were incuded in group 2 (4 had PCT levels > 0.5 ng/ml). Eighteen had presumed osteomyelitis and 22 presumed septic arthritis. Group 3 comprised 291 children (9 PCT levels > 0.5 ng/ml) who recovered without antibiotic treatment. The specificity of the PCT as a marker of bacterial infection (comparing Group 1 and Group 3) was 96.9% [95% CI, 94.2-98.6], the sensitivity 25% [95% CI, 3.2-65.1], the positive predictive value (PPV) 18.2% [95% CI, 2.3-51.8] and the negative predictive value (NPV) 97.9% [95% CI, 95.5-99.2].</p> <p>Conclusion</p> <p>PCT is not a good screening test for identifying skeletal infection in children. Larger studies are needed to evaluate still more the place of PCT measurements in the diagnosis of osteomyelitis and septic arthritis.</p

Evidence of spinal stiffening following fusionless bipolar fixation for neuromuscular scoliosis: a shear wave elastography assessment of lumbar annulus fibrosus

Objectives There are no established criteria for stiffness after fusionless surgery for neuromuscular scoliosis (NMS). As a result, there is no consensus regarding the surgical strategy to propose at long-term follow-up. This study reports the first use of shear wave elastography for assessing the mechanical response of lumbar intervertebral discs (IVDs) after fusionless bipolar fixation (FBF) for NMS and compares them with healthy controls. The aim was to acquire evidence from the stiffness of the spine following FBF. Patients and methods Nineteen NMS operated on with FBF (18 ± 2y at last follow-up, 6 ± 1 y after surgery) were included prospectively. Preoperative Cobb was 89 ± 20° and 35 ± 1° at latest follow-up. All patients had reached skeletal maturity. Eighteen healthy patients (20 ± 4 y) were also included. Shear wave speed (SWS) was measured in the annulus fibrosus of L3L4, L4L5 and L5S1 IVDs and compared between the two groups. A measurement reliability was performed. Results In healthy subjects, average SWS (all disc levels pooled) was 7.5 ± 2.6 m/s. In NMS patients, SWS was significantly higher at 9.9 ± 1.4 m/s (p < 0.05). Differences were significant between L3L4 (9.3 ± 1.8 m/s vs. 7.0 ± 2.5 m/s, p = 0.004) and L4L5 (10.3 ± 2.3 m/s vs. 7.1 ± 1.1 m/s, p = 0.0006). No difference was observed for L5S1 (p = 0.2). No correlation was found with age at surgery, Cobb angle correction and age at the SWE measurement. Conclusions This study shows a significant increase in disc stiffness at the end of growth for NMS patients treated by FBF. These findings are a useful adjunct to CT-scan in assessing stiffness of the spine allowing the avoidance of surgical final fusion at skeletal maturity

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

Agénésie radiale (évaluation des résultats thérapeutiques à moyen et long terme, à propos de 25 cas)

PARIS7-Xavier Bichat (751182101) / SudocSudocFranceF

La synovite villonodulaire de l'enfant (à propos de 6 cas)

PARIS7-Xavier Bichat (751182101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Un nouveau modèle pour étudier la physiopathologie des chondrodysplasies liées à la FGFR3

FGFR3 (Fibroblast Growth Factor Receptor 3) est responsable d'une famille de chondrodysplasies de sévérité variable regroupant l'hypochondroplasie, forme modérée, l'achondroplasie, nanisme le plus fréquent et le nanisme thanatophore, forme sévère. Afin de comprendre les conséquences des mutations activatrices sur le développement squelettique, un nouveau modèle murin a été généré exprimant une mutation de nanisme thanatophore. Les souris mutantes présentent un nanisme sévère évolutif avec des os longs courts et trapus, une plaque de croissance désorganisée et un retard d'ossification épiphysaire. De plus, des anomalies de l'épithélium sensoriel de la cochlée ont été mises en évidence et sont responsables d'une surdité chez la souris mutante. Parallèlement, une étude a été réalisée chez l'humain montrant également un retard d'âge osseux et une surdité neurosensorielle dans l'achondroplasie. Ces résultats confirment le rôle primordial de FGFR3 dans l'ossification enchondrale et l'audition.FGFR3 (Fibroblast Growth Factor Receptor 3) cause several chondrodysplasias, including hypochondroplasia, mild phenotype, achondroplasia, most common form of human dwarfism, and thanatophoric dysplasia, severe dwarfism. To investigate the role of activating FGFR3 mutation in skeletal development, we introduced fgfrS mutation in mouse genome corresponding to the thanatophoric dysplasia. The mutant mice displayed severe dwarfism with shortened long bones, growth plate disturbed and secondary ossification center delayed. In addition, the mutant mice exhibited mild deafness with defect in epithelial sensory cells of the inner ear. At the same time, a human study was performed: a bone age delay and a sensorineural hearing loss were also observed in achondroplasia patients. Our results demonstrate the crucial role of FGFRS in endochondral ossification and auditory system.PARIS5-BU Méd.Cochin (751142101) / SudocSudocFranceF

Mise en place d’un dispositif de connaissance, suivi et évaluation socio-économique et environnemental de la Nouvelle Aire Protégée (NAP) du Makay, Madagascar

Covering an area of approximately 4,000 km2, the New Protected Area of Makay, Madagascar, formalized in2017, is emblematic of the richness of biodiversity, with an exceptional rate of endemism, and the natural andcultural heritage of Madagascar. This document presents the challenges and the methodological options forsetting up a socio-economic and environmental knowledge, monitoring and evaluation system. The deviceconsists of three articulated arms, combining social sciences and natural and earth sciences, both quantitativeand qualitative: a quantitative arm of socio-economic and environmental surveys among the populations ofMakay; an ecological monitoring arm; and a qualitative socio-anthropological arm. By putting the people wholive on the edge of the Makay at the heart of the device, it proposes to push the frontier of existinginformation systems, still underdeveloped in this field, despite their decisive importance on a global scale, inorder to better understand the links between conservation and development.D’une superficie d’environ 4 000 km2, la Nouvelle Aire Protégée du Makay, Madagascar, officialisée en 2017,est emblématique de la richesse de la biodiversité, avec un taux d’endémisme exceptionnel, et du patrimoinenaturel et culturel malgache. Le présent document expose les enjeux et les options méthodologiques pour lamise en place d’un dispositif de connaissance, suivi et évaluation socio-économique et environnemental. Ledispositif se compose de trois bras articulés, combinant sciences sociales et sciences de la nature et de la terre,aussi bien quantitatifs que qualitatifs : un dispositif quantitatif d’enquêtes socio-économiques etenvironnementales auprès des populations du Makay ; un dispositif de suivi écologique ; un dispositif qualitatifde type socio-anthropologique. En mettant les populations qui vivent aux pourtours du Makay au coeur dudispositif, il se propose de repousser la frontière des systèmes d’informations existants, à ce jour balbutiantsdans ce domaine, malgré leur importance décisive à l’échelle mondiale, afin de mieux comprendre les liensentre conservation et développement

Towards an inclusive nature conservation initiative: Preliminary assessment of stakeholders’ representations about the Makay region, Madagascar

International audienceThe existence of multiple perspectives and representations of different stakeholders poses critical challenges to conservation initiatives worldwide. Thus, to foster more just and sus- tainable agendas in protected areas (PAs), this diversity of perspectives must be better understood, acknowledged, and tackled. In this article, we aimed to initiate this understand- ing for the Makay region in Madagascar, a poorly-known region where a ‘New Protected Area’ has been gazetted. In combining mental models and social representation theory, we explored different stakeholders’ perspectives about the Makay social-ecological system, and how differences in stakeholders’ viewpoints could challenge the success of an inclusive, just, and sustainable conservation program. We conducted semi-structured interviews with 32 respondents having different expertise on the Makay. During interviews, respondents were guided towards the elicitation of their individual cognitive map (ICM) of the Makay social-ecological system. ICMs were then analyzed in combining quantitative and qualita- tive. Respondents described the Makay through a total of 162 components, including 51 components that constituted the central zone of the Makay’s representation. In particular, respondents pointed to insecurity issues caused by zebu thieves, as well as to environmen- tal challenges relative to anthropogenic fires and hunting. On the contrary, they considered mining activities and timber harvesting as more peripheral problems. Through a multivariate clustering analysis, we discriminated two clusters of respondents with contrasting visions about the Makay, ecocentric vs. social-ecological, which was largely influenced by respon- dents’ background. In comparing the two clusters’ representations, we found that they had dissimilar diagnoses about key socio-environmental challenges in the Makay and how to address them. This ambiguity in respondents’ viewpoints stresses the need to increase research efforts in the Makay region to fill current knowledge gaps about this poorly known social-ecological system, and to foster social learning between stakeholders concerned by the Makay new PA

Activating Fgfr3 Y367C mutation causes hearing loss and inner ear defect in a mouse model of chondrodysplasia.

International audienceFibroblast growth factor receptor 3 (FGFR3) is a key regulator of skeletal development and activating mutations in FGFR3 cause skeletal dysplasias, including hypochondroplasia, achondroplasia and thanatophoric dysplasia. The introduction of the Y367C mutation corresponding to the human Y373C thanatophoric dysplasia type I (TDI) mutation into the mouse genome, resulted in dwarfism with a skeletal phenotype remarkably similar to that of human chondrodysplasia. To investigate the role of the activating Fgfr3 Y367C mutation in auditory function, the middle and inner ear of the heterozygous mutant Fgfr3(Y367C/+) mice were examined. The mutant Fgfr3(Y367C/+) mice exhibit fully penetrant deafness with a significantly elevated auditory brainstem response threshold for all frequencies tested. The inner ear defect is mainly associated with an increased number of pillar cells or modified supporting cells in the organ of Corti. Hearing loss in the Fgfr3(Y367C/+) mouse model demonstrates the crucial role of Fgfr3 in the development of the inner ear and provides novel insight on the biological consequences of FGFR3 mutations in chondrodysplasia