French national diagnostic and care protocol for Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.

A rare chest tumor in a 7-year old girl with a neurodegenerative disease.

We report the case of a 7-year-old girl with a history of San Filippo disease who presented with gingivitis and painful chest tumefaction. Microbiology of this tumefaction identified Aggregatibacter actinomycetemcomitans (AA), a slowly growing, commensal, Gram negative bacillus that is a very unusual cause of thoracic infection. We discuss this case in the light of available literature of pediatric cases of AA thoracic infection. Conclusion: a tumor-like chest mass in a patient with multiple disabilities should evoke an invasive AA infection.

Severe adenovirus pneumonia with hemophagocytic syndrome and respiratory failure.

We report the case of an 18-month-old infant with severe serotype 3 adenovirus pneumonia, exceptionally associated with hemophagocytic syndrome. Treatment included cidofovir and mechanical ventilation for 13 days. The child developed chronic respiratory insufficiency due to bronchiectasis and bronchiolitis obliterans.

[Bronchiectasis revealing triple A syndrome]. / Une dilatation de bronches révélant un syndrome du triple A.

We report on the case of a 3-year-old child presenting bilateral bronchiectasis due to recurrent pneumonia with esophageal achalasia. The final diagnosis was triple A syndrome. This presentation is particularly atypical and rare at this age.

[Chest radiographs in infants hospitalized for acute bronchiolitis: real information or just irradiation?]. / La radiographie de thorax chez le nourrisson hospitalisé pour bronchiolite aiguë : réelle information ou simple irradiation ?

OBJECTIVE: To describe the chest radiographs of infants hospitalized for acute bronchiolitis and to assess whether patient management changed after radiography. STUDY DESIGN: All infants hospitalized in our pediatric unit with a first episode of acute bronchiolitis from October 2010 to March 2012 were included in the study. Infants with chronic disease or transferred from a pediatric intensive care unit were excluded. The following data were collected: sex, age, neonatal history, atopy, tobacco exposure, admission criteria, treatment, laboratory parameters, ultrasonography and its outcome, results of chest radiography on admittance, the reason for a second chest radiograph, change in management as a result of the radiograph. RESULTS: The study comprised 232 infants (median age 2.2 months, boys 56%, positive respiratory syncytial virus 73.4%). Among them, 227 children had a routine chest radiograph revealing distension and/or bronchial wall thickening (n=141, 62.4%), focal opacity (19.9%), or atelectasis (17.7%). This radiograph led to the prescription of antibiotics in six patients (2.6%) and allowed the diagnosis of vascular abnormality to be made in one case (0.4%). Thirty-five patients (15.4%) had a second chest radiograph during their hospitalization owing to oxygen dependency (n=21), respiratory distress (n=11), persistent fever (n=2), or no reason specified (n=1). Pneumonia (n=7) and/or atelectasis (n=15) were then found in 62.9%. Patient management (antibiotics, postural maneuvers) was modified in six patients (17.1%). CONCLUSIONS: Routine chest radiographs contribute only partially to the treatment of infants hospitalized for acute bronchiolitis. However, radiography is useful when the hospitalized child does not improve at the expected rate or if the disease is severe. The indication of chest radiography in infants hospitalized for acute bronchiolitis should be discussed on a case by a case basis.

[Chlamydia trachomatis pneumonia: two atypical case reports]. / Pneumonie du nourrisson à Chlamydia trachomatis: à propos de 2 cas atypiques.

We report on 2 recent and misleading cases of infants hospitalized for Chlamydia trachomatis pneumonia: the first one, 29 days old, had a clinically relevant presentation but was born by caesarean section; the second one, 43 days old, presented with acute respiratory distress. Microbiological diagnosis was achieved by the specific detection of C. trachomatis by molecular biology from a nasopharyngeal sample. These 2 clinical cases illustrate the need to systematically look for C. trachomatis during bacterial-like pneumonia in infants under 3 months of age.

[Clustered cases of intrafamily invasive Streptococcus pyogenes infection (or group A streptococcus)]. / Cas groupés intrafamiliaux d'infection invasive communautaire àStreptococcus pyogenes (ou streptocoque du groupe A).

Streptococcus pyogenes or group A streptococcus (GAS) is responsible for serious invasive infections with a risk of secondary infection in patients with more contact than in the general population. Regardless of clustering, few intrafamilial invasive infections have been reported despite a recent increase in the incidence of invasive GAS disease. We report the cases of two brothers, one a boy of 8.5 years with toxic shock syndrome with no bacteria identified and the second, 1 week later, his 14.5-year-old brother in hospital for sepsis due to GAS. The occurrence of a confirmed case of invasive GAS and a probable case within such a short period met the definition of clustered cases. Both brothers showed no risk factors for invasive disease and no gateway including skin was found. Antibiotic therapy was initiated in the family as recommended by the French Higher Council of Public Hygiene.

[Neonatal botryomycosis]. / Tuméfactions multiples chez un nouveau-né révélant une botryomycose.

Botryomycosis is an uncommon bacterial infection. It occurs in two forms: cutaneous and visceral. Fewer than 30 pediatric cases have been reported. We present the first case of a 14-day-old newborn with botryomycosis revealed by four tumefactions located in the inguinal and popliteal hollows, without a biological inflammatory syndrome. Pathological examination of the sample demonstrated botryomycosis. The culture collection found Staphylococcus aureus. Progression was favorable with appropriate prolonged antibiotic therapy. Predicting factors such as immunodeficiency or cystic fibrosis were excluded.

[Hemothorax and hereditary multiple exostosis in a 9-year-old boy]. / Hémothorax et maladie exostosante multiple héréditaire chez un enfant de 9 ans.

We report a case of spontaneous hemothorax in a 9-year-old boy due to costal exostosis in the context of hereditary multiple exostosis disease. This is an unusual complication, whose pathophysiology remains unclear.

[Infective endocarditis in children without underlying heart disease: a retrospective study analyzing 11 cases]. / Endocardite infectieuse sur coeur sain chez l'enfant: étude rétrospective de 11 cas.

The aim of our study was to determine the different characteristics of infective endocarditis in children without underlying heart disease. This was a descriptive, retrospective study including all cases of infective endocarditis without underlying heart disease occurring in children under 18 years of age, hospitalized at the Timone Children's Hospital in Marseille, France, between 1997 and 2008. The clinical, microbiological, and echocardiography data; treatment; and outcome were reviewed for each case. Over an 11-year period, 26 children were hospitalized with infective endocarditis. Eleven children (7 boys) had no underlying heart disease (42 %). Their mean age was 8 years and 3 months. Underlying conditions including neoplasm, preterm birth, and central venous catheter were found in 6 cases. A heart murmur was observed in 82 % of the children. A microorganism was isolated in 10 children (91 %). Staphylococcus aureus was the most common agent (45 %), followed by fungi (18 %). Echocardiography detected cardiac complications in 7 cases (64 %). Ninety-one percent of the children received intravenous antibiotics for a mean duration of 45 days. Eighty-two percent of our patients required surgical intervention. In our series, 91 % of the patients met the modified Duke criteria defining infective endocarditis. In-hospital mortality was 11 %. Embolic complications were seen in 5 cases (45 %), patients whose cultures yielded S. aureus or fungal organisms were more likely to present complications. Infective endocarditis without heart disease has particular features that differ from those of congenital heart disease. This diagnosis must be considered when predisposing factors are present.

[Assessment of the French Consensus Conference for Acute Viral Bronchiolitis on outpatient management: progress between 2003 and 2008]. / Application de la Conférence de consensus sur la bronchiolite aiguë du nourrisson en médecine générale : évolution entre 2003 et 2008.

BACKGROUND: The 2000 French Consensus Conference for Acute Viral Bronchiolitis management underlined clinical practice recommendations. OBJECTIVE: To assess the impact of these guidelines on management of acute bronchiolitis, 3 and 8 years after publication. METHODS: A standardized questionnaire was mailed to 762 general practitioners (GPs) in 2003 and 800 GPs in 2008. It described two clinical case scenarios (moderate acute viral bronchiolitis and severe acute bronchiolitis) and inquired about the physician's conduct in each case. The primary outcome was the percentage of responders who described patient management in accordance with current guidelines: correct diagnosis and treating the patient symptomatically in an outpatient setting in the first case and correct diagnosis in addition to requiring an inpatient setting for the second case. Secondary outcome was the percentage of responders who would have given unnecessary or potentially harmful treatments. Physician responses in 2003 and 2008 were compared using the Student t-test and the Chi(2) test. Multivariate analysis was conducted to assess the potential factors associated with good adherence to guidelines. RESULTS: Nearly 25% of the questionnaires were returned in 2003 and 2008. Patient management was described in accordance with the guidelines in only 6% in 2003 and 20% in 2008 (p< or =0.001). Unnecessary treatments were prescribed in 77% in 2003 vs 60% in 2008 (p<0.001) and potentially dangerous treatments in 38% in 2003 vs 22 % in 2008 (p=0.006). Using multivariate analysis, the GP's age was inversely correlated with adherence to clinical recommendations (adjusted odds ratio [adOR]=0.93; 95% CI=0.87-0.98; p=0.01). Fifty-four percent of GPs reported knowing these guidelines, but only 57% of them declared that the latter has modified their practice. DISCUSSION: GPs are still prescribing unnecessary or even potentially dangerous treatments for bronchiolitis. On the other hand, severe cases are correctly diagnosed and well managed in an inpatient setting. CONCLUSION: Three and 8 years after their publication, adherence to guidelines is insufficient.

[Coverage rate of influenza vaccine in healthcare workers in the 12 cystic fibrosis centres of the Greater South Region of France in 2005/2006]. / Couverture vaccinale vis-à-vis de la grippe des soignants des douze Centres de Ressources et de Compétences de la Mucoviscidose du Grand-Sud de la France en 2005-2006.

INTRODUCTION: In France annual influenza vaccination is recommended for healthcare workers but few data regarding coverage are available. The objective of the present study was to evaluate influenza vaccine coverage rate in healthcare workers from the 12 cystic fibrosis centres in the Greater South Region of France during the 2005/2006 'flu season. METHODS: An observational, descriptive telephone survey was performed from February to April 2006 to collect information about the vaccine status of all the healthcare workers in the 12 cystic fibrosis centres of the Muco-Sud and Muco-Med networks. RESULTS: During the 2005/2006 'flu season a 59.4% influenza vaccine coverage rate was achieved in the 128 healthcare workers. The influenza vaccine coverage rate ranged from 81.4% (physicians) to 16.7% (social workers). Vaccination was usually performed in October (42.1%) or November (39.5%) by occupational medicine officers (65.8%). The most frequently reported reason for non-vaccination was "vaccine useless as the disease is benign" (36.7%). CONCLUSIONS: The influenza vaccine coverage rate in healthcare workers from the 12 cystic fibrosis centres in the Greater South Region of France is high, but still too low in view of the risks influenza may incur for cystic fibrosis patients. It fails to meet one of the objectives of French Public Health Law (at least 75% influenza vaccine coverage rate for healthcare workers by 2008).

[Kystic pleuropulmonary blastoma fortuitly discovered in an infant]. / Pleuropneumoblastome kystique de découverte fortuite chez un nourrisson.

Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, associated with a poor outcome. We report the case of a 7 week-old boy with a pleuropulmonary blastoma classified as type I. Disease was discovered at a chest X-ray performed as a work-up for a benign acute viral bronchiolitis. The final diagnosis was brought by pathology: a bronchopulmonary malformation had not been ruled out by clinical, radiological and macroscopic findings. Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, for which the poor outcome and tough diagnosis justify a surgical attitude when an intrathoracic bullous lesion is found in an infant.

[Nebulized drugs: the evolution?]. / Nébulisations médicamenteuses : évolution ?

The revival of nebulization as a drug delivery route is real. The current delivery systems respond to the new European norms, the new mesh-vibrating nebulizers allow delivering drugs more quickly, other nebulizers, more performant because of less drug losses and of a better lung deposition of the drug, are in progress. Only 12 drugs are commercialized for nebulization. All are available in dispensaries, some requiring a first prescription by a physician working in a hospital (cystic fibrosis drugs), others requiring a prescription from only some specialists as paediatricians or pulmonologists (bronchodilators). Works are in progress concerning the diameter and shape of the drug particles (nanotechnology) and also concerning the use of nebulized drugs for a systemic effect (vaccines, insulin, cyclosporine, anticancerous agents, etc.).

[Lambert-Eaton myastenic syndrome revealing neuroblastoma in 2 children]. / Syndrome de Lambert-Eaton et neuroblastome chez l'enfant: à propos de 2 observations.

Lambert-Eaton myasthenic syndrome is a paraneoplasic syndrome which can reveal a primitive tumor. Frequently, the first diagnosis is myasthenia gravis. This disease is extremely rare in children. Only 10 cases have been reported in the last 35 years. We report 2 new observations occurring in very young patients, aged 2 and 3 years, with a ganglioneurobastoma as primitive tumor.

[The effects of tobacco on children]. / Les effets du tabac chez l'enfant.

INTRODUCTION: Smoking remains an important topic. It is estimated that at present half the children born in France have been, or will be, exposed to the toxic effects of passive smoking. This paper aims to review the evidence of the effects of passive smoking, both intra and extra-uterine. STATE OF THE ART: Extra-uterine passive smoking is implicated in numerous respiratory (asthma, allergy, infections...) and non respiratory diseases (neoplastic, dental, ophthalmic, digestive, cardio-vascular and infective). Intra-uterine exposure is even more dangerous and leads to abnormalities of the pregnancy (placental abnormalities, prematurity...), of the foetus (behavioural problems, malformations) and long term problems for the unborn child. Passive smoking is responsible for pulmonary morphological abnormalities (pulmonary hypoplasia, reduction in elasticity, increased deposition of collagen and alteration of alveolar structure) and functional disorders (reduced compliance, increased airways resistance, bronchial hyperreactivity). Finally it causes a disturbance of respiratory control, promoting all the factors responsible for sudden cot death. CONCLUSIONS: The ill effects of passive smoking fully justify efforts to inform and persuade the medical profession of its duty to fight this scourge.

[Cystic intrathoracic hemolymphangioma: a rare differential diagnosis of acute bronchiolitis in an infant]. / Hémolymphangiome kystique intrathoracique: un diagnostic différentiel rare de bronchiolite aiguë chez un nourrisson.

Hemolymphangioma is a benign malformation of the lymphatic vessels. Cervical localisation is the most frequent. Mediastinal localisation is rare, but may be life-threatening because of airway compression. We report on a four-month-old boy who presented, in a context of epidemics, with clinical signs of acute bronchiolitis requiring mechanical ventilation for several days. Chest X-rays showed an important mediastinal mass with airways compression. Diagnosis was evoked on CT-scan aspects and confirmed by histology. Surgery allowed complete resolution, but dysphonia and oropharyngeal disorders persisted for several months. This rare congenital malformation is reviewed.