Electromyographic Activity of Lower Limb Muscles during Ankle Destabilizing Tests.

Ankle destabilizing devices were developed to improve the recruitment of the evertor muscles. Nevertheless, the activity of lower-leg muscles has never been compared to each other during functional tests performed with destabilization. The objectives were i) to compare the electromyographic activity between the lower-leg muscles during four functional tests performed with ankle destabilization, and ii) to determine sex-related differences in neuromuscular activation. Twenty-six healthy volunteers (13 males, 13 females) performed the modified Star Excursion Balance Test (mSEBT), unipedal balance and weight-bearing inversion and eversion tests with a destabilizing device, while recording electromyographic activity of the peroneus longus and brevis, tibialis anterior, gastrocnemius lateralis and gluteus medius. The activity of peroneal muscles was significantly greater than other muscles during all functional tests. Furthermore, the anterior direction of the mSEBT was the one implying the greatest activity of the peroneus longus muscle compared to the posteromedial (p=0.003) or posterolateral (p<0.001) directions. Finally, no significant sex-related differences in neuromuscular activity were reported. This study highlights the effectiveness of the destabilizing device to involve specifically the peroneal muscles when performing various functional tests. This device should be used by clinicians to be more specific to the stabilizers of the ankle joint during functional exercises.

Ethical issues encountered by French intensive care unit caregivers during the first COVID-19 outbreak.

PURPOSE: We aimed to describe the ethical issues encountered by health care workers during the first COVID-19 outbreak in French intensive care units (ICUs), and the factors associated with their emergence. METHODS: This descriptive multicentre survey study was conducted by distributing a questionnaire to 26 French ICUs, from 1 June to 1 October 2020. Physicians, residents, nurses, and orderlies who worked in an ICU during the first COVID-19 outbreak were included. Multiple logistic regression models were performed to identify the factors associated with ethical issues. RESULTS: Among the 4,670 questionnaires sent out, 1,188 responses were received, giving a participation rate of 25.4%. Overall, 953 participants (80.2%) reported experiencing issue(s) while caring for patients during the first COVID-19 outbreak. The most common issues encountered concerned the restriction of family visits in the ICU (91.7%) and the risk of contamination for health care workers (72.3%). Nurses and orderlies faced this latter issue more than physicians (adjusted odds ratio [ORa], 2.98; 95% confidence interval [CI], 1.87 to 4.76; P < 0.001 and ORa, 4.35; 95% CI, 2.08 to 9.12; P < 0.001, respectively). They also faced more the issue "act contrary to the patient's advance directives" (ORa, 4.59; 95% CI, 1.74 to 12.08; P < 0.01 and ORa, 10.65; 95% CI, 3.71 to 30.60; P < 0.001, respectively). A total of 1,132 (86.9%) respondents thought that ethics training should be better integrated into the initial training of health care workers. CONCLUSION: Eight out of ten responding French ICU health care workers experienced ethical issues during the first COVID-19 outbreak. Identifying these issues is a first step towards anticipating and managing such issues, particularly in the context of potential future health crises.

RéSUMé: OBJECTIF: Notre objectif était de décrire les enjeux éthiques rencontrés par les personnels de santé lors de la première éclosion de COVID-19 dans les unités de soins intensifs (USI) françaises, ainsi que les facteurs associés à leur apparition. MéTHODE: Cette enquête multicentrique descriptive a été réalisée en distribuant un questionnaire à 26 unités de soins intensifs françaises, du 1er juin au 1er octobre 2020. Les médecins, les internes, le personnel infirmier et les aides-soignant·es qui travaillaient dans une unité de soins intensifs pendant la première éclosion de COVID-19 ont été inclus·es. Des modèles de régression logistique multiple ont été réalisés pour identifier les facteurs associés aux questions éthiques. RéSULTATS: Parmi les 4670 questionnaires envoyés, 1188 réponses ont été reçues, soit un taux de participation de 25,4 %. Dans l'ensemble, 953 personnes participantes (80,2 %) ont déclaré avoir éprouvé un ou des problèmes alors qu'elles s'occupaient de patient·es lors de la première éclosion de COVID-19. Les problèmatiques les plus fréquemment rencontrées concernaient la restriction des visites des familles dans les USI (91,7 %) et le risque de contamination pour les personnels de la santé (72,3 %). Le personnel infirmier et les aides-soignant·es étaient davantage confronté·es à ce dernier problème que les médecins (rapport de cotes ajusté [RCa], 2,98; intervalle de confiance [IC] à 95 %, 1,87 à 4,76; P < 0,001 et RCa, 4.35; IC 95 %, 2,08 à 9,12; P < 0,001, respectivement), tout comme ils étaient davantage confrontées à la question d'« agir contrairement aux directives médicales anticipées du/de la patient·e ¼ (RCa, 4,59; IC 95 %, 1,74 à 12,08; P < 0,01 et RCa, 10,65; IC 95 %, 3,71 à 30,60; P < 0,001, respectivement). Au total, 1132 répondant·es (86,9 %) estimaient que la formation en éthique devrait être mieux intégrée à la formation initiale des personnels de santé. CONCLUSION: Huit travailleuses et travailleurs de santé français·es des soins intensifs sur dix ont été confronté·es à des problèmes éthiques lors de la première éclosion de COVID-19. L'identification de ces enjeux est une première étape vers leur anticipation et leur gestion, en particulier dans le contexte d'éventuelles crises sanitaires futures.

Pediatric anesthetic for tracheobronchial foreign body extraction: A survey of practice in France.

BACKGROUND: Tracheobronchial foreign body aspiration is a classic pediatric emergency, and its associated morbidity particularly depends on the anesthetic management, which differs according to the center and the practitioner. AIMS: The aim of this study was to evaluate the different anesthetic practices for tracheobronchial foreign body extraction. METHODS: A survey was sent via email to the member physicians of the Association des Anesthésistes Réanimateurs Pédiatriques d'Expression Française (ADARPEF). The survey included 28 questions about the organizational and anesthetic management of an evolving clinical case. RESULTS: A total of 151 physicians responded to the survey. Only 13.2% of the respondents reported that their institution had a management protocol, and 21.7% required a computerized tomography scan before the procedure was performed for children who were asymptomatic or mildly symptomatic during the night. There were 56.3% of the respondents who reported that extraction with a rigid bronchoscope is the only procedure usually performed in their institution. Regarding rigid bronchoscopy, 47.0% used combined intravenous-inhalation anesthesia. The objective was to maintain the child on spontaneous ventilation for 63.6% of the respondents, but anesthesia management differed according to the physician's experience. CONCLUSIONS: Our study confirms the diversity of practices concerning anesthetic for tracheobronchial foreign body extraction and found reveal differences in practice according to physician experience.

Fatigue of hip abductor muscles implies neuromuscular and kinematic adaptations of the ankle during dynamic balance.

OBJECTIVE: Determine whether hip abductor muscle fatigue influenced ankle kinematic and muscle activity during ankle-destabilized tasks. DESIGN: Cross-sectional study. METHODS: Twenty-six healthy, active participants performed two tests (Star Excursion Balance Test, SEBT; Weight Bearing Inversion Test, WBIT) for assessment of dynamic balance and ability to control inversion. Participants were equipped with an ankle-destabilizing sandal in inversion and eversion to perform both tests, which were completed before and after a fatiguing exercise of hip abductor muscles (up to 50% reduction in strength). Electromyographic activity of peroneus longus (PL) and brevis (PB), tibialis anterior, gastrocnemius lateralis (GastL) and gluteus medius (GlutM) muscles were recorded. In addition, ankle kinematics were recorded using an inertial measurement unit. RESULTS: Hip abductor fatigue induced a significant decrease in SEBT scores in three directions (p < 0.01). During SEBT, ankle supination decreased by 3.2° in the anterior and posteromedial directions (p < 0.01). Muscle activity of GastL increased during achievement of three directions (p < 0.05) in response to hip abductor fatigue. In posteromedial direction, PL (p < 0.001) and GlutM (p < 0.01) activity increased with fatigue. During WBIT, inversion angular velocity was not impacted by fatigue while, PB and GastL activity increased after fatiguing exercise (p < 0.005). CONCLUSION: A decrease in SEBT performance and EMG adaptations with proximal fatigue attest to the importance of the hip abductor muscle in dynamic postural control. This could have important implications in building injury prevention programs. Changes in ankle supination may reflect a protective strategy of the joint in response to hip fatigue.

Management of primary spontaneous pneumothorax in teenagers: An 11-year study.

AIM: Management of teenagers with primary spontaneous pneumothorax (PSP) is not consensual. We report our experience over an 11-year period. METHODS: For each patient under 20 years hospitalised with PSP from 2008 to 2018, demographic data, smoking habits, clinical presentation, hospitalisation unit, radiological management and its results, therapeutic management (observation, needle aspiration, chest tube drainage and surgery), complications, length of stay, given advice at discharge and recurrence were collected. RESULTS: Seventy patients were included in different paediatric or adult surgery or pulmonology wards (82.9% boys; 16.8 ± 1.7 years; one severe presentation; 18/58 smokers). Chest CT-scan (n = 42/70, 60%) revealed blebs/bullae in 18/39 examinations (46.2%). Treatment consisted of observation (14/70, 20%), needle aspiration (2/70, 2.9%), chest tube (53/70, 75.7%) and video-assisted thoracoscopy surgery (27/70, 38.6%). Half patients with interventional procedure presented complications. A median of 10 chest X-rays was noted during a median stay of 8 days. Advice concerning sport practice, flying, smoking, etc., was variably delivered. PSP recurrence concerned 35/70 patients (50%) without identified predictive factors. CONCLUSION: Compared to recent recommendations of a more conservative approach, chest CT-scan and interventional strategy are overused in our teenagers with PSP. Observation, more or less needle aspiration, should be clearly the first-line treatments.

How doctors communicated with parents in a neonatal intensive care: Communication and ethical issues.

AIM: Doctors have a moral and legal obligation to keep patients and their families informed, and this is an integral part of care. We explored the communication strategies used by doctors when they spoke to parents in a French neonatal intensive care unit (NICU). METHODS: This was a single-centre qualitative pilot study carried out from October 2015 to January 2016. We asked five doctors (three female) to audiotape their discussions with the parents of newborn infants during their NICU stay. The doctors' mean age was 43 years, and they had a mean of 14 years of NICU experience. Each discussion was subjected to thematic content analysis. RESULTS: We analysed 40 discussions carried out between doctors on 26 newborn infants. Five communication strategy themes emerged: building understanding, how the communication was constructed, the role of the doctor, and of the parents, in the overall care of the newborn infant and how the information given to the parents developed over time. CONCLUSION: Analysing the content of the information discussed with parents provided us with the opportunity to understand the communication and ethical issues surrounding the delivery of information in a NICU. This could be used to improve future discussions between doctors and parents.

Intra-articular injections of platelet-rich plasma in symptomatic knee osteoarthritis: a consensus statement from French-speaking experts.

PURPOSE: There has been much debate regarding the use of intra-articular injections of platelet-rich plasma (PRP) as symptomatic treatment for knee osteoarthritis. The objective of this consensus was to develop guidelines for PRP injections in knee osteoarthritis according to the French National Authority for Health recommendations. METHODS: Fifteen physicians from different French-speaking countries (10 rheumatologists, 4 specialists in rehabilitation and sports medicine and 1 radiologist) were selected for their expertise in the areas of PRP and osteoarthritis. A comprehensive literature review was conducted on Medline including all published therapeutic trials, open studies, meta-analysis and systematic reviews focusing on the effects of PRP in knee OA, as well as fundamental studies concerning the characteristics of the various types of PRP and their mechanisms, indexed before April 2019. Using the method recommended by the French National Authority for Health inspired by the Delphi consensus process, 25 recommendations were finally retained and evaluated. The recommendations were classified as appropriate or not appropriate, with strong or relative agreement, or uncertain if a consensus was not achieved. RESULTS: Among the 25 recommendations selected, the main ones are the following: (1) Intra-articular injections of PRP are an effective symptomatic treatment for early to moderate knee osteoarthritis. This recommendation was considered appropriate with a relative agreement (Median = 8; rank = 6-9). Level of evidence 1A. (2) A PRP treatment sequence in knee osteoarthritis may include 1-3 injections. This recommendation was considered appropriate with a strong agreement (Median = 9; rank = 7-9). Level of evidence 1A. (3) Leucocytes-poor PRP should be preferred in knee osteoarthritis. This recommendation was considered appropriate with a relative agreement (Median = 8; rank = 5-9). Level of evidence 5. (4) Intra-articular PRP knee injections should be performed under ultrasound or fluoroscopic guidance. This recommendation was considered uncertain with no consensus (Median = 8; rank = 3-9). Level of evidence 5. (5) PRP should not be mixed with an anesthetic or intra-articular corticosteroid. This recommendation was considered appropriate with a relative agreement (Median = 9; rank = 6-9). Level of evidence 5 CONCLUSION: Those 25 recommendations should standardize and facilitate the use of IA PRP injections, which are considered by experts as an effective treatment especially in early or moderate knee OA. Although a strong or relative agreement from the experts was obtained for most of the recommendations, many of them had a very low level of evidence (Level 5) and were principally based on the clinical experience of the experts.

Characterization of DCL4 missense alleles provides insights into its ability to process distinct classes of dsRNA substrates.

In the model plant Arabidopsis thaliana, four Dicer-like proteins (DCL1-4) mediate the production of various classes of small RNAs (sRNAs). Among these four proteins, DCL4 is by far the most versatile RNaseIII-like enzyme, and previously identified dcl4 missense alleles were shown to uncouple the production of the various classes of DCL4-dependent sRNAs. Yet little is known about the molecular mechanism behind this uncoupling. Here, by studying the subcellular localization, interactome and binding to the sRNA precursors of three distinct dcl4 missense alleles, we simultaneously highlight the absolute requirement of a specific residue in the helicase domain for the efficient production of all DCL4-dependent sRNAs, and identify, within the PAZ domain, an important determinant of DCL4 versatility that is mandatory for the efficient processing of intramolecular fold-back double-stranded RNA (dsRNA) precursors, but that is dispensable for the production of small interfering RNAs (siRNAs) from RDR-dependent dsRNA susbtrates. This study not only provides insights into the DCL4 mode of action, but also delineates interesting tools to further study the complexity of RNA silencing pathways in plants, and possibly other organisms.

A specific dsRNA-binding protein complex selectively sequesters endogenous inverted-repeat siRNA precursors and inhibits their processing.

In plants, several dsRNA-binding proteins (DRBs) have been shown to play important roles in various RNA silencing pathways, mostly by promoting the efficiency and/or accuracy of Dicer-like proteins (DCL)-mediated small RNA production. Among the DRBs encoded by the Arabidopsis genome, we recently identified DRB7.2 whose function in RNA silencing was unknown. Here, we show that DRB7.2 is specifically involved in siRNA production from endogenous inverted-repeat (endoIR) loci. This function requires its interacting partner DRB4, the main cofactor of DCL4 and is achieved through specific sequestration of endoIR dsRNA precursors, thereby repressing their access and processing by the siRNA-generating DCLs. The present study also provides multiple lines of evidence showing that DRB4 is partitioned into, at least, two distinct cellular pools fulfilling different functions, through mutually exclusive binding with either DCL4 or DRB7.2. Collectively, these findings revealed that plants have evolved a specific DRB complex that modulates selectively the production of endoIR-siRNAs. The existence of such a complex and its implication regarding the still elusive biological function of plant endoIR-siRNA will be discussed.

[Hit two birds with one stone: the multiple properties of (viral) RNA silencing suppressors]. / D'une pierre deux coups : les multiples caractéristiques des suppresseurs (viraux) de l'interférence par l'ARN.

In any organism, gene expression regulation is provided by multiple factors to maintain a harmonious development of individuals. Discovered in the late 1990s, RNA interference (RNAi) completely remodelled the way in which gene expression regulations were initially apprehended. RNAi provides fine regulation at the cellular level and allows organisms to control their development, maintain their genomic integrity and fight against different stresses like viral infection. Exogenous or endogenous double-stranded RNAs initiate RNAi and are recognized and cleaved by Dicer protein in about 20 nucleotide duplexes small RNAs (sRNAs). One strand of the duplex is loaded into a ribonucleoproteic complex, named RISC (RNA-induced silencing complex), composed of at least one ARGONAUTE protein and a sRNA. Therefore, the expression of any RNA possessing the complementary siRNA sequence of the small RNA will be specifically silenced either at the transcriptional or post-transcriptional level. RNAi plays a prominent role in the defence against viral infection and the last two decades of research have refined our knowledge of proteins involved in this pathway. Many viruses counteract the antiviral action of RNAi through the expression of factors (viral suppressor of RNA silencing [VSR]) that were first identified on virally infected plants. However, in mammals, the antiviral role of RNAi remains controversial. Indeed, viral infections are controlled by the interferon response and the antiviral action of RNAi has not been clearly demonstrated in vivo. In this review, the main modes of defence suppression used by VSR and endogenous RNAi suppressors will be presented. Finally, the role of viral non-coding RNAs (ncRNAs) acting as suppressors of RNAi will be discussed.

Hit two birds with one stone: the multiple properties of (viral) RNA silencing suppressors.

In any organism, gene expression regulation is provided by multiple factors to maintain a harmonious development of individuals. Discovered in the late 1990s, RNA interference (RNAi) completely remodelled the way in which gene expression regulations were initially apprehended. RNAi provides fine regulation at the cellular level and allows organisms to control their development, maintain their genomic integrity and fight against different stresses like viral infection. Exogenous or endogenous double-stranded RNAs initiate RNAi and are recognized and cleaved by Dicer protein in about twenty nucleotide duplexes small RNAs (sRNAs). One strand of the duplex is loaded into a ribonucleoproteic complex, named RISC (RNA induced silencing complex), composed of at least one ARGONAUTE protein and a sRNA. Therefore, the expression of any RNA possessing the complementary siRNA sequence will be specifically silenced either at the transcriptional or post-transcriptional level. RNAi plays a prominent role in the defence against viral infection and the last two decades of research have refined our knowledge of proteins involved in this pathway. Many viruses counteract the antiviral action of RNAi through the expression of factors (VSR, Viral suppressor of RNA silencing) that were first identified on virally infected plants. However, in mammals the antiviral role of RNAi remains controversial. Indeed, viral infections are controlled by the interferon response and the antiviral action of RNAi has not been clearly demonstrated in vivo. In this review, the main modes of defence suppression used by VSR and endogenous RNAi suppressors will be presented. Finally, the role of viral non-coding RNAs (ncRNAs) acting as suppressors of RNAi will be discussed.

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register.

OBJECTIVE: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. STUDY DESIGN: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. RESULTS: A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year. CONCLUSIONS: Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.

Peri-operative respiratory adverse events in children with upper respiratory tract infections allowed to proceed with anaesthesia: A French national cohort study.

BACKGROUND: Peri-operative respiratory adverse events (PRAEs) in paediatric patients with upper respiratory tract infections (URTIs) remain inadequately explored in patients allowed to proceed to anaesthesia and surgery. OBJECTIVE: To determine the incidence and risk factors of PRAE in children with URTI allowed to proceed to anaesthesia. DESIGN: Multicentre cohort study performed over 6 months in France. SETTING: Sixteen centres with dedicated paediatric anaesthetists. PATIENTS: Eligible patients were aged from 0 to 18 years with URTI symptoms on admission or a history of such over the preceding 4 weeks. MAIN OUTCOMES: The primary outcome of the study was to determine predictors of PRAE. Secondary outcomes were: predictors of peri-operative arterial desaturation and of the decision to proceed with anaesthesia and surgery in children with URTI. RESULTS: Overall, 621 children were included and 489 (78.7%) anaesthetised. Of those anaesthetised, 165 (33.5%) and 97 (19.8%) experienced PRAE and arterial desaturation, respectively. Factors predictive of PRAE included patient age, tracheal intubation and the absence of midazolam premedication. Factors predictive of peri-operative arterial desaturation included patient age, anaesthetist experience, endoscopic procedures and the presence of other PRAE. Factors predicting proceeding to anaesthesia in the context of URTI included anaesthetist experience, emergency procedures and the absence of severe URTI symptoms. CONCLUSION: The risk of PRAE in patients anaesthetised in the presence of URTI was similar to previous publications - close to 30%. In the light of our findings, first, current rescheduling indications should be questioned, and second, further medical and organisational strategies should be investigated to reduce PRAE in children with URTI. TRIAL REGISTRATION: The study was registered in the European Networks of Centers for Pharmacoepidemiology and Pharmacovigilance (EUPAS16436).

New DRB complexes for new DRB functions in plants.

Double-stranded RNA binding (DRB) proteins are generally considered as promoting cofactors of Dicer or Dicer-like (DCL) proteins that ensure efficient and precise production of small RNAs, the sequence-specificity guide of RNA silencing processes in both plants and animals. However, the characterization of a new clade of DRB proteins in Arabidopsis has recently challenged this view by showing that DRBs can also act as potent inhibitors of DCL processing. This is achieved through sequestration of a specific class of small RNA precursors, the endogenous inverted-repeat (endoIR) dsRNAs, thereby selectively preventing production of their associated small RNAs, the endoIR-siRNAs. Here, we concisely summarize the main findings obtained from the characterization of these new DRB proteins and discuss how the existence of such complexes can support a potential, yet still elusive, biological function of plant endoIR-siRNAs.

Comparing Three Methods for Dilution Accuracy of Intravenous Preparations.

PURPOSE: Dilutions of intravenous medications may be inaccurate. The mixing technique may be a crucial factor. DESIGN: Three factors of dilution were tested: volume for dilution (large vs small), method for mixing (shaking vs inversion), and number of maneuvers (3 times vs 10). METHODS: Dilutions of glucose in saline solution were made by nurses, after a random factorial plan. The judgment criteria were the comparison between measured (Cmes) and expected (Cexp) concentration. FINDING: Cmes (n = 40) ranged from 89.5% to 123.6% of Cexp and was more accurate when made with a large volume (98.4% of Cexp vs 106.5%) and when mixed by inversion (100.6% of Cexp vs 104.6%). CONCLUSIONS: Inversion rather than shaking and dilution in a large volume is a simple procedure for bedside medication preparation that allows better accuracy. The 3 versus 10 mixing procedures resulted in the same accuracy, which may be important for these time-consuming procedures. These results should be confirmed in clinical situations.

Viscoelasticity in Achilles tendonopathy: quantitative assessment by using real-time shear-wave elastography.

PURPOSE: To investigate the differences in viscoelastic properties between normal and pathologic Achilles tendons (ATs) by using real-time shear-wave elastography (SWE). MATERIALS AND METHODS: The institutional review board approved this study, and written informed consent was obtained from 25 symptomatic patients and 80 volunteers. One hundred eighty ultrasonographic (US) and SWE studies of ATs without tendonopathy and 30 studies of the middle portion of the AT in patients with tendonopathy were assessed prospectively. Each study included data sets acquired at B-mode US (tendon morphology and cross-sectional area) and SWE (axial and sagittal mean velocity and relative anisotropic coefficient) for two passively mobilized ankle positions. The presence of AT tears at B-mode US and signal-void areas at SWE were noted. RESULTS: Significantly lower mean velocity was shown in tendons with tendonopathy than in normal tendons in the relaxed position at axial SWE (P < .001) and in the stretched position at sagittal (P < .001) and axial (P = .0026) SWE. Tendon softening was a sign of tendonopathy in relaxed ATs when the mean velocity was less than or equal to 4.06 m · sec(-1) at axial SWE (sensitivity, 54.2%; 95% confidence interval [CI]: 32.8, 74.4; specificity, 91.5%; 95% CI: 86.3, 95.1) and less than or equal to 5.70 m · sec(-1) at sagittal SWE (sensitivity, 41.7%; 95% CI: 22.1, 63.3; specificity, 81.8%; 95% CI: 75.3, 87.2) and in stretched ATs, when the mean velocity was less than or equal to 4.86 m · sec(-1) at axial SWE (sensitivity, 66.7%; 95% CI: 44.7, 84.3; specificity, 75.6%; 95% CI: 68.5, 81.7) and less than or equal to 14.58 m · sec(-1) at sagittal SWE (sensitivity, 58.3%; 95% CI: 36.7, 77.9; specificity, 83.5%; 95% CI: 77.2, 88.7). Anisotropic results were not significantly different between normal and pathologic ATs. Six of six (100%) partial-thickness tears appeared as signal-void areas at SWE. CONCLUSION: Whether the AT was relaxed or stretched, SWE helped to confirm and quantify pathologic tendon softening in patients with tendonopathy in the midportion of the AT and did not reveal modifications of viscoelastic anisotropy in the tendon. Tendon softening assessed by using SWE appeared to be highly specific, but sensitivity was relatively low.

A new muscle glycogen storage disease associated with glycogenin-1 deficiency.

We describe a slowly progressive myopathy in 7 unrelated adult patients with storage of polyglucosan in muscle fibers. Genetic investigation revealed homozygous or compound heterozygous deleterious variants in the glycogenin-1 gene (GYG1). Most patients showed depletion of glycogenin-1 in skeletal muscle, whereas 1 showed presence of glycogenin-1 lacking the C-terminal that normally binds glycogen synthase. Our results indicate that either depletion of glycogenin-1 or impaired interaction with glycogen synthase underlies this new form of glycogen storage disease that differs from a previously reported patient with GYG1 mutations who showed profound glycogen depletion in skeletal muscle and accumulation of glycogenin-1.

Comparison of a high-flow humidified nasal cannula to nasal continuous positive airway pressure in children with acute bronchiolitis: experience in a pediatric intensive care unit.

UNLABELLED: The objective of the current study is to compare the use of a nasal continuous positive airway pressure (nCPAP) to a high-flow humidified nasal cannula (HFNC) in infants with acute bronchiolitis, who were admitted to a pediatric intensive care unit (PICU) during two consecutive seasons. We retrospectively reviewed the medical records of all infants admitted to a PICU at a tertiary care French hospital during the bronchiolitis seasons of 2010/11 and 2011/12. Infants admitted to the PICU, who required noninvasive respiratory support, were included. The first noninvasive respiratory support modality was nCPAP during the 2010/11 season, while HFNC was used during the 2011/2012 season. We compared the length of stay (LOS) in the PICU; the daily measure of PCO2 and pH; and the mean of the five higher values of heart rate (HR), respiratory rate (RR), FiO2, and SpO2 each day, during the first 5 days. Thirty-four children met the inclusion criteria: 19 during the first period (nCPAP group) and 15 during the second period (HFNC group). Parameters such as LOS in PICU and oxygenation were similar in the two groups. Oxygen weaning occurred during the same time for the two groups. There were no differences between the two groups for RR, HR, FiO2, and CO2 evolution. HFNC therapy failed in three patients, two of whom required invasive mechanical ventilation, versus one in the nCPAP group. CONCLUSION: We did not find a difference between HFNC and nCPAP in the management of severe bronchiolitis in our PICU. Larger prospective studies are required to confirm these findings.