Assessment of general emergency medicine physicians' medical education regarding management of pediatric emergencies in western Normandy, France.

OBJECTIVES: General emergency medicine physicians sometimes have to deal with acute management of pediatric emergencies. The objectives of this study were to assess the pediatric medical education background of emergency physicians, how often they encounter pediatric emergencies, as well as the knowledge and organizational gaps regarding the management of children in general emergency departments. METHODS: A survey was conducted from March 25 to June 25, 2017, by emailing an anonymized questionnaire to all senior emergency physicians of the 22 general emergency departments of western Normandy public hospitals. RESULTS: A total of 81 responses were analyzed. In all, 83% percent of respondents had previously worked in a pediatric department. In total, 90% of the respondents reported caring for children within their emergency department and 93% out-of-hospital (100% of them during primary interventions and 39% during secondary interventions such as inter-hospital transfers). Fourteen percent of the respondents considered that the pediatric medical education they received was adapted to their current practice, while 73% reported experiencing difficulties during management of pediatric emergencies (technical difficulties, unsuitable material and therapeutics, relational problems, personal apprehension, disease- or age-specific difficulties, especially with children under the age of 2-3 years). CONCLUSION: Most general emergency physicians report caring for children despite a lack of medical education in pediatrics. Pediatric medical education as well as collaborations between general practitioners and specialized pediatric teams should be enhanced to better match the needs of general emergency departments and improve the quality of primary and acute care for children.

Caffeine treatment for bronchiolitis-related apnea in the pediatric intensive care unit.

INTRODUCTION: Apnea is commonly encountered in children with bronchiolitis. Despite the lack of recommendations regarding bronchiolitis-related apnea (BRA) management, some pediatric intensive care unit (PICU) practitioners use caffeine treatment based on extrapolation from the recommendations for prematurity-related apnea management. The objectives of this study were to describe the management of BRA in our PICU, evaluate the caffeine prescription rate for this indication, and explore its potential effects on clinical outcomes. METHODS: This was a retrospective study in a university hospital PICU between January 1st, 2009 and December 31st, 2016. All children under 1 year of age admitted to the PICU with a diagnosis of BRA were included. Patients were allocated to a control group or a caffeine group depending on the administration of caffeine. RESULTS: In total, 54 infants were included and caffeine treatment was administered to 49 (91%) of them. Patient characteristics were similar between the two groups. Ventilatory support was initiated for 50 patients (93%). Supportive care and length of PICU stay were similar between the two groups. Caffeine was not associated with adverse events. CONCLUSION: Caffeine treatment in BRA could be considered as a local standard practice. This retrospective study was underpowered to show any benefit of caffeine treatment on clinical outcomes. This treatment was not associated with significant adverse effects. We raised the question of the appropriate caffeine dosing regimen for BRA in this postterm population. Further studies on this topic are warranted.

[Pasteurella multocida meningoencephalitis in a 14-year-old child]. / Méningo-encéphalite à Pasteurella multocida chez un enfant de 14 ans.

Pasteurella multocida is generally responsible for local infections through animal bites. It can be a cause of meningitis, which tends to affect people at the extreme ages of life or suffering from immunodeficiency. A 14-year-old teenager was hospitalized with typical signs of bacterial meningitis. P. multocida was evidenced in the cerebrospinal fluid. Progression was marked by a degradation on the 4th day of treatment, despite intravenous antibiotic therapy with third-generation cephalosporin, followed by a single seizure on the 7th day of treatment. The CT scan and magnetic resonance imaging showed pansinusitis but no intracerebral complications. Later progression was favorable, without neurological sequelae. The mode of contamination was inoculation via the upper airways with sinusitis. P. multocida meningitis is rare. The contamination does not always involve animal trauma.

[Life-threatening sleepwalking (Elpenor's syndrome) in a 10-year-old child]. / Somnambulisme avec mise en danger vital (syndrome d'Elpénor) chez un enfant.

Though benign in the majority of cases, sleepwalking sometimes causes injuries due, among other causes, to falls. Such accidents can be life-threatening - a situation that has been termed Elpenor syndrome (in reference to an accident experienced by a character in Homer's epic The Odyssey) - in particular when entailing defenestration. This syndrome has been described in adults and adolescents; we report here a case in a child. OBSERVATION: This 10-year-old girl was admitted at night to our hospital after a 3-m fall at home. She was alert (Glasgow score, 15) at admission; a frontal wound and a deformation of the right wrist were noted. Brain CT scans showed a frontal skull fracture and frontal lobe contusion, wrist x-rays showed a displaced right fracture. The patient underwent urgent neurosurgery (wound excision and suture after reduction of skull fracture) and closed reduction and immobilization of the wrist fracture, both under general anesthesia. She underwent a psychiatric assessment in the intensive care unit 3 days after her fall. She was alert, well-oriented in time and space, and spoke fluently. She had no memory of her fall, only remembering going to bed in the evening before the accident and waking up in the ambulance on the way to the hospital. She displayed no sign of a concurrent mental illness and no suicidal ideas. Her parents reported that the evening of the accident she and her two brothers had all fallen asleep about 11:00 pm while watching TV, in the double bed of the guest room, placed just beside its window. At approximately 1:00 am, her father, who was going to bed and had just made noise in the hall, heard a cry from the guest room. He entered the room immediately and saw the opened window and his daughter lying on the outside ground; the brothers only awakened after the fall. The family had returned 2 days before from a 6-month stay in the United States, with jet-lag, sleep deprivation, and a disorganized sleep/wake rhythm in the patient. There was no medication before the accident, no substance use (including caffeine), and no concurrent medical problem. Over the 2 preceding years, the patient had undergone two witnessed episodes of early-nighttime arousal with altered consciousness and calm wandering (including going downstairs on one occasion), both strongly suggesting sleepwalking. There was a history of sleepwalking in her father and her older brother. Life-threatening sleepwalking (Elpenor syndrome) was diagnosed. The child and her parents were educated about sleepwalking; regularization of sleep schedules and sleep extension (avoidance of sleep deprivation, short napping when possible) were prescribed. We also recommended securing the home (bed, windows, and stairways). No pharmacological treatment was instituted. During the following 18 months, the child manifested only one noted sleepwalking episode, without risk-taking. She had no neurological or psychopathological sequela from her accident, of which she never had a memory. CONCLUSION: Elpenor syndrome can occur in a child; consequently, it is important to inform parents of children with sleepwalking about the necessity of always securing the night-time environment.

[Management of patients under 18years of age by adult intensive care unit professionals: Level of training, workload, and specific challenges]. / Prise en charge de patients mineurs par les équipes de réanimation pour adultes : expérience pédiatrique, estimation de l'activité et difficultés ressenties par les soignants.

In routine practice, intensive care physicians rarely have to manage children under 18years of age, particularly those under 15. This study's objectives were to assess the quality of training in pediatrics of adult intensive care teams, to document the workload generated by care of pediatric patients, and to identify the difficulties encountered in managing minors as patients. A survey was administered in Lower Normandy from 4 April 2012 to 1 September 2012. Physicians, residents, nurses, and nurses' aides practicing in one of the nine intensive care units of Lower Normandy were asked to complete an electronic or paper format questionnaire. This questionnaire assessed their level of pediatric training, the workload management of pediatric patients entailed, and the challenges posed by these patients. One hundred and nine questionnaires were returned (by 26 attending physicians, 18 residents, 38 nurses, and 27 nurses' aides). Eighty-three of the respondents (76%) had no experience in a pediatric unit of any kind. Forty-two percent thought that the pediatric age range lies between 3months and 15years of age. However, more than 50% of respondents would like the upper limit to be 16years or even older. Ninety-three respondents (85%) estimated having some exposure to pediatric patients in their routine practice, but this activity remained quite low. Seventy-three (67%) reported difficulties with the management of these young patients. This survey provides current information regarding the level of training of adult intensive care unit professionals and their concerns about managing patients under 18years of age, both in terms of workload and specific challenges.

[Winter viral ecology in a pediatric intensive care unit: a prospective study]. / Étude prospective de l'écologie virale hivernale dans un service de réanimation pédiatrique.

INTRODUCTION: Viral respiratory infections are common in children, most of which are due to a virus. They can lead to serious infections, and these children may require treatment in a pediatric intensive care unit (PICU). This prospective study evaluated the epidemiology of respiratory viruses and associated illnesses among children hospitalized in a PICU during the three winter months of 2012-2013. METHODS: All the children admitted to the PICU, University Hospital of Caen, France, were included. Nasal swabs were collected and specimens were tested using a single real-time PCR (polymerase chain reaction). RESULTS: Of the 105 patients admitted to the PICU during the study period, 84 fulfilled the inclusion criteria. The "respiratory group" included 37 patients with respiratory symptoms at admission while the "nonrespiratory group" included 47 patients with no respiratory symptoms. The 84 nasal swabs collected included 54 that were considered positive (64.3%) and 70 viruses were detected. The most commonly detected virus was RSV (n=28; 40.0% positive samples), followed by HRV (n=24; 34.3%). Viruses were more frequently detected in the respiratory (86.5%) than in the nonrespiratory (42.6%) group (P<0.001). Statistical analysis by subgroups revealed that RSV infections were significantly more frequent in the respiratory group (54.1%) than in the nonrespiratory group (6.4%) (P<0.001). There was no difference for HRV (32.4% and 27.7%) or for the other viruses. No difference in duration of hospitalization or duration of mechanical ventilation was demonstrated depending on the virus detected. DISCUSSION: The use of the very sensitive multiplex PCR technique increased virus detection rates in both symptomatic and asymptomatic subjects. CONCLUSION: We have confirmed the frequency of RSV infections in a PICU and found that many patients without respiratory symptoms have respiratory infections caused by viruses. The impact of these infections on patient outcome should now be analyzed in order to demonstrate the role played by respiratory viruses.

[Verapamil intoxication: beware of the delayed effect]. / Intoxication aigüe au vérapamil : attention à l'effet retard.

Intoxication with calcium channel inhibitory drugs is rare but mortality rates reach 10 %. We report the case of a 5-year-old girl who had ingested five 240-mg tablets of extended-release verapamil (VLP) and a tablet of bromazepam. Thirty hours after the ingestion she had a vasoplegic shock, heart conduction disorders, and metabolic complications. She was treated in pediatric intensive care with continuous epinephrine and insulin and recovered completely 60h after the ingestion. This case underlines the danger of calcium channel blocker overdose, increased by the extended-release mechanism: the drug effect is to slow down gastric motility - which explains the latency of the symptoms - but this also increases the drug absorption, inducing a vicious circle. These mechanisms in calcium blocker intoxication need to be kept in mind in order to decrease the mortality of such accidents.

[Acanthosis nigricans in children and Crouzon syndrome]. / Acanthosis nigricans chez l'enfant et syndrome de Crouzon.

BACKGROUND: Crouzon syndrome with acanthosis nigricans is a rare form of Crouzon syndrome in which craniosynostosis and facial dysmorphism are associated with acanthosis nigricans. PATIENTS AND METHODS: Cutaneous examination of a 9-year-old child presenting bicoronal craniosynostosis revealed acanthosis nigricans of the cervical, axillar, inguinal and popliteal regions which appeared at the age of two. He had a dysmorphic face including a large forehead, hypertelorism, mid-face hypoplasia, prognathism and low-set ears. These clinical anomalies suggested a case of Crouzon syndrome with acanthosis nigricans, which was later confirmed by the finding of a mutation in the FGFR3 gene. DISCUSSION: Acanthosis nigricans in children is often a cutaneous marker of insulin resistance. However, it may also form part of diverse diseases, notably those of genetic origin. The association of craniosynostosis and acanthosis nigricans allows incrimination of the FGFR3 gene from the outset and diagnosis of Crouzon syndrome with acanthosis nigricans. In the present case, dermatological examination allowed an aetiology of craniosynostosis to be determined.

[Endovascular occlusion of the intracranial aneurysms--an alternative for surgical treatment].

Aneurysms on cerebral blood vessels due to their localization, size, relationship with neurovascular structures, even today, represent besides the constant progress of the diagnostic techniques and instruments in the operating theaters, a great therapeutic challenge. Gigantic aneurysms, aneurysms localized within the cavernous sinus and aneurysms of the vertebrobasilary confluence carry a great perioperative risk of mortality and morbidity. In fact, development of the endovascular procedures for the occlusion of their lumen made possible great progress in the treatment of aneurysms of this localization. In a not so small number of cases endovascular procedure means remodeling of the lumen of the main blood vessel. Endovascular obliteration of aneurysms has a certain advantage as regards the surgical treatment of aneurysms in which, on the basis of the expected natural course and presumed operative risk, may be judged that there exists unacceptably great operative risk. We present a personal series and results in 6 patients with aneurysms of various localizations.

Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease.

BACKGROUND: Pompe disease is a progressive metabolic neuromuscular disorder resulting from deficiency of lysosomal acid alpha-glucosidase (GAA). Infantile-onset Pompe disease is characterized by cardiomyopathy, respiratory and skeletal muscle weakness, and early death. The safety and efficacy of recombinant human (rh) GAA were evaluated in 18 patients with rapidly progressing infantile-onset Pompe disease. METHODS: Patients were diagnosed at 6 months of age and younger and exhibited severe GAA deficiency and cardiomyopathy. Patients received IV infusions of rhGAA at 20 mg/kg (n = 9) or 40 mg/kg (n = 9) every other week. Analyses were performed 52 weeks after the last patient was randomized to treatment. RESULTS: All patients (100%) survived to 18 months of age. A Cox proportional hazards analysis demonstrated that treatment reduced the risk of death by 99%, reduced the risk of death or invasive ventilation by 92%, and reduced the risk of death or any type of ventilation by 88%, as compared to an untreated historical control group. There was no clear advantage of the 40-mg/kg dose with regard to efficacy. Eleven of the 18 patients experienced 164 infusion-associated reactions; all were mild or moderate in intensity. CONCLUSIONS: Recombinant human acid alpha-glucosidase is safe and effective for treatment of infantile-onset Pompe disease. Eleven patients experienced adverse events related to treatment, but none discontinued. The young age at which these patients initiated therapy may have contributed to their improved response compared to previous trials with recombinant human acid alpha-glucosidase in which patients were older.

[Tricuspid valve endocarditis after umbilical piercing]. / Endocardite tricuspide après un piercing ombilical.

We report a case of a 16 years old girl who was affected by a septicemia with a septic arthritis of the wrist and a tricuspid endocarditis, 3 months after a navel piercing. The blood culture and the liquid of puncture showed Staphylococcus aureus. A treatment prolonged by intravenous antibiotics permitted a progressive regression of endocarditis, without surgical intervention. The piercing must be achieved in conditions of strict asepsis, it must be advised against to the patient with congenital heart disease. In case of realization of a piercing an antibiotic prophylaxis must be achieved, while using preference pristinamycin in case of cutaneous piercing and amoxicillin in case of mucous piercing. Indeed, infective endocarditis after body piercing are more often brought back with a generally sterner evolution that in our observation.

[Fulminant influenza type A associated myocarditis: a fatal case in an 8 year old child]. / Myocardite fulminante à virus Influenza A: à propos d'un cas fatal chez une enfant de 8 ans.

Fulminant Influenza type A-associated myocarditis are very rare in children. The clinical presentation is non specific, like flu illness, cardiogenic shock or sudden cardiac arrest. We report the case of a eight years old girl with a fulminant Influenza A-associated myocarditis with a fatal evolution despite the use of an extracorporeal membrane oxygenation (ECMO). The aim of this observation is to remind that influenza in children, usually considered as a benign illness, can exceptionally be complicated by a fulminant myocarditis. Because the possibility to recover a full myocardial function, the persistence of severe heart failure despite the medical treatment should conduct rapidly to a mechanical circulatory assistance.

[Acute myocarditis with Toxoplasma gondii. A case report in a newborn]. / Myocardite aiguë à toxoplasme. A propos d'un cas survenu chez un nourrisson.

Acute myocarditis with Toxoplasma Gondii in immunocompetent patients is rare and the paediatric cases touch old children. These myocarditis lead sometimes cardiac insufficiency and sometimes mimic a myocardial infarction. The evolution is often favorable, even when there is no pest-destroying treatment. We report a case of myocarditis toxoplasma Gondii with which has occurred in a 11 month and half old infant, whose evolution was favorable with a symptomatic and pest-destroying treatment. The interest of this observation is related to the scarcity of acute myocarditis caused by toxplasmosis in infant without immunoinsufficiency.

[Otogenic cavernous sinus thrombophlebitis. A case report]. / Thromboses otogènes du sinus caverneux. A propos d'un cas.

OBJECTIVES: Acquire knowledge concerning the diagnosis and treatment of otogenic cavernous sinus thrombophlebitis. PATIENT AND METHODS: We report a case of otogenic cavernous sinus thrombophlebitis (CST) in a 6-year-old boy. RESULTS: CST diagnosis was based on clinical signs (septic illness, ocular nerve palsy and chemosis), and neuro-imaging confirmed the diagnosis. Contrast-enhanced CT scan demonstrates different signs: (i) filling defect or heterogeneous enhancement of cavernous sinus, (ii) cavernous sinus enlargement with bulging of the lateral wall, (iii) intensive enhancement of lateral wall corresponding to enhancement of a collateral network replacing the thrombosed cavernous veins, (iiii) and sometimes indirect orbital signs (exophthalmus, densification of the retro-orbital fat, superior ophthalmic dilatation with partial or no enhancement in case of thrombosis extension). The bacteria most frequently found are Streptococci, Streptococcus pneumoniae, Staphylococcus aureus, Gram-negative rods and anaerobes combined. The therapeutic management of CST consists of intravenous administration of appropriate antibiotics combined radical mastoidectomy if medical therapy has failed. Anticoagulant therapy is controversial. CONCLUSION: Knowledge of early diagnosis of otogenic cavernous sinus is important because prior to the antibiotic era, CST was almost always fatal. Currently, therapeutic outcome is not always favorable due to high morbidity after aggressive treatment.

Benzdiamidine.

The molecule of benzene-1,4-dicarboxamidine or benzdiamidine, C(8)H(10)N(4), reveals C(i) symmetry. Hydrogen bonds utilize the amino groups as double donors, whereas the imino groups act as double acceptors. The network formed is similar to that observed in the crystal packing of terephthalamide.

Bis(amino acid) oxalyl amides as ambidextrous gelators of water and organic solvents: supramolecular gels with temperature dependent assembly/dissolution equilibrium.

Bis(LeuOH) (1a), bis-(ValOH) (2a) and bis(PhgOH) (5a) (Phg denotes (R)-phenylglycine) oxalyl amides are efficient low molecular weight organic gelators of various organic solvents and their mixtures as well as water, water/DMSO, and water/DMF mixtures. The organisational motifs in aqueous gels are dominated primarily by lipophilic interactions while those in organic solvents are formed by intermolecular hydrogen bonding. Most of the gels are thermoreversible and stable for many months. However, 2a forms unstable gels with organic solvents which upon ageing transform into variety of crystalline shapes. For some 1a/alcohol gels, a linear correlation between alcohol dielectric constants (epsilon) and gel melting temperatures (Tg) was found. The 1H NMR and FTIR spectroscopic investigations of selected gels reveal the existence of temperature dependent network assembly/dissolution equilibrium. In the 1H NMR spectra of gels only the molecules dissolved in entrapped solvent could be observed. By using an internal standard, the concentration of dissolved gelator molecules could be determined. In FTIR spectra, the bands corresponding to network assembled and dissolved gelator molecules are simultaneously present. This enabled determination of the Kgel values by using both methods. From the plots of InKgel versus 1/T, the deltaHgel values of selected gels have been determined (-deltaHgel in 10-36 kJ mol(-1) range) and found to be strongly solvent dependent. The deltaHgel values determined by 1H NMR and FTIR spectroscopy are in excellent agreement. Crystal structures of 2a and rac-5a show the presence of organisational motifs and intermolecular interactions in agreement with those in gel fibres elucidated by spectroscopic methods.

Intermolecular contacts in the crystal packing of 2,2'-(N,N'-oxalyldiimino)bis(3-phenylpropanamide) dimethyl sulfoxide solvate.

In the title compound, C20H22N4O4*C2H6OS, two distinct hydrogen-bond systems connect oxalamide groups in one pattern and primary amide groups in the other to form a two-dimensional network perpendicular to the c axis. These hydrophilic layers are joined to the three-dimensional structure through C--H...pi interactions. The hydrogen-bonded waved layers shape holes which are occupied by disordered dimethyl sulfoxide solvent molecules.

meso-N,N'-oxalyldivaline.

The title compound, 2,2'-(oxalyldiimino)bis(3-methylbutanoic acid), C12H20N2O6, possesses a centre of symmetry. In the crystal, molecules are connected by hydrogen bonds between oxamide and carboxyl groups, similar to the pattern of the monoclinic forms of HO-Gly-CO-CO-Gly-OH and HO-Aib-CO-CO-Aib-OH (Gly is glycine and Aib is 2-aminoisobutyric acid). The characteristic torsion angles in the title compound are close to those in peptide alpha-helices.

The synthesis of novel 6,5- and 6,6-membered fused heterocyclic compounds derived from thymine.

Novel pyrimido[1,2-a]pyrimidinones 14, 15 and 16 and imidazo[1,2-a] pyrimidinones 19 and 20, designed as conformationally constrained analogues of 1-(3-amino-2-hydroxypropyl)thymine and 1-(2-amino-3-hydroxypropyl)thymine, respective ly, were synthesized by the ring-opening/ ring-closure rearrangement of the corresponding byciclic oxygen-containing amino compounds 12 and 17.