Continuous Deep Sedation Until Death of Children at the End of Life: French Physicians' Opinions.

Objectives: To evaluate physicians' opinions concerning continuous deep sedation until death (CDSUD) and implementation of Claeys-Leonetti; a law intended to be applicable to all patients, but without a specific framework for children thus giving rise to ethically and legally complex situations. The secondary objective was to identify if physicians' characteristics could influence their opinions. Study Design: This was a national, multicenter, noninterventional cross-sectional survey from January 30, 2020, until March 1, 2020. The target population consisted of French physicians involved in children's end-of-life situations. The validated questionnaire explored respondents' characteristics and their opinions on four hypothetical pediatric clinical cases. Results: Analysis was conducted on 391 respondents. The oncological situation was more easily recognized as end of life compared with the neurological pathology (77% vs. 40.4%). Dependence on mechanical ventilation was another major factor influencing physicians in identifying end-of-life situations. Physicians clearly recognized the difference in intention between CDSUD and euthanasia. They accepted to implement CDSUD more easily in newborns. The withdrawal of artificial nutrition and hydration gave rise to divergent opinions. Respondents were in favor of adolescents' decision-making autonomy and their access to drafting advance directives. The child's best interest prevailed in case of objection by parents, except in situations outside the law's framework or in cases of disagreement within the health care team. Conclusion: Results of our study showed differences in the interpretation of the law concerning the CDSUD application framework and provide elements for reflection, which may ultimately contribute to the development of specific guidelines in CDSUD in children at the end of life.

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.

Impact of cardiac surgical timing on the neurodevelopmental outcomes of newborns with Complex congenital heart disease (CHD).

Background: More than half of infants with complex congenital heart disease (CHD) will have a neurodevelopmental disorder of multifactorial causes. The preoperative period represents a time-window during which neonates with complex CHD are in a state of hypoxia and hemodynamic instability, which fosters the emergence of brain injuries and, thus, affects early brain networks and neurodevelopmental outcomes. Currently, there is no consensus regarding the optimal age for cardiac surgery in terms of neurodevelopmental outcomes, and its definition is a real challenge. Our aim is to determine the relationship between cardiac surgical timing and long-term neurodevelopmental outcomes for various types of complex CHD. Methods: We hypothesize that earlier surgical timing could represent a neuroprotective strategy that reduces perioperative white matter injuries (WMIs) and postoperative morbidity, leading to improved neurodevelopmental outcomes in infants with complex CHD. Firstly, our prospective study will allow us to determine the correlation between age at the time of surgery (days of life) and neurodevelopmental outcomes at 24 months. We will then analyze the correlation between age at surgery and (i) the incidence of WMIs (through pre- and postoperative MRIs), (ii) postoperative morbidity, and (iii) the duration of the hospital stay. Implications and Dissemination: This research protocol was registered in the Clinical Trial Registry (National Clinical Trial: NCT04733378). This project aims to help launch the first Neurocardiac Investigation Clinic in Marseille - AP-HM - to propose an overall personalized monitoring and treatment program for patients operated on for complex CHD.

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.

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.

Anesthetic management of pulmonary surgery in newborns and infants.

The main congenital pulmonary airways malformations in newborns and infants requiring surgery are cystic adenoid malformation, congenital lobar emphysema and bronchogenic cyst. The surgical treatment preferably via thoracoscopy is recommended within the first year of life to avoid the risk of pneumopathy. A monopulmonary ventilation is then required by the surgeon to operate the diseased lung. The anesthetic management of intraoperative mono-pulmonary ventilation in newborns and infants is always challenging for the anesthesiologist. The main objective of this study was to describe anesthetic protocol for thoracoscopy and variations of monitored parameters during a mono-pulmonary ventilation procedure in newborns and infants.

[General anesthesia for Crisponi syndrome: case report]. / Anestesia geral para Síndrome de Crisponi: relato de caso.

Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19°C. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.

General anesthesia for Crisponi syndrome: case report / Anestesia geral para Síndrome de Crisponi: relato de caso

Abstract Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19ºC. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.

Resumo A Síndrome de Crisponi é uma condição clínica hereditária grave e rara caracterizada por contrações musculares, trismo, apneia, distúrbios na alimentação, picos de febre alta e inexplicável, e falência de múltiplos órgãos. Descrevemos o cuidado perioperatório de paciente pediátrica com 17 meses de idade, portadora da Síndrome de Crisponi, submetida a gastrostomia endoscópica. A temperatura da sala de cirurgia foi cuidadosamente monitorizada e mantida a 19ºC. A paciente foi submetida a agentes anestésicos inalatórios e venosos. O cuidado cirúrgico e perioperatório desenvolveram-se sem incidentes. As crises de febre na Síndrome de Crisponi originam-se de mutação no gene CRLF1, o que as diferenciam do mecanismo fisiopatológico da hipertermia maligna.

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).

Serum adipokines, adipose tissue measurements and metabolic parameters in patients with advanced radiographic knee osteoarthritis.

We conducted the present study to evaluate the serum levels of adipokines (leptin, total and high molecular adiponectin, resistin), a marker of cartilage breakdown (C2C), and ghrelin together with body composition in patients with knee osteoarthritis (OA). Fifty patients and 50 sex-matched healthy subjects (HS) were evaluated. Knee OA was scored according to the Kellgren-Lawrence (KL) grade. Body composition parameters including lean mass and measurements of fat mass (total fat, adiposity, fat in the android and gynoid regions, visceral fat and trunk/legs fat ratio) were obtained using dual energy X-ray absorptiometry. Most of the recruited patients (88%) had advanced knee OA with KL grade 3 or 4. The patients had higher body mass index than HS (p < 0.0001). Serum leptin, high molecular adiponectin, resistin and ghrelin levels did not differ between patients and HS. Total adiponectin was higher in women with OA compared to women from the HS group (p = 0.004). Total fat mass, adiposity and measurements of central adiposity (fat in the android region, trunk/lower limbs fat ratio and visceral fat) were increased in patients with knee OA (all p < 0.05). Total adiponectin was borderline associated with the severity of OA. Our results show that total adiponectin is significantly increased in women with advanced knee OA. Independently of gender, patients with severe knee OA were characterized by a significant excess of fat with a distribution toward the visceral region. This abnormal body composition may contribute to the cardiometabolic profile that is described in patients with knee OA.

Peri-operative management of overweight and obese children and adolescents.

Obesity has become endemic, even in children. Systemic complications associated with obesity include metabolic syndrome, cardiovascular disease, and respiratory compromise. These comorbidities require adequate investigation, targeted optimisation, and, if surgery is required, specific management during the peri-operative period. Specific peri-operative strategies should be used for paediatric patients who are overweight or obese to prevent postoperative complications, and optimising the respiratory function during surgery is particularly crucial. This Review aims to provide up-to-date information on peri-operative management for physicians who are caring for children and adolescents (usually younger than 18 years) who are overweight or obese undergoing surgery, including bariatric surgery. We have particularly focussed on the physiological consequences of obesity-namely, obstructive sleep apnoea, respiratory compromise, and pharmacological considerations.

Neurodevelopment and Health-Related Quality of Life in Infants Born with Gastroschisis: A 6-Year Retrospective French Study.

Introduction Quantify quality of life (QoL) outcomes in gastroschisis children is little assessed. The primary objective was to describe the long-term outcome of newborns with gastroschisis treated in three tertiary care hospitals of France in terms of neurodevelopment and QoL. Materials and Methods The study reported was a cross-sectional, descriptive multicentric retrospective study assessing the outcome of newborns with gastroschisis, born between January 1, 2009, and December 31, 2014, treated at two large and French level III neonatal intensive care units. Long-term outcome data were assessed by questionnaires sent to the infants' parents. Questionnaires explored global health, neurological development, and quality of life (overall assessment including socio-economic and medical), Age & Stages Questionnaire, infants' quality of life (KIDSCREEN), and quality of parents' life (General Questionnaire Short Form-36). Results In this study, 50% of the survivor's families answered the assessment form (n = 33). The average follow-up age was 40 months, ranging from 8 months to 6 years. Cases of simple gastroschisis more often had a normal score for "communication" (p = 0.033), while patients who received morphine for a longer duration had significantly lower scores for the items "communication" and "problem resolving" (p = 0.024 and p = 0.011, respectively). Children's QoL was significantly lower for patients with gestational age younger than 36 weeks (p = 0.023) and for patients born following "fetal cause delivery" (p = 0.022). Parents had a significantly higher physical composite score if their child underwent primary closure (p = 0.012). Conclusion Our analyses confirm the idea that cases of complex gastroschisis and preterm delivery may lead to poorer outcome. Such hindsight (40 months in mean) allowed for an interesting assessment of development long after the patient's initial hospitalization and to confirm these results, a standardized neuropsychological evaluation of patients should be done when at least 6 years old. An accurate assessment of the social environment and its impact on the development and QoL of children will be fundamental to avoid selection bias.

Upper airway modifications in head extension during development.

BACKGROUND: One of the requirements of laryngoscopy is to determine which head position will result in optimal visualization. Our hypothesis was that parameters derived from magnetic resonance imaging (MRI) can help quantify the effect of age on airway modifications due to head extension during development. METHOD: In children undergoing planned MRI, additional sequences on the upper airways were performed: one in a near-neutral position, the other with the head extended at 35°. The axis of the face, the pharynx, the larynx, the trachea, and the line of glottic visualization were determined. The following angles were calculated: the Visu-Lar angle, formed by the line of glottic visualization and the laryngeal axis, and the Phar-Lar angle, formed by the pharyngeal and laryngeal axes. RESULTS: One hundred and fifty-five patients (1 to 222 months of age [25-145] months) were included and 54% were under general anaesthesia. Age had no effect on the variation in the Visu-Lar angle, which diminished as a function of head extension, nor on the variation in the Phar-Lar angle, which was minimal in the neutral position. During extension, anatomical axes rotated similarly, and the visualization axis rotated the most, followed by the pharyngeal and laryngeal axes. These results were not correlated with general anaesthesia. CONCLUSION: Regardless of age, head extension diminished the Visu-Lar angle, and increased the Phar-Lar angle. This study supports that, as in adults, head extension is probably the key factor for good visualization conditions during laryngoscopy on children, but clinical data is needed to confirm this result.

Golimumab in radiographic and nonradiographic axial spondyloarthritis: a review of clinical trials.

Axial spondyloarthritis (Ax SpA) refers to chronic inflammatory rheumatic diseases that mainly affect the axial skeleton, leading to erosions and new bone formation in the sacroiliac joints and/or the spine. Ax SpA includes the radiographic form of the disease, ie, ankylosing spondylitis (AS), and the nonradiographic Ax SpA (non-Rx Ax SpA) forms. Anti-tumor necrosis factor alpha (TNFα) agents are used in the treatment of Ax SpA in patients who do not respond to or are intolerant to nonsteroidal anti-inflammatory drugs. In these patients, anti-TNFα agents show promising results by targeting the inflammatory process and providing symptomatic relief. Golimumab is a fully human anti-TNFα agent that is currently approved for the treatment of both AS and non-Rx Ax SpA in Europe. This review focuses on the results of clinical trials with golimumab for the treatment of AS (GO-RAISE studies) and non-Rx Ax SpA (GO-AHEAD study) and on the effects of this agent on imaging findings (radiographic progression, magnetic resonance imaging inflammation) as well as on biological parameters. Overall, golimumab is a valid therapeutic option in patients with AS and non-Rx Ax SpA in Europe.

Prognostic value of abdominal sonography in necrotizing enterocolitis of premature infants born before 33 weeks gestational age.

OBJECTIVE: The purpose of this study was to assess the prognostic value of abdominal sonography in necrotizing enterocolitis (NEC) in preterm infants with a gestational age less than 33 weeks of gestation, using surgery and/or death as the primary outcome and stenosis as the secondary outcome. METHODS: A retrospective study of 95 premature infants (mean gestational age: 28.6 weeks), presenting with NEC between January 2009 and November 2011 and who underwent plain abdominal radiography and sonography, was performed. In uni- and multivariate analyses, radiographic and sonographic findings were correlated with complications ('surgery and/or death' and 'stenosis'). RESULTS: Sonographic findings of free intraperitoneal air (odd ratio [OR]=8.0; IC, 1.4-44.2), free abdominal fluid (OR 3.5; IC 1.3-9.4), portal venous gas (OR 3.9; IC, 1.2-12.9), and bowel wall thickening (OR 2.8; IC,1.1-7.2) were significantly associated with surgery and/or death. Intramural gas was significantly correlated (OR=11.8; IC, 1.5-95.8) with intestinal stenosis following NEC. None of the radiographic findings were associated with complications. CONCLUSION: Abdominal sonography is a reliable tool for the prognostic assessment of NEC in preterm infants.

Esophageal atresia, small omphalocele and ileal prolapse through a patent omphalomesenteric duct: a methimazole embryopathy? [Corrected].

Newborns prenatally exposed to methimazole (active metabolite of carbamizole) for maternal hyperthyroidism may present some disorders in common, but the phenotype is not well defined. Choanal atresia is the most frequent, and other anomalies such as esophageal atresia and aplasia cutis were described with this embryopathy. Additionally, patent omphalomesenteric duct or Meckel's diverticulum in similar association was reported in some patients. The predisposed genetic background has to be considered. We report the case of a newborn exposed to carbamizole during the first 4 weeks of pregnancy and define an association related to prenatal methamizole exposure consisting of esophageal atresia, small omphalocele, and ileal prolapse through a patent omphalomesenteric duct.

Health-related quality of life and its determinants in children with a congenital diaphragmatic hernia.

BACKGROUND: The development of new therapeutics has led to progress in the early management of congenital diaphragmatic hernia (CDH) in pediatric intensive care units (PICU). Little is known about the impact on the quality of life (QoL) of children and their family. The aim of this study was to assess the impact of CDH treated according to the most recent concepts and methods outlined above on child survivors' QoL and their parents' QoL. PATIENTS AND METHODS: This study incorporated a cross-sectional design performed in two PICU (Marseille, France). Families of CDH survivors born between 1999 and 2008 were eligible. The following data were recorded: socio-demographics, antenatal history and delivery, initial hospitalization history. Self-reported data were collected by mail, including current clinical problems of the children (13-symptom list), children's QoL (Kidscreen-27 questionnaire), and parents' QoL (Short-Form 36 questionnaire). Children's QoL score was compared with controls and QoL of survivors of childhood leukemia. Parent's QoL was compared with controls. Non-parametric statistics were employed. RESULTS: Forty-two families agreed to participate and questionnaires were completed by 32 of them. Twenty-one children had a current clinical problems related to CDH. All the QoL scores of CHD survivors were significantly lower compared with controls. The physical well-being dimension was significantly higher for CHD survivors compared with survivors of childhood leukemia. Gastro-esophageal reflux at discharge, antenatal diagnosis, length of stay in the PICU, and neuropsychological and respiratory issues significantly impacted QoL scores of children. The parents of CHD survivors had significantly poorer score in emotional role dimension compared with controls. CONCLUSION: The impact of CDH on QoL seems to be important and must be understood by clinicians who treat these children and their parents.

Ustekinumab in chronic immune-mediated diseases: a review of long term safety and patient improvement.

Ustekinumab is a fully human monoclonal antibody targeting the common p40 subunit shared by interleukin (IL)-12 and IL-23. Ustekinumab prevents the interaction of IL-12 and IL-23 with their cell surface receptors, and thus blocks T helper (Th)-1 IL-12 and Th-17 IL-23 inflammatory pathways. Ustekinumab has been evaluated in the treatment of various chronic immune-mediated diseases including, psoriasis, psoriatic arthritis, Crohn's disease, and multiple sclerosis. It led to a rapid and durable improvement in psoriasis area and severity index in patients with moderate to severe psoriasis. Ustekinumab also improved joint symptoms of psoriatic arthritis. Results in Crohn's disease were more mitigated, albeit with a symptomatic improvement in patients refractory to tumor necrosis factor-α inhibitors. Ustekinumab did not reduce the number of magnetic resonance imaging brain lesions in multiple sclerosis. The most common adverse events to have been observed during clinical trials are mild in intensity, and include respiratory tract infections, nasopharyngitis, headaches, and injection site reactions. A pooled analysis of clinical trial data indicated no specific patterns of infection or malignancy under long-term ustekinumab administration. Ustekinumab is easy to use, has a comfortable therapeutic regimen, improves quality of life in patients, and thus appears to be an attractive biological treatment that is adapted and accepted by patients with moderate to severe psoriasis.