[Simultaneous supraventricular tachycardia in twin premature newborns of a monochorionic pregnancy]. / Tachycardie supraventriculaire simultanée chez des jumeaux monozygotes prématurés.

Supraventricular tachycardia (SVT) is the most common pediatric arrhythmia. SVT occurs more frequently in infants under 1 year of age, most of whom have structurally normal hearts. Pejorative predisposing factors such as congenital heart disease exist in only a few patients. In patients with a healthy heart, SVT is due to re-entry via an accessory pathway. Some articles describe occurrence of SVT in 1 of the fetuses or newborns from multiple gestation pregnancies, but few cases report simultaneous start of SVT in twins. This observation describes simultaneous occurrence of SVT in both premature infants from a monochorionic twin gestation. Successful emergency treatment consisted in physical maneuvers increasing vagal activity. The twin neonates then received a prolonged anti-arrhythmic amiodarone treatment. Both of their electrocardiograms were normal, without Wolf-Parkinson-White syndrome. Both of their echocardiography results showed aneurysms of the atrial septum. At 6 months of age, SVT had not recurred. This case describes an original presentation of simultaneous SVT in twins and highlights the efficacy of vagal maneuvers to manage stable patients. We discuss the existence of benign predisposing heart structural abnormalities.

Non-classic cystic fibrosis associated with D1152H CFTR mutation.

BACKGROUND: Limited knowledge exists on phenotypes associated with the D1152H cystic fibrosis transmembrane conductance regulator (CFTR) mutation. METHODS: Subjects with a D1152H allele in trans with another CFTR mutation were identified using the French Cystic Fibrosis Registry. Phenotypic characteristics were compared with those of pancreatic insufficient (PI) and pancreatic sufficient (PS) cystic fibrosis (CF) subjects in the Registry (CF cohort). RESULTS: Forty-two subjects with D1152H alleles were identified. Features leading to diagnosis included chronic sinopulmonary disease (n = 25), congenital absence of the vas deferens (n = 11), systematic neonatal screening (n = 4), and genetic counseling (n = 2). Median age at diagnosis was 33 [interquartile range (IQR, 24-41)] years in D1152H subjects. Median sweat chloride concentrations were 43.5 (39-63) mmol/l in D1152H subjects and were markedly lower than in PI and PS CF subjects (p < 0.05). Bronchiectasis was present in 67% of D1152H subjects, but Pseudomonas aeruginosa colonization and pancreatic insufficiency were present in <30% of subjects. Estimated rates of decline in forced expiratory volume in 1 s (FEV(1)) were lower in D1152H subjects vs PI CF subjects (p < 0.05). None of the D1152H subjects identified since 1999 had died or required lung transplantation. CONCLUSIONS: When present in trans with a CF-causing mutation, D1152H causes significant pulmonary disease, but all subjects had prolonged survival.

Antibiotic prophylaxis for the prevention of recurrent urinary tract infection in children with low grade vesicoureteral reflux: results from a prospective randomized study.

PURPOSE: Antibiotic prophylaxis is given to children at risk for urinary tract infection. However, evidence concerning its effectiveness in grade I to III vesicoureteral reflux is lacking. The objective of this study was to determine whether antibiotic prophylaxis reduces the incidence of urinary tract infection in young children with low grade vesicoureteral reflux. MATERIALS AND METHODS: Children 1 month to 3 years old with grade I to III vesicoureteral reflux were assigned randomly to receive daily cotrimoxazole or no treatment, and followed for 18 months. A urinary tract infection constituted an exit criterion. Infection-free survival rates were calculated using the Kaplan-Meier method and compared using the log rank test. RESULTS: A total of 225 children were enrolled in the study. Distribution of gender, age at inclusion and reflux grade were similar between the 2 groups. There was no significant difference in the occurrence of urinary tract infection between the 2 groups (17% vs 26%, p = 0.2). However, a significant association was found between treatment and patient gender (p = 0.017). Prophylaxis significantly reduced urinary tract infection in boys (p = 0.013), most notably in boys with grade III vesicoureteral reflux (p = 0.042). CONCLUSIONS: These data suggest that antibiotic prophylaxis does not reduce the overall incidence of urinary tract infection in children with low grade vesicoureteral reflux. However, such a strategy may prevent further urinary tract infection in boys with grade III reflux.

[Family observation of Kawasaki disease: 2 cases in sister and brother]. / Forme familiale de la maladie de Kawasaki: à propos de 2 cas dans une fratrie.

This article presents the clinical observation of 2 cases of children suffering from Kawasaki disease in the same family at a 7-year interval. This observation suggests a genetic predisposition to the disease. Epidemiological surveys conducted in Japan for some years point to a genetic factor but without proof. Childhood systemic febrile vasculitis is hard to recognise, so its diagnosis is often made lately, while there is an effective therapy, which prevents from serious cardiovascular complications. Identification of its precise cause, a necessary step before any diagnostic laboratory test, will be a major breakthrough.

[Nebulisation in childhood asthma]. / La nébulisation dans l'asthme de l'enfant.

The inhaled route way, which directly brings an appropriate amount of drug in the right place, is at the present time the recommended route for asthma treatments. The development of spacer devices, easier to use, has reduced the indications of nebulised treatments, but these are still essential in some situations. Nebulisation of short acting beta-2-agonists, which usually requires oxygen administration, is still the first line treatment of a severe acute asthma attack. Ipratropium bromide can be added in the nebulisation, depending on the severity of the attack and the response to beta-2-agonists. Sodium cromoglycate is not used in the prophylactic treatment of childhood asthma any more, but nebulised budesonide is still very useful in moderate to severe persistent asthma in children under 5 years of age, after failure of a treatment with a spacer device. The quality of nebulisation is fully dependent on the equipment used, and on the practical conditions of its realization. It is of most extreme importance to use a compressor/nebulizer couple which is validated for the delivered drug, and that the child breathes, depending on age, via a mouth peace or a facial mask.

[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.

Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: a 10-year retrospective study in a French region (Brittany).

Until the year 2000, systematic cystic fibrosis (CF) neonatal screening was only performed in a few regions of France. The Brittany region began in 1989, but not the neighboring region of Loire-Atlantique. The present study compares the clinical evolution of both affected populations 10 years after screening was started. Although the 77 screened and 36 nonscreened children were followed in different CF centers, they were included in similar care protocols. The clinical characteristics at diagnosis and their evolution over a 10-year period of all the children affected with CF and born between January 1, 1989 and December 31, 1998, excluding those with meconium ileus, were compared. There were no significant differences in sex ratio, gestational age, anthropometric data at birth, frequency of deltaF508 homozygotes, proportion of pancreatic-insufficient patients, and mean age between the two populations. Age at diagnosis was lower in the screened group (38 days vs. 472 days, P < 10(-7)), as was the delay in supplementation with pancreatic enzymes (1.7 months vs.15.9 months, P < 10(-7)). The proportion of children who were hospitalized at least once was higher among the nonscreened than the screened patients (86% vs. 49%, P < 10(-4)). Z-scores for weight and height were significantly better in the screened population, not only in the first years of life, but also at 5 years old for height and 8 years old for weight. The Shwachman and Brasfield scores were higher among the screened children during the whole period of follow-up. No significant differences in colonization by Pseudomonas aeruginosa nor in lung function were found. Given the homogeneity in the characteristics and the follow-up of both populations, the benefits in terms of nutrition and clinical well-being of neonatal screening appear to be clear, thus confirming the advantages of its general implementation.

[Inhalers and nebulizers in the children]. / Inhalation et nébulisation chez l'enfant.

Inhaled way is the most efficient method to deliver drugs for the treatment of respiratory diseases. Pulmonary deposition depends upon the inhalation system and technique. The evaluation of the drug deposition is difficult, particularly in children. Because of irradiation, scintigraphic studies which permit the best approach of the pulmonary deposition are rare in children. Various inhalation systems are available: nebuliser, metered-dose inhaler with or without spacer device, dry powder inhaler. Whatever the system used, the control of asthma will be only possible with the regular evaluation of the child inhalatory technique.

[Evaluation of systematic pulmonary function testing for asthma in children aged three to five years]. / Intérêt des épreuves fonctionnelles respiratoires systématiques dans l'évaluation de l'asthme chez l'enfant de trois à cinq ans.

UNLABELLED: Pulmonary function testing is not usually done in the preschool child, despite the recent data showing early deterioration in airway function in asthma. METHODS: We evaluated feasibility and clinical interest of flow-volume loop by forced expiratory maneuver and measure of airway resistance by interrupter technique (interrupter resistance), before and after inhalation of salbutamol, in 75 children aged three to five years seen in the ambulatory setting for asthma. RESULTS: Feasibility rate (92%) and reproducibility rate (91%) of those techniques were good, so that 84% of the cases could be exploited (63 children of 75). We found few significant associations between clinical parameters and flow-volume loop. We found a significant association between elevated interrupter resistance at basis and night-symptoms (P = 0.03), between diminished interrupter resistance after salbutamol and exercise-symptoms (P = 0.03), symptoms in the ambulatory setting (P = 0.02) and absence of inhaled corticosteroid treatment (P = 0.046). Pulmonary function testing resulted in treatment modification in 14% of cases. CONCLUSION: Our study shows that flow-volume loop and measure of airway resistance by interrupter technique can be done with a good reproducibility in the preschool child. Interrupter resistance appears to be better correlated than flow-volume loop with usually evaluated clinical parameters.

[Treatment of cytomegalovirus pneumonia with ganciclovir in an immunocompetent infant]. / Traitement d'une pneumopathie à cytomégalovirus par ganciclovir chez un nourrisson immunocompétent.

UNLABELLED: Cytomegalovirus infection is common in the immunodepressed child, and because of its severity, is usually treated with intravenous ganciclovir. It is also common in the immunocompetent child, but is usually asymptomatic, so that there is no indication for ganciclovir treatment, because of the potential toxicity of the drug. CASE REPORT: We report a case of symptomatic cytomegalovirus infection in a six-week-old infant, presenting with interstitial pneumonia associated with weight and height growth failure. Cytomegalovirus was found by polymerase chain reaction in the bronchovalveolar lavage fluid and in the blood. This child was successfully treated with 15 days of intravenous ganciclovir, and experienced no adverse effect of this treatment. The child was free of symptoms and free of immunodeficiency after seven months of follow-up. CONCLUSION: Immunocompetent patients with cytomegalovirus infection, pulmonary involvement and weight and height growth failure, may benefit from intravenous ganciclovir treatment. Further studies, with larger number of cases, are yet needed to define more precisely the indications of such a treatment in the immunocompetent child.

[Acute intestinal invagination revealing celiac disease in a 9-month-old infant]. / Invagination intestinale aiguë révélant une maladie coeliaque chez un nourrisson de neuf mois.

UNLABELLED: In most cases bowel intussusception is idiopathic in children. Indirect causes, such as celiac disease, are rare. CASE REPORT: A nine-month-old girl was admitted for a bowel intussusception diagnosed by ultrasonography, yet not confirmed by barium enema. The girl underwent a thorough check up, due to a loss of weight and denutrition symptoms which evidenced immunoglobulin A anti-gliadin, immunoglobulin G anti-gliadin, immunoglobulin A anti-endomysium and antireticulin positive antibodies. Celiac disease was confirmed by bowel biopsy, which revealed a subtotal villous atrophy. Evolution was favourable under a gluten free diet. COMMENTS: The description of a bowel intussusception associated with celiac disease is common. This observation appears to be the earliest case ever described: the patient was only nine months old. The initial procedure is routine: diagnosis by ultrasonography, therapeutic enema and surgery only in case of failure of medical treatment. Intussusception associated with celiac disease often presents in an atypical way: elementary forms, spontaneously resolvent and recidivious. The knowledge of this clinical set of symptoms leads to an early diagnosis of celiac disease. The gluten free diet prevents the recurrence of intussusception.

[Symptomatic treatment of cough in children]. / Les traitements symptomatiques de la toux chez l'enfant.

Treatment of chronic cough should always be etiologic. Recurrent viral infections are the most frequent problem. Utilisation of antitussive drugs must be limited, of short duration and without contraindications. Antitussive medications are classified in opiate and nonopiate, with central or peripheral action.

[Does neonatal screening of cystic fibrosis affect outcome? Comparative study of two cohorts in Britanny and Loire-Atlantique with follow-up after ten years]. / Le dépistage néonatal systématique améliore-t-il le pronostic de la mucoviscidose? Etude comparative de deux cohortes en Bretagne et en Loire-Atlantique avec un recul de dix ans.

UNLABELLED: Neonatal screening for cystic fibrosis was started in Brittany in 1989 but not in the adjacent department of Loire-Atlantique. This study compares the outcome from the children of both populations nine years after the beginning of the screening. Those children were seen in different centers but with the same following guidelines. POPULATION AND METHODS: All children with cystic fibrosis born between 01/01/89 and 31/12/97 in Brittany and the Loire-Atlantique, excluding the meconium ileus, were compared for their initial characteristics and their outcome after nine years of follow-up. RESULTS: There was no significant difference between both populations for sex ratio, gestational age, birth biometry, percentage of homozygotes delta F508, and mean age of children. Age at diagnosis was lower in Brittany (37 vs 372 days, P < 10(-7)), as was the delay for starting pancreatic supplementation (1.5 vs 14.3 months, P < 10(-7)). Percentage of children hospitalized at least once was higher in Loire-Atlantique (84.4 vs 40.3%, P < 10(-4)). There was no significant difference for colonization with Pseudomonas aeruginosa. Z-scores for weight and height were better in Brittany, as were Shwachman's and Brasfield's scores. CONCLUSION: The homogeneity of both populations and their follow-up points out that even if the numbers of children are small and the study is retrospective, some benefits of neonatal screening appear, which are already found in other countries where it is partly practiced. This leads us recommend its general use in our populations, which should be associated with the follow-up of the screened children in cystic fibrosis centers to achieve the most of its benefits.

[Mycoplasma pneumoniae pneumonia in a four-year-old child with transient abscess in the right lower lobe]. / Pneumopathie à Mycoplasma pneumoniae chez un enfant de quatre ans avec abcédation bulleuse transitoire du lobe inférieur droit.

UNLABELLED: The frequency of Mycoplasma pneumoniae infection among community-acquired pneumonia, underestimated for a long time, is now better known. Severe evolution is yet uncommon. Differential diagnosis with Streptococcus pneumoniae is often difficult. CASE REPORT: A 4-year-old child was admitted for a right lower lobe pneumonia, with very high values of white blood cell count and CRP, worsening despite a treatment with high doses of amoxicillin, then with cefotaxime and vancomycin. Diagnosis of M. pneumoniae infection was considered only on the tenth day after admission and confirmed on the thirteenth day. Clinical outcome rapidly improved with macrolide antibiotherapy. Radiologic outcome consisted, two months after the beginning of the pneumonia, in abscess of the right lower lobe, which recovered in one month with continuing oral antibiotherapy. CONCLUSION: Lung abscess is very rare in M. pneumoniae pneumonia, as only two other cases were described in the literature. In all three cases, macrolide therapy was delayed. Those cases highlight the importance of considering M. pneumoniae infection in a beta-lactams-resistant community-acquired pneumonia, whatever its severity may be, and to start macrolide antibiotherapy. Our case also shows the possibility of a conservative treatment in case of pulmonary abscess, if clinical tolerance is good.