Multidisciplinary advisory teams to manage multidrug-resistant tuberculosis: the example of the French Consilium.

SETTING: The World Health Organization (WHO) recommends that multidrug-resistant tuberculosis (MDR-TB) treatment should be managed in collaboration with multidisciplinary advisory committees (consilia). A formal national Consilium has been established in France since 2005 to provide a centralised advisory service for clinicians managing MDR-TB and extensively drug-resistant (XDR-TB) cases.OBJECTIVE: Review the activity of the French TB Consilium since its establishment.DESIGN: Retrospective description and analysis of the activity of the French TB Consilium.RESULTS: Between 2005 and 2016, 786 TB cases or contacts of TB cases were presented at the French TB Consilium, including respectively 42% and 79% of all the MDR-TB and XDR-TB cases notified in France during this period. Treatment regimens including bedaquiline and/or delamanid were recommended for 42% of the cases presented at the French TB Consilium since 2009. Patients were more likely to be presented at the French TB Consilium if they were born in the WHO Europe Region, had XDR-TB, were diagnosed in the Paris region, or had resistance to additional drugs than those defining XDR-TB.CONCLUSION: The French TB Consilium helped supervise appropriate management of MDR/XDR-TB cases and facilitated implementation of new drugs for MDR/XDR-TB treatment.

Potentially preventable tuberculosis cases in children exposed to a contaminant case.

Early screening is recommended in children exposed to a contagious case of tuberculosis (TB), to prevent rapid progression to active TB. The aim of this study was to evaluate the percentage of potentially preventable cases of pediatric TB stemming from inadequate screening. The data gathered on children aged 0 to 10 years, who were evaluated by the Paris Center for TB Control (CLAT75) between January 2009 and December 2013, were extracted and retrospectively analyzed. French National Guidelines for screening were used as reference. During the study period, 1232 children 0-10 years were screened, because of a known exposure to an index case, including 124 (10%) with criteria for latent tuberculosis infection (LTBI) and 26 (2%) with active TB. Twelve additional cases of TB were reported, diagnosed based on symptoms or systematic exams. As a whole, 68% of pediatric TB cases were diagnosed at screening around an adult index case, highlighting the quality of the screening network. Among the 38 TB cases, 19 (50%) had a missed opportunity for potential prevention, due to the absence of screening despite a known contaminant (n=2) or to screening not in compliance with current recommendations (n=17). Delayed first evaluation was the most frequent error of the screening procedures. In conclusion, despite the quality of the screening network set up in Paris, half of the pediatric TB cases in this study did not undergo the recommended screening procedures. A significant reduction in the number of pediatric TB cases can be expected through the optimization of screening networks.

[Newly formed French residents in pediatrics are not well prepared for conducting pediatric resuscitation after medical school]. / Le deuxième cycle des études médicales prépare-t-il les étudiants hospitaliers à leurs fonctions d'internes ? Exemple de l'arrêt cardiaque de l'enfant.

BACKGROUND: Medical schools aim to prepare medical students for their residency responsibilities. However, in France, there is no assessment of medical students' skills when they start their residency. GOAL: The objective of this study was to assess the quality of basic life support delivered by first-year residents in pediatrics during a simulated pediatric cardiopulmonary arrest. MATERIALS AND METHODS: First-year residents in pediatrics were assessed during a simulated pediatric cardiopulmonary arrest. Their performance score (based on adherence to international guidelines) and no-flow and no-blow fractions were recorded. RESULTS: Forty-two first-year residents were evaluated. Their median performance score was 4 out of 13. No-blow and no-flow fractions were 55 and 81 %, respectively. There was no correlation between their skills and their knowledge assessed during the national ranking exam at the end of the 6th year of medical school. CONCLUSION: At the beginning of their residency, pediatric residents are not able to properly provide basic life support. The introduction of simulation in French medical schools may be an effective way to improve their skills.

[Why use long-term macrolide therapy in pediatric pulmonology?]. / Pourquoi utiliser les macrolides au long cours en pneumologie pédiatrique ?

Macrolides are well-known antibiotics exerting antimicrobial as well as anti-inflammatory and immunomodulatory effects. Since the observation of a dramatic improvement in lung disease and survival in patients with diffuse panbronchiolitis, macrolides have been used over the long term in several chronic respiratory diseases. This review describes the results of trials that have evaluated long-term macrolides in the treatment of cystic fibrosis, non-cystic fibrosis bronchiectasis, and asthma, particularly focusing on the impact on children. It also provides new insights on the potential effects of macrolides on diffuse parenchymal lung diseases.

[Bronchopulmonary dysplasia-associated pulmonary arterial hypertension of very preterm infants]. / Hypertension artérielle pulmonaire des anciens grands prématurés bronchodysplasiques.

Bronchopulmonary dysplasia (BPD) of very preterm infants is a multifactorial chronic lung disease and its incidence has not decreased despite improvements in neonatal intensive care, including lung protective strategies. Pulmonary hypertension (PH) can complicate the course of BPD. Mortality in infants with BPD-associated PH is thought to be very high, but its incidence is unknown and a standard diagnostic and therapeutic strategy has not been well defined. In this article, we will first describe the current knowledge on the BPD-associated PH and the current treatments available for this pathology. We will then present the HTP-DBP Study, carried out in Paris (France) starting in 2012. The diagnosis of PH is suspected on echocardiographic criteria, but cardiac catheterization is considered the gold standard for diagnosis and evaluation of the severity of PH. Moreover, pulmonary vasoreactivity testing is used to guide the management of patients with PH. The pathogenesis of BPD-associated PH is poorly understood and even less is known about appropriate therapy. Today, optimizing ventilation and reducing the pulmonary vascular tone with specific pulmonary vasodilatator drugs are the main goals in treating HTP-associated DBP. Animal studies and a few clinical studies suggest that medications targeting the nitric oxide (NO) signaling pathway (NO inhalation, oral sildenafil citrate) could be effective treatments for BPD-associated PH, but they have not been approved for this indication. The HTP-DBP study is a French multicenter prospective observational study. The objective is to evaluate the frequency of BPD-associated PH, to describe its physiopathology, its severity (morbidity and mortality), and the effectiveness of current treatments.

New surfactant protein C gene mutations associated with diffuse lung disease.

BACKGROUND: Mutations in the surfactant protein C gene (SFTPC) have been recently associated with the development of diffuse lung disease, particularly sporadic and familial interstitial lung disease (ILD). OBJECTIVE: We have investigated the prevalence and the spectrum of SFTPC mutations in a large cohort of infants and children with diffuse lung disease and suspected with surfactant dysfunction. METHOD AND RESULTS: 121 children were first screened for the common SFTPC mutation, p.Ile73Thr (I73T). Ten unrelated patients were shown to carry this mutation. The I73T mutation was inherited in six cases, and appeared de novo in four. The 111 patients without the I73T mutation were screened for the entire coding sequence of SFTPC. Of these, eight (seven unrelated) subjects were shown to carry a novel mutant allele of SFTPC. All these seven new mutations are located in the BRICHOS domain except the p.Val39Ala (V39A) mutation, which is in the surfactant protein C (SP-C) mature peptide. CONCLUSIONS: Our results confirm that SFTPC mutations are a frequent cause of diffuse lung disease, and that I73T is the most frequent SFTPC mutation associated with diffuse lung disease.