[Fatal evolution of a dilated cardiomyopathy during a primary Epstein-Barr virus infection]. / Myocardiopathie dilatée d'évolution fatale au cours d'une primo-infection par le virus Epstein-Barr.

We report the observation of a 2-year-old child who developed an acute heart failure during a primary EBV infection. The viral serological diagnosis showed CMV and EBV IgM positivity. The detection of EBV genome by PCR and the characteristic evolution of EBV serological response confirmed a primary EBV infection.

[Ataxia-telangiectasia: a review]. / Ataxie-télangiectasie: de la clinique à la physiopathologie.

Ataxia-telangiectasia (AT) is an autosomal recessive inherited disease caused by mutational inactivation of the ATM gene. It is a multisystemic disease, characterized by progressive neurological dysfunction, especially in the cerebellum, oculo-cutaneous telangiectasia, immunodeficiency, recurrent sino-pulmonary infections and high incidence of neoplasms. The responsible gene, ATM, encodes a large protein that belongs to a family of protein kinases with a phosphatidylinositol 3-kinase (Pi3K) domain. ATM is a key regulator of cell cycle checkpoints that causes DNA repair or apoptosis. Several studies report ATM function in target cells (such as neurons, fibroblast, endothelium, germ cells, lymphocytes). The pleiotropic phenotypes of AT reflect the multifaceted activities of ATM protein. In nucleus (lymphocytes, fibroblasts, germ cells) ATM is involved in regulation of cell-cycle checkpoints; in cytoplasm ATM regulates redox state (neurons).

Microbiologic contamination study of nebulizers after aerosol therapy in patients with cystic fibrosis.

BACKGROUND: To evaluate the contamination of delivery systems after an aerosol therapy session in patients with cystic fibrosis who have chronic Pseudomonas aeruginosa infection. METHODS: Fifty-three patients with cystic fibrosis were enrolled in the study from March 1996 to June 1997. All patients were age 7 years or older and had P aeruginosa infection. They also had been treated with recombinant deoxyribonuclease and were capable of producing sputum for culture. RESULTS: Nine devices were excluded for the study. A total of 44 nebulizers were included: 37 from patients with P aeruginosa colonization with a count of 10(6) colony-forming units/mL or more and 7 with a count of between 10(5) colony-forming units/mL and 10(6) colony-forming units/mL. CONCLUSION: This study demonstrates that in the absence of cleaning, nebulizers of patients with cystic fibrosis who are infected with P aeruginosa are likely to be contaminated by a pathogenic flora.

Activation of alveolar macrophages from children with the acquired immunodeficiency syndrome-related complex.

The ability of alveolar macrophages (AM) to release hydrogen peroxide (H2O2), an indicator of AM function, was studied in five children with the acquired immunodeficiency syndrome (AIDS) related complex and, for comparison, in 11 children without disorders of the lung parenchyma. In the AIDS-related complex group, pulmonary manifestations were mild, and lung involvement was suspected by moderate clinical and/or radiological features. None had a past history of opportunistic infections; neither did any have lymphopenia. Cytologic study of the bronchoalveolar lavage (BAL) fluid revealed increased cellularity with increased percentage of lymphocytes. The study of H2O2 release was performed on unstimulated AM and on AM stimulated by phorbol myristate acetate (PMA). Under both experimental conditions, the amount of H2O2 accumulated in the medium was significantly increased in the group with AIDS-related complex (P less than 0.001). As no enhanced oxidative activity has been reported in AM from patients with full-blown AIDS, an increased ability of AM to release oxygen metabolites from children with AIDS-related complex may reflect an initial and temporary step in the course of the LAV/HTLV-III pulmonary disease. Determining AM activation might be a reliable method of assessing the evolution of lung disorder in AIDS.

Role of viruses and atypical bacteria in exacerbations of asthma in hospitalized children: a prospective study in the Nord-Pas de Calais region (France).

We studied the role of viruses and atypical bacteria in children hospitalized with exacerbated asthma by a prospective study of children with acute asthma admitted to the Department of Pediatrics in Lille, and to 15 hospitals in the Nord-Pas de Calais region, from October 1, 1998-June 30, 1999. We included children aged 2-16 years with active asthma, defined as three or more recurrent episodes of reversible wheezing. The severity of asthma and of asthmatic exacerbations was recorded. Immunofluorescence assays (IFA) on nasopharyngeal secretions (NPS), serological tests, or both, were used for detection of influenza virus, respiratory syncytial virus (RSV), adenovirus, parainfluenza virus, and coronavirus. Polymerase chain reaction (PCR) assays on NPS were used for rhinovirus and enterovirus. Serological tests for Chlamydia pneumoniae and Mycoplasma pneumoniae were performed. A control group of asymptomatic asthmatic outpatients was examined for respiratory viruses (using IFA and PCR). Eighty-two symptomatic children (mean age, 7.9 years) were examined. Viruses were detected in 38% (enterovirus, 15.8%; rhinovirus, 12%; RSV, 7.3%). Serological tests for atypical bacteria were positive in 10% of patients (C. pneumoniae, 5%; M. pneumoniae, 5%). Among the 27 control subjects (mean age, 7.9 years), one PCR was positive for enterovirus. There was no correlation between severity of chronic asthma or asthmatic exacerbations and the diagnosis of infection. Atypical bacterial pathogen infections were linked with prolonged asthmatic symptoms. In conclusion, we confirmed the high incidence of viral infection in acute exacerbations of asthma, especially enteroviruses or rhinoviruses. Persistent clinical features were more frequently associated with atypical bacterial infections, suggesting that these infections should be investigated and treated in cases of persistent asthmatic symptoms.

Idiopathic pulmonary fibrosis in infants.

Idiopathic pulmonary fibrosis is a poorly characterized disease in infants. In the present report, we reviewed our experience with 10 infants during a 10-year period. In 9 patients, onset of symptoms occurred before the age of 2 months and included tachypnea, cough, and inadequate weight gain. However, despite the presence of these symptoms, diagnosis was delayed for 3 months at which time the infants were referred to the pediatric pulmonary department, when the diagnosis was confirmed by open lung biopsy. At the time of admission, bronchoscopy with alveolar lavage was performed in 9 children and showed severe alveolitis with an increase in the neutrophil count. Nine infants were treated with prednisone alone or in combination with chloroquine, colchicine, or cyclophosphamide; all these patients died despite treatment. One infant was treated with pulses of methylprednisolone because of a failure in response to oral prednisone. This girl who displayed similar clinical, radiological, and histological abnormalities as the other children at the time of diagnosis is the only child still alive after 3 years of follow-up. She is now free of respiratory symptoms and has a normal growth curve. The present report raised two important points: (1) a thorough evaluation of characteristic symptoms should lead to an early diagnosis of pulmonary fibrosis in infants; and (2) administration of pulse therapy using corticosteroids has been helpful and needs to be evaluated further.

[Treatment of ventricular tachycardia in infancy and childhood with amiodarone]. / Traitement des tachycardies ventriculaires du nourrisson et de l'enfant par l'amiodarone.

Ventricular tachycardia, especially in its apparently primary form, is rare in children and difficult to treat, often requiring aggressive methods of reduction or antiarrhythmic drugs unsuited for paediatric practice. Therefore, we investigated the use of amiodarone whose efficacity in the treatment of resistant ventricular tachycardia and good tolerance in children have been established. Three infants, aged from 9 to 15 months, and two children aged 6 and 7 years with apparently primary VT were selected. Etiological investigations were negative in four cases but in one of the older children a left ventricular fibroma was diagnosed and removed surgically. Amiodarone was administered orally at a dose of 500 mg/m2/24 hrs for 5 to 15 days, and then 250 mg/m2/24 hrs for one month in the surgical patient and for 9 to 39 months in the four "idiopathic" cases. Reduction of VT was obtained in all cases 8 to 48 hours after the first dose. There was only one recurrence, attributed to an over-rapid reducing in dosage; it quickly regressed after returning to the initial dosage. Stable sinus rhythm was maintained at long-term: 18 months, 2 years and 5 years after tailing off a course of 20, 40 and 1 month's treatment in 3 children. These cases are considered to be cured, but in one of these children two courses of amiodarone were required, the second for a relapse 3 months after stopping a 9 month's course of therapy. The other two children are still under treatment after 9 and 15 months with no recurrences. There were no hemodynamic, ocular or thyroid side effects. On the other hand, three cases of photosensitivity, two minor and one major requiring termination of therapy after a 20 months course, were observed. In conclusion, amiodarone would appear to be the treatment of choice for ventricular tachycardia in children, reduction of the arrhythmia being obtained in all cases even by oral administration within reasonable limits: its prophylactic value is excellent and clinical tolerance very satisfactory: a definitive cure can be hoped for an idiopathic VT providing that maintenance therapy has been sufficiently prolonged (2 years).

[Medium- and long-term results of palliative surgery in complex cyanotic cardiopathies. A propos of 228 cases and 292 operations]. / Résultats à moyen et long terme de la chirurgie palliative dans les cardiopathies cyanogènes complexes. A propos de 228 observations et de 292 interventions.

The results of palliative surgery are assessed in a retrospective study of 228 children operated between 1968 and 1977 for complex cyanotic congenital cardiac malformations, the longest follow-up period being 10 years. The surgery consisted in revascularising the lung by systemo-pulmonary anastomoses: Blalock-Taussing, Potts, Waterston, cavo-pulmonary shunts or, on the contrary, of avoiding pulmonary hypertension by limiting pulmonary flow with banding. In addition to these two techniques, in some cases atrial septal defects were created to improve mixing of the two circulations; equalise the pressures between the two atria and relieve the left ventricle (Blalock-Hanlon procedure) or during catheterisation (Rashkind manoeuvre). The results of pulmonary revascularisation by systemo-pulmonary anastomoses were generally satisfactory with respect to the complexity of the malformations. There were 56% good results, 82 out of 146 cases. It often provided the time for curative surgery to be performed under better conditions for the children. The results were worse in children with pulmonary hypertension, with only 33% of good results, 25 out of 82 children, and there were many complications of banding which additional problems at the time of definitive repair. Despite the tendency to widen the indications of so-called curative surgery even in very small infants, palliative surgery retains a role in complex cardiac malformations either by allowing the children to reach an optimal age for repair or by being the only possible therapeutic attitude.

[Antibacterial therapy outside of Pseudomonas aeruginosa]. / Traitement anti-infectieux en dehors du Pseudomonas aeruginosa.

Hemophilus influenzae and Staphylococcus aureus (SA) are the predominant pathogens in infants with cystic fibrosis (CF). SA was the major cause of death in the pre-antibiotic era. The reason of the association of SA in CF is unclear. SA causes early damage of the respiratory tract and paves the way for Pseudomonas aeruginosa (PA). Based of this hypothesis, some centers use prophylactic antibiotics, but their efficacy is not proved and may favor growth of PA. Treatment of exacerbations is mandatory. Oral antibiotics are preferred in most cases, although few controlled clinical studies have been reported. Emergence of methicillin-resistant Staphylococcus aureus (MRSA) strains appeared during the recent years. Treatment of MRSA is difficult, patients segregation is discussed.

[Pleurisy: diagnostic and therapeutic management]. / Les pleurésies. Conduites diagnostique et thérapeutique.

Diagnosis of pleural effusion is difficult in children. The etiologies are numerous; however, infectious agents are more frequent. Thoracocentesis proves to be the first-line diagnostic tool. Light's criteria are the best for distinguishing whether the effusion is a transudate or an exudate. If the patient has an exudative pleural effusion, other tests are indicated to determine the etiology and in some cases the treatment: macroscopic appearance, cytology and differential white cell count (level of glucose, lactate dehydrogenase, adenosine deaminase, pH, bacterial cultures). Others investigations--biopsy of pleura by thoracoscopy or video-assisted thoracoscopy, bronchofibroscopy, CT scan--are sometimes useful. Intrapleural instillation of urokinase appears to be useful and safe. Evaluation is necessary for video-assisted thoracoscopy used early.

[Cytomegalovirus pneumopathies in pediatric intensive care units]. / Pneumopathies à cytomégalovirus en réanimation pédiatrique.

BACKGROUND: Cytomegalovirus (CMV) infection can result in major complications in immunocompromised infants and children. CMV pneumonia may be difficult to diagnose and the true pathogenic role of the virus in the disease is not always clear. This report describes a cohort of 20 children who suffered from CMV pneumonia. POPULATIONS AND METHODS: Twenty children aged 1 month to 11 years 10 months were admitted to our intensive care unit between 1981 and 1990 because of pneumonia with evidence of CMV infection. They were classified into three groups: group I (cases 1-10) with hemopathy or cancer, group II (cases 11-14) with AIDS, and group III (cases 15-20): non immunodeficient or immunosuppressed children. CMV infection was diagnosed after isolation of CMV from bronchoalveolar lavage (BAL) fluid (15 patients), lung biopsy revealing intranuclear inclusions or CMV antigens, or CMV-positive cultures (four patients), CMV-positive urine cultures (one patient). RESULTS: Clinical manifestations and X-rays findings were unspecific; interstitial pneumonia was found only in immunodeficient patients. CMV pneumonia was diagnosed only in two patients on post mortem examination. Concomitant pneumocystis carinii was found on BAL in two patients (group I) and two others (group II). Thirteen patients required ventilation. Eleven patients were given ganciclovir for 2 or 3 weeks; one of them was given a single dose. This treatment was well tolerated. Mortality was 90% in group I, 100% in group II and 33% in group III. CONCLUSION: Ganciclovir did not appear to benefit the immunocompromised patients with CMV pneumonia. Future treatment should include hyperimmune CMV immunoglobulins plus ganciclovir. Careful hand washing is important for all those caring for these patients to prevent contamination as is the use of CMV-negative blood products.

[Bronchial mucoepidermoid carcinoma: apropos of 3 cases]. / Carcinomes mucoépidermoïdes bronchiques: à propos de trois observations.

BACKGROUND: Mucoepidermoïd carcinomas (MEC) are very rare (less than 20 cases reported in the literature) and potentially malignant bronchial gland carcinomas. PATIENTS: Three children, two boys (11 and 7 years old) and one girl (5 years old) suffered from respiratory symptoms such as cough, recurrent pneumonia and/or hemoptysis for 2 to 12 months. Bronchial endoscopy showed a mass into the left (two cases), or the right main bronchus (one case). Chest CT scan identified local extension, and lung-associated lesions. Histopathological study concluded to MEC in the three cases. The patients were treated by segmental bronchial resection, completed with left upper lobectomy (two cases), bronchotomy (one case). All the tumor could be removed; there was no metastasis. The outcome was uneventful with a 8 to 24 months follow-up. CONCLUSION: Bronchial tumors of children must be considered in patients with chronic cough, recurrent pneumonia and/or hemoptysis and require bronchial endoscopy for their diagnosis.

[Effect of rhDNase on the respiratory function and nutritional status of children and adolescents with mucoviscidosis]. / Effects de la rhDNase sur la fonction respiratoire et le statut nutritionnel de l'enfant et de l'adolescent atteints de mucoviscidose.

BACKGROUND: In 1994 we started recombinant human deoxyribonuclease (rhDNase) in every cystic fibrosis (CF) patient whatever his (her) clinical condition, provided they were aged more than 5 years and forced vital capacity (FVC) was > or = 40%. POPULATION AND METHODS: We reviewed retrospectively the effects of rhDNase in 69 CF children and adolescents during a 2-year follow-up. Patients (35 boys, 34 girls) received 2.5 mg of rhDNase once daily from a mean age of 8.5 years (range 5-16.4). Baseline spirometric values (% predicted) and nutritional status were as followed: FVC = 84.8 +/- 21.7; forced expiratory volume in 1 second (FEV1) = 80.8 +/- 22.2; peak flow = 89.7 +/- 34.2, forced expiratory fraction 25-75% (FEF 25-75) = 71.8 +/- 32.8; Z score weight/height = -0.41 +/- 1.14; Z score weight/age = -0.48 +/- 1.25, body mass index = 15.4 +/- 1.8; caloric intake = 107 +/- 25% of recommended dietary allowances (RDA). Patients had a Shwachman-Kulczycki's score of 87 +/- 9. Spirometric and nutritional data were analysed after 1, 3, 6, 12, 18 and 24 months of treatment and compared to baseline values (changes evaluated as percent change from mean baseline for spirometric data). Shwachman-Kulczycki's score was calculated after 24 months of rhDNase. RESULTS: An improvement of FVC (+10.7%, P < 0.001) and FEV1 (+12%, P < 0.01) was noted after one month of treatment and was maintained throughout the following 2 years around 8.7% (6.4-11.4) for FVC and 8.2% (7.3-9.1) for FEV1, P < or = 0.01. This was particularly observed in children aged 5 to 10 years, in boys and in patients with a baseline FVC under 70% predicted. There was no significant change in FEF 25-75. We observed an improvement of daily caloric intake from the third month (P < 0.05) and of body mass index from the sixth month (P = 0.02). This was particularly noted in girls. Z score weight/age was improved only during the first 3 months of treatment while Z score weight/height increased only after a 2 year follow-up. There was no significant change in Shwachman-Kulczycki's score after 24 months of rhDNase. CONCLUSION: rhDNase in CF children in effective on lung function as well as on nutritional status and the response to this treatment can be evaluated after the first 3 months.

[Respiratory virus infections in children]. / Viroses respiratoires chez l'enfant.

Eighty to ninety percent of pathogens responsible for acute respiratory infections in children are viruses, but despite advances in virology these organisms are isolated in only 20 to 45 percent of the cases. Studies conducted outside hospitals have provided epidemiological data. The virus most frequently encountered is the respiratory syncytial virus. The main clinical feature of these respiratory viral infections is obstruction of the bronchioles, and their immediate or delayed danger is the risk of chronic obstructive bronchitis. Treatment is symptomatic, but specific antiviral agents, notably ribavirin, are useful in severe infections.

[Intralobar pulmonary sequestration manifested by neonatal cardiac insufficiency]. / Séquestration pulmonaire intralobaire révélée par une insuffisance cardiaque néonatale.

The authors report the case of an intralobar pulmonary sequestration in a newborn presenting congestive heart failure. The clinical symptoms mimicked a persistent ductus arteriosus. Clinical, radiographic and echographic data suggested the diagnosis which was confirmed by the angiography. A surgical treatment was realised at seven weeks consisting in the ligation of the abnormal artery and a right lower lobectomy. This is the 9th case found in the literature. A congestive heart failure in a neonate with normal intracardiac anatomy may suggest a pulmonary sequestration.

[Bronchogenic cysts in the carina]. / Kystes bronchogéniques de la carène.

Between 1977 and 1990, 11 children with carinal bronchogenic cysts were operated in our institution: 8 girls and 3 boys, ranging in age from 1 month to 5 years. All were symptomatic (acute respiratory distress and recurrent bronchiolitis). Chest X-ray showed an unilateral over distension in 10/11 cases. Barium oesophagogram showed a compression in 6/10 cases. Bronchoscopy noticed an extrinsic compression in 10/11 cases and a tracheal and/or bronchial diskinesia in 5/11 cases. The computed tomography showed a low density mass in 4/4 cases. 9 cysts were left-sided and 2 right-sided. Both children underwent a second surgery for a second cyst. 2 pneumonectomies for complete parenchyma destruction were realised. 1 left pulmonary hypoplasia was noticed. A tracheal and/or bronchial diskinesia in post-operative was noticed in 5/6 cases. The clinical and functional respiratory following was good in 10/11 cases. An early surgery treatment is necessary before definitive sequelae.

[Reproducibility of the shuttle walk test in children with cystic fibrosis]. / Reproductibilité du test de marche en navette chez des enfants atteints de mucoviscidose.

INTRODUCTION: Exercise testing is useful in the respiratory evaluation of patients with cystic fibrosis. The shuttle walk test (SWT) is a progressive, externally paced, exercise test requiring the subject to walk/run back and forth between two fixed points. The aim is to assess the reproductibility of the SWT in paediatric patients with cystic fibrosis. METHODS: This prospective study recruited 31 children with stable disease. The patients performed two SWT one day (SWT 1 and 2) and two others (SWT 3 and 4) within 15 days. Only SWT 2 and 4 were assessed for reproducibility. RESULTS: 61% were boys, median age (range): 12.9 (7-18.9) years, median Shwachman score (range): 80 (65-100), median values for FEV1 and FVC (range): 92 (55-154) and 92 (64-140)% predicted, respectively. Median distance for SWT 2-4 (range): 910 (580-1020) and 925 (540-1020) metres. Reproducibility for SWT distance and physical activity measured by an accelerometer is very good (intra-class correlation coefficient=0.90 and 0.92, respectively). SWT distance correlated with physical activity (p=3.10(-4)) and weight (p=0.03). SWT distance was independent of the following parameters: height, weight-for-age Z-score, FEV1, FVC, Shwachman score, colonisation with Pseudomonas aeruginosa. CONCLUSIONS: The SWT is reproducible in paediatric patients with cystic fibrosis and provides assessment of respiratory performance that complements spirometric measures of lung function.

[Flexible bronchoscopy in children. Experience at French centers of pediatric pneumology]. / La fibroscopie bronchique chez l'enfant. Expertise des centres français de pneumologie pédiatrique.

INTRODUCTION: Fibreoptic bronchoscopy (FB) is an important diagnostic examination in paediatric pulmonology. In 2002 the Paediatric Pulmonology and Allergy Club undertook a retrospective study to establish the current status of fibreoptic bronchoscopy among its members. METHODS: In 2001 sixty five paediatric pulmonologists carried out an average of 116 examinations (+/- 111) in 35 paediatric centres. FB was performed either in an operating theatre (15 centres), a dedicated bronchoscopy suite (6 centres) or an endoscopy suite shared with gastro-enterologists (7 centres). Other examinations were performed in areas dedicated to, or associated with intensive care. General anaesthesia was routinely used in 18 centres. The others used sedation including an equimolar mixture of oxygen and nitrous oxide in 14 centres. Ten centres performed less than 50 examinations, 12 between 51 and 100, 4 between 101 and 200 and 8 centres more than 200 in the year. Seventy two per cent of the children were less than 6 years old. The washing and disinfection procedures were manual in 20 centres and automatic in 15. RESULTS: Three principal indications were reported: persistent wheezing, suspicion of a foreign body and ventilatory difficulties. Cough, desaturation and fever were the most frequently reported side effects. CONCLUSIONS: This is the first survey in paediatric pulmonology in France. It shows a wide variation in the practice of fibreoptic bronchoscopy in children.

[Otorhinolaryngologic involvement in cystic fibrosis]. / Atteinte de la sphère ORL dans la mucoviscidose.

ENT involvement is very frequent in mucoviscidosis, particularly rhinitis and sinusitis. This prospective study, which spanned from September 1st, 1988 to August 31st, 1989 anc included 27 children with cystic fibrosis of pancreas was carried out with the intent of determining the incidence of the various associated diseases, their bacteriologic profile, and the optimal therapeutic procedure. Less than a child in 5 is symptomless when the nose and sinuses are affected. Polyposis is found to occur in 6/27 cases, and all children have sinus radio-opacities. Ear disease is rare (1/4 of cases) and is manifested by asymptomatic tubal dysfunction with minimal audiometric repercussions. Bacteriologic specimens of sputum and sinus purulent discharge show simultaneous germ positivity (10 times out of 11). Our therapeutic recommendations are based on our own experience as well as literature data, and are, in our opinion, only relevant for children with clinical signs of disease, as opposed to advocating systematic treatment of sinus foci found on X-ray.

[New pharmacological approaches: rhDNase]. / Nouvelles approches pharmacologiques: la rhDNase.

rhDNase (Pulmozyme) is a new agent in the therapeutic strategy for patients with cystic fibrosis. It is one of the first specific treatments aimed at the respiratory tract. It affects the extracellular DNA which is present in abundant quantities in the bronchial secretions of these patients. rhDNase significantly reduces the incidence of infections and improves respiratory function. It should be used as a major treatment in combination with all other treatments in patients over 5 years of age with a vital capacity of at least 40% the theoretical value. It is important to schedule the respiratory exercises as a function of rhDNase intake. The long-term therapeutic benefit remains to be evaluated.