Gestational Age, Socioeconomic Context and Infection-Related Hospital Admissions of Infants Born With Gestational Age Less Than 33 Weeks.

Using data from a regional medical follow-up network database of preterm infants born with gestational age (GA) <33 weeks, we found that low GA and deprived socioeconomic neighborhoods increased incidence of infection-related hospitalization during the first year of life. Respiratory tract infections rates were higher in extremely preterm infants.

Les soignants de périnatalité face à la COVID-19 : stress, qualité de vie et préoccupations Perinatal caregivers coping with covid-19 : stress, quality of life and concerns.

AIMS: The first wave of the COVID-19 pandemic generated "risks" and uncertainties as well as organizational changes among French perinatal caregivers. Our study aimed to investigate the psychosocial impact of the first wave on this population. METHOD: Our participants (N=565) were invited to answer an online questionnaire that included questions on various indices of health and quality of life at work (e.g., ProQoL, perceived stress) and other questions on the impact of the pandemic on work organization. An open-ended question was designed to identify the participants' three most frequently perceived preoccupations with regard to the health situation. RESULTS: In addition to highlighting the multifactorial nature of participants' preoccupations, our results illustrated the effect of professional status and type of motherhood on the different indices of health and quality of life at work. When it was found that the pandemic had an impact on work organization and on teams, lower health and quality of work life scores were recorded. On the other hand, when positive impacts on organization were reported, mainly in terms of reduced work intensity, they were associated with higher health and quality of work life scores. CONCLUSION: We explain this last result as either one actual effect of the pandemic on work organization, or as a phenomenon of cognitive rationalization.

Real-life study of the role of high-flow nasal cannula for bronchiolitis in children younger than 3 months hospitalised in general pediatric departments.

We aimed to describe the real-life role of high-flow nasal cannula (HFNC) for bronchiolitis in infants under 3 months of age admitted to three general pediatric departments during the 2017-2018 epidemic period. We retrospectively assessed the clinical severity (Wang score) for every 24-h period of treatment (H0-H24 and H24-H48) according to the initiated medical care (HFNC, oxygen via nasal cannula, or supportive treatments only), the child's discomfort (EDIN score), and transfer to the pediatric intensive care unit (PICU). A total of 138 infants were included: 47±53 days old, 4661±851.9 g, 70 boys (50.7%), 58 with hypoxemia (42%), Wang score of 6.67±2.58, 110 (79.7%) staying for 48 consecutive hours in the same ward. During the H0-H24 period, only patients treated with HFNC had a statistically significant decrease in the severity score (n=21/110; -2 points, P=0.002) and an improvement in the discomfort score (n=15/63; -3.8 points, P<0.0001). There was no difference between groups during the H24-H48 period. The rate of admission to the PICU was 2.9% for patients treated for at least 24 h with HFNC (n=34/138, 44% with oxygen) versus 16.3% for the others (P=0.033). Early use of HFNC improves both clinical status and discomfort in infants younger than 3 months admitted for moderately severe bronchiolitis, whatever their oxygen status.

Pediatric emergency room visits for neurological conditions: Description and use of pediatric neurologist advice.

INTRODUCTION AND OBJECTIVE: Information on the spectrum and frequencies of pediatric neurological presentations to emergency departments is vital to optimize quality of care. The objective of this study was to determine the incidence of pediatric neurological emergencies and to analyze the impact of specialist neurological advice in emergency care. PATIENTS AND METHODS: We performed a retrospective descriptive study of pediatric emergency room visits for neurological reasons at the Timone University Hospital in Marseille over a 6-month period (from October 2017 to March 2018). RESULTS: Of the 14,572 emergencies analyzed, 370 (2.5%) were for neurological conditions. These were most commonly seizures (56.7% of cases), headache (19.7%), and motor or sensory deficits (5.1%). The most frequent diagnosis was epileptic seizure (30%), followed by febrile seizure (26.1%) and migraine (15%). Around two in every five patients (37.6%) required hospitalization. Neurological emergencies requiring critical care occurred at a frequency of about one per month (1.6% of cases). A pediatric neurologist was consulted in 37.3% of cases, resulting in a modification of the diagnosis or treatment in 66% of these referrals. CONCLUSION: The results of this study suggest that a formal referral system between the emergency department and pediatric neurologists would be useful.

[Complementary exams in child abuse: A French national study in 2015]. / Explorations complémentaires face à une situation d'enfant en danger : état des lieux des pratiques en France en 2015.

INTRODUCTION: Even if there are HAS (French National Health Authority) guidelines on shaken baby syndrome, many other child abuse situations are not included in these recommendations. The aim of this study was to invent the complementary exams in cases of child abuse in France and compare the practice to existing guidelines. MATERIAL AND METHODS: This was a multicenter study by email to 128 French hospitals (35 university hospitals and 93 general hospitals) that receives children in emergency and hospitalization settings. Three child abuse clinical situations were included in a clinical case multiple-choice format concerning the further explorations. We described the main results and evaluated their adherence to the HAS protocol for case 1. RESULTS: Of 128 hospitals surveyed, 104 responded, for an 81 % response rate, which corresponded to 274 doctors. Analysis of the results showed great heterogeneity in practices. The majority of physicians (99 %) performed systematic explorations in the situation of physical abuse, while only 27 % undertook such exams in situations of serious neglect. The situation of sexual abuse was the most consensual in terms of diagnostic tests for the detection of sexually transmitted diseases, but other types of associated abuse were not sought. In the first case, the HAS guidelines were respected in less than half of the cases for all complementary exams except the eye fundus exam. Abdominal imaging was insufficiently performed (40 % of cases). Examinations that were not indicated were still prescribed. Moreover, siblings under 2 years of age were examined in only one-third of cases (n=88/274; 32 %). Practices were not influenced by the age of the child. CONCLUSION: This study illustrates the heterogeneity in the use of complementary exams in cases of child abuse in France. Common protocols throughout the country would be useful, standardizing the most relevant exams for potential medical-legal issues, and facilitating exchanges concerning practices between different centers.

Melioidosis in New Caledonia: a dominant strain in a transmission hotspot.

Melioidosis is an infectious disease caused by Burkholderia pseudomallei, a bacterium endemic in Southeast Asia and northern Australia. In New Caledonia, sporadic cases were first described in 2005; since then, more cases have been identified. To improve our understanding of melioidosis epidemiology in New Caledonia, we compared the local cases and B. pseudomallei isolates with those from endemic areas. Nineteen melioidosis cases have been diagnosed in New Caledonia since 1999, mostly severe and with frequent bacteraemia, leading to three (16%) fatalities. All but one occurred in the North Province. Besides sporadic cases caused by non-clonal strains, we also identified a hotspot of transmission related to a clonal group of B. pseudomallei that is phylogenetically related to Australian strains.

[Sexual behaviour in schizophrenic patients: the impact of antipsychotics]. / Comportements sexuels chez le patient schizophrène: impact des antipsychotiques.

In spite of the frequency of sexual dysfunction in schizophrenic patients and antipsychotic-treated schizophrenic patients, few studies have been performed. The relationship of schizophrenia to sexual pathology is variable and complex, and of course different between men and women. Few evaluation methods have been proposed or validated. Antipsychotics may improve some aspects of sexual behaviour in schizophrenic patients. However, sexual dysfunction is also a possible side effect of these drugs. The evaluation of antipsychotics is often restricted to prolactin measurement, the relationship with sexual disorders of which has not been fully established. Preliminary data suggest that the capacity to induce sexual disorders differs from one antipsychotic to another. The available data on the mechanisms of sexual dysfunction, the pharmacological profile and the sexual effects of classical neuroleptics (haloperidol and thioridazine) and second generation antipsychotics available in France (amisulpride, clozapine, risperidone, olanzapine) are reviewed.

Late-onset metachromatic leukodystrophy: genotype strongly influences phenotype.

BACKGROUND: P426L and I179S are the two most frequent mutations in juvenile and adult metachromatic leukodystrophy (late-onset MLD), which, in contrast to infantile MLD, show marked phenotypic heterogeneity. OBJECTIVE: To search for genotype-phenotype correlations in late-onset MLD. METHODS: The authors reviewed the clinical course of 22 patients homozygous for mutation P426L vs 20 patients heterozygous for mutation I179S, in which the second arylsulfatase A (ASA) mutation had also been determined. RESULTS: P426L homozygotes principally presented with progressive gait disturbance caused by spastic paraparesis or cerebellar ataxia; mental disturbance was absent or insignificant at the onset of disease but became more apparent as the disease evolved. In contrast, compound heterozygotes for I179S presented with schizophrenia-like behavioral abnormalities, social dysfunction, and mental decline, but motor deficits were scarce. Reduced peripheral nerve conduction velocities and less residual ASA activity were present in P426L homozygotes vs I179S heterozygotes. CONCLUSION: The characteristic clinical differences between homozygous P426L and compound heterozygous I179S patients establish a distinct genotype-phenotype correlation in late-onset metachromatic leukodystrophy.

Coincidence of two genetic forms of Charcot-Marie-Tooth disease in a single family.

The authors report a family in which two affected first cousins had a severe demyelinating Charcot-Marie-Tooth disease (CMT) phenotype. One had related parents, and there were no other affected relatives, suggesting an autosomal recessive mode of inheritance. Molecular studies showed that a de novo duplication in 17p11.2 and a second mutation in MTMR2 were present.

A randomized clinical trial comparing doses and efficacy of lormetazepam tablets or oral solution for insomnia in a general practice setting.

Lormetazepam is a short-acting benzodiazepine hypnotic which is beneficial in shortening the time to onset of sleep. The aim of the study was to assess a new formulation of lormetazepam (oral solution) in comparison with lormetazepam tablets in out-patients with insomnia. This trial was an open randomized parallel group study conducted by 30 general practitioners. One hundred and eight patients took 0.5 mg on the first night and were allowed to increase their dosage by 0.25 mg (for oral solution) and 0.5 mg (for tablets), respectively, each day and every 2 days. The patients assessed the efficacy, acceptability and tolerance of lormetazepam using a diary card and a set of visual analogue scales assessing their sleep. Over 14 days of treatment, the mean daily dose of lormetazepam was lower in the oral solution group than in the tablets group (0.78 mg versus 0.97 mg). The cumulated dose of lormetazepam was lower with the oral solution (18% reduction). No significant difference between the two groups was found in the assessment of sleep characteristics. The occurrence of side effects did not differ between the two groups. These results suggest that a unitary dose as achieved by an oral solution of lormetazepam allows easier determination of the minimal individual effective dose.

Amphetamine-like stimulant cessation in an abusing patient treated with bupropion.

OBJECTIVE: Bupropion sustained release is considered to be a weak inhibitor of dopamine and serotonin reuptake. METHOD: We report the case of an amphetamine-abusing patient who self-administered bupropion. RESULTS: Since 30 years, a 52-year-old women used amphetamine derivates. She explained her need for amphetamine use in order to perform daily activities. Recently, she decided to experiment with bupropion. She abruptly stopped taking clobenzorex and simultaneously started taking bupropion (150 mg/day). The seventh day she reported a concomitant intake of clobenzorex; this induced adverse effects. Whilst taking bupropion, she described experiencing an euthymic state without any compulsion to take amphetamine drugs and was able to perform daily activities. After stopping it, no symptoms of withdrawal were reported by the patient. CONCLUSION: This observation supports an another report suggesting that bupropion may be of help in weaning from amphetamine users and should be confirmed by clinical trials.

[Review of quality of life instruments used in end-stage renal disease]. / Qualité de vie et insuffisance rénale chronique terminale: le point sur les différents questionnaires existants.

Health related quality of life (HRQOL) indicators take into account the personal perception of health, and are proposed as an alternative for efficacy indicators in medical and therapeutic decision making. They provide, due to elaboration and validation of a questionnaire, a standardised assessment of the health status perception. This paper provides a review of a variety of HRQOL instruments developed for patients suffering end-stage renal disease (ESRD). Generic instruments are designed to be applicable in general population and disease-targeted instrument are potentially more sensitive to the characteristics of a specific population. Among HRQOL instruments, we found 4 generic questionnaires (the Sickness Impact Profile, the SF 36, the Nottingham Health Profile and the EQ-5D), 3 disease-targeted questionnaires developed for ESRD patient undergoing dialysis (the Kidney Disease Quality of Life instrument, the Kidney Disease Questionnaire and the Choice Health Experience Questionnaire), 1 questionnaire specific for ESRD patients (the HRQOL questionnaire), and 2 specific disease-targeted instruments for renal transplant (the Kidney Transplant Questionnaire and the ESRD Symptom Checklist-Transplantation Module). In France, very few studies on the quality of life of ESRD patients were published; no specific questionnaire validated in French is yet published.

Autonomic and respiratory dysfunction in Charcot-Marie-Tooth disease due to Thr124Met mutation in the myelin protein zero gene.

OBJECTIVE: To report the clinical and electrophysiological characteristics of a family presenting Charcot-Marie-Tooth disease (CMT) associated with autonomic nervous system disturbances. METHODS: We studied nerve conduction values, postural adaptation, sympathetic skin reflex, the variation in heart rate by the Valsalva ratio and pupillometry in 7 members of a French family in which CMT due to a Thr124Met mutation in the myelin protein zero (MPZ) gene was diagnosed. RESULTS: Clinical and laboratory evidence of autonomic nervous system disturbances were found in the affected individuals. The clinical phenotype was characterized by sensorimotor peripheral neuropathy, defined as axonal type by electrophysiological studies, and was associated with severe pain, bladder dysfunction, sudorimotor disturbances and abolished pupillary reflex to light. Moreover, two patients had severe restrictive respiratory insufficiency requiring noninvasive mechanical ventilation. CONCLUSIONS: Our study demonstrates that autonomic disturbances may be one of the major clinical signs associated with CMT secondary to MPZ gene mutation in codon 124. Testing of pupillary reflex allows the discrimination of affected and unaffected subjects in our family. However, involvement of the autonomic nervous system in this type of neuropathy is unclear and further studies are required to elucidate the role of the MPZ gene in the autonomic nervous system.

[Reaction surveillance: organization in public care/hospitals in Marseille]. / Réactovigilance: quelle organisation à l'Assistance publique-Hôpitaux de Marseille ?

For the French Drug Agency (AFSSAPS), the aim of the reactovigilance is to monitor the adverse effects resulting from the use of IVD-MD. More precisely, the goal is to evaluate all incidents and risks that can result in inadequate results or negative effects to the user and therefore for the patient. According to the French Law and before the transposition in the French Law of the European Guideline 98/79/CE, the University Hospital of Marseille has decided to organize its own reactovigilance network. Since 2001, an institutional organization has been set up. For each of the 33 labs of the University hospital one reactovigilant has been nominated as well as one coordinating reactovigilant. Specialized structures have been created: one central committee and one experts group. Standardized operating procedures have been established. The reactovigilance system is also integrated to the vigilance coordination network of the Marseille University Hospital. This organization allows to achieve tracability of all alerts, as well as information and training for the professionals. Four missions are defined: collection and management of all incidents IVD-MD-related; diffusion of AFFSAPS and industrials alerts to all labs in order to take appropriate measures; Tracability of alerts and incidents in all labs and in central committee, tracability of reactives according to French guidelines (GBEA). For the first 10 months of 2002, 46 alerts have been forwarded to all lab's correspondants. In the same period, one real adverse event has been locally notified and after analyse will be forwarded to the AFSSAPS. All these factors should contribute to the health professionals interest and participation in reactovigilance activities.

Weight gain profiles of new anti-psychotics: public health consequences.

Anti-psychotic medications are an important therapeutic option for many individuals with schizophrenia. Recently, a growing interest has been observed on weight gain, which is now a well-known adverse effect of many anti-psychotics. As obesity is frequently a comorbid condition with schizophrenia, patients with schizophrenia are inherently at increased risk of developing obesity-related conditions such as cardiovascular disease and type 2 diabetes. The consequences of excessive weight gain (obesity) associated with anti-psychotic drugs are likely to include adverse effects on health, social burden and poor compliance or even discontinuation of therapy by the patients. In this article, we focus on different aspects of weight gain induced by anti-psychotics. This review comprises the following sections: (i) the pharmacological basis of anti-psychotic-induced weight gain and metabolic effects with a review of all anti-psychotics that can be used in patients with schizophrenia; (ii) the clinical impact of the body weight gain (morbidity, psychatric consequences, mortality); (iii) the management of obesity (identification of risk factors including pharmacogenetics, diet, behavioural therapies, pharmacological approach). An understanding of these aspects is important for those who prescribe anti-psychotics in order to provide the patient the best therapeutic management.

[Effects of a subanaesthetic dose of ketamine on emotional and behavioral state in healthy subjects]. / Evaluation psychocomportementale de l'administration de faible dose de kétamine chez le sujet sain.

Schizophrenic patients suffer from positive (delusions, hallucinations) and negative signs (social withdrawal) as well as emotional disturbance that included quantitative (blunted affect) and qualitative impairments (discordance of emotional level). Ketamine, a phencyclidine derivative, is a non competitive N-methyl-D-aspartate (NMDA) glutamate receptor antagonist. In healthy subjects its administration induces some positive symptoms (perceptual distortions.), negative symptoms (emotional deficit, apathy, social withdrawal) and cognitive changes (memory impairments and perseverations) that resemble some aspects of the symptoms of schizophrenia. A double blind cross over, placebo controlled was performed in 12 normal subjects with 2 sessions separated by one week of wash-out to determine ketamine-induced effects on behavioral and emotional responses. During each session, subjects received either ketamine or placebo (saline) infusion. A subanesthetic dose of ketamine (0,5 mg/kg) was administered by constant perfusion over 60 min. Behavioral and cognitive responses were assessed using positive and negative symptoms scales (BPRS, items from SAPS and SANS), vigilance and mood visual analog scale, subjective feelings using the Addiction Research Center Inventory (ARCI) and the Profile of Mood States (POMS). Using Philippot's method, emotions were elicited by films segments which induce a diversity of predictable emotions (fear, anger, sadness, joy, disgust and neutral state) and emotional responses were assessed by the Differential Emotions Scale (DES Izard). Low dose of ketamine induced significant effects on 7-items BPRS score (positive and negative items) and significant effects on positive and negative symptoms from SANS and SAPS. This was associated with emotional blunting of visually-induced responses that resemble aspects of schizophrenic emotional impairments. Ketamine impaired ARCI subscales (benzedrine subscale, pentobarbital-chlorpromazine subscale and LSD subscale). The recent findings of ketamine's pharmacology and imaging studies allow to draw several hypothesis related to neurotransmitter systems (glutamate, dopamine, serotonin interactions) and cerebral areas (particularly prefrontal cortex, anterior cingulate cortex, hippocampus) underlying some of these ketamine-induced effects.

[Treatment of non-dopamine-dependent signs in parkinsonian syndromes: evaluation and results]. / Traitements des signes non dopamine-dépendants des syndromes parkinsoniens: évaluation et résultats.

With dopaminergic systems, non dopaminergic neurotransmission probably plays a major role in parkinsonian syndromes (Multiple System Atrophy, Progressive Supranuclear Palsy, Pure Autonomic Failure, Cortical basal degeneration, Lewy Body Disease). A better understanding of the pathophysiology of these syndromes led to the development of molecules that interact with non dopaminergic systems. Thus, freezing, gait and balance disorders, dysautonomia and neuropsychiatric disorders are likely to benefit from specific treatments. However, due to methodological difficulties related to the evaluation of such molecules, controlled trials are rather rare and the results are often partial and sometimes unclear.

[The major trends of the French accreditation process. A review of the first 182 reports published]. / Les grandes tendances de l'accréditation. Bilan des 182 premiers comptes-rendus publiés.

OBJECTIVES: To reply to the following questions: what is the status of the accreditation process and what are the general trends pointed out by the experts-visitors ? METHODS: A study was conducted on the 182 accreditation reports regarding health centres, published by the Anaes from June 2000 to July 2002. A reading scale was developed and data was registered in the database. Statistical analysis first described the hospitals (legal status, location, size, number of sites, presence or lack of technical devices, emergency units, and consultations) and then matched them with the specific parameters of the accreditation process (date of involvement, date of audit, date of the accreditation deliberation, number of experts and visitors and number and type of decisions. RESULTS: The results underlined the most frequent decisions among the 10 referentials of the accreditation process. The patients' case reports and the management of quality and prevention predominated. Recent French laws and the decision of the Anaes to apply these themes in their strategic orientation can explain this. COMMENTS: The large hospital size and multi-sites organisation appeared to be negative elements and justify the apprehension felt by the 'major hospitals'.

[Factors influencing the appropriation of clinical guidelines by physicians]. / Facteurs influençant l'appropriation des recommandations médicales par les médecins.

DEFINITION: Clinical guidelines (CG) constitute a means to facilitate the decision-making process, defining what is and is not appropriate in fields concerned with public health matters or unregulated practices. METHOD: A bibliographical review has been conducted on factors likely to influence physicians' compliance to CG. These factors involve physicians, the guidelines themselves and the actions taken to encourage their implementation. FACTORS RELATED TO PHYSICIANS: In physicians' attitudes, we find not only their propensity to ask for help, to admit mistakes and weakness, but also their wish for continuing medical education. Practicing in an establishment or group consulting offices enhances their awareness of CG. The use of CG and the consequences of their implementation, as well as legal or financial concerns, increase physicians' confidence in the benefits of CG for themselves and their patients. FACTORS RELATED TO GUIDELINES: Clinical guidelines should be issued by multi-professional groups, among which there should be recognized experts and day-to-day specialists. Their access should be easy and their updating frequent. The adaptation to the individual conditions of local practices is essential. THE IMPORTANCE OF IMPLEMENTATION: Once established, an intensive dissemination and active appropriation policy should be enacted. On the other hand, it is unlikely that passively diffused and non-updated CG will be adopted by newly qualified or long-established physicians installed in routine practice. Account must also be taken of the nature of certain CG, considered restrictive or coercive, and the targeted physician's attitudes and practicing habits - which is still unusual in France.

A comprehensive endocrine description of Kennedy's disease revealing androgen insensitivity linked to CAG repeat length.

Our study aims to provide a comprehensive view of the endocrine features in Kennedy's disease (KD). Twenty-two men with KD underwent detailed endocrine investigations. Clinical signs of partial androgen resistance were present in more than 80% of the patients, with gynecomastia being the most prominent. Gynecomastia was postpubertal but appeared before muscular weakness in most cases. Thirteen patients had alteration of testicular exocrine function. Hormonal profile of partial androgen resistance was present in 86% of the patients, with an elevated testosterone level in 68%. Androgen insensitivity seems to appear later in life in KD, similar to the development of neurological signs. Although we confirm the previously reported correlation between the CAG repeat length and the early onset of the neurological disease, we describe a significant correlation between repeat length and the age of onset of gynecomastia as well as biological indexes of androgen insensitivity. This is supported by numerous in vitro data correlating variations in the CAG tract with androgen receptor activity; the longer the CAG repeats, the weaker the receptor transactivation. Ours is the first study to show such a clear and prominent pattern of androgen insensitivity in KD. In clinical practice, KD patients are often misdiagnosed as having amyotrophic lateral sclerosis. Careful examination of the endocrine component could avoid such a deleterious misdiagnosis.