Respiratory morbidity in children with congenital heart disease.

OBJECTIVE: To evaluate the respiratory outcome in children with congenital heart disease (CHD), considering recent management procedures and the CHD pathophysiology. DESIGN AND SETTING: Clinical and functional respiratory outcome were evaluated in 8-year-old children with isolated CHD followed up from birth in the prospective population-based EPICARD cohort. PATIENTS: Children were assigned to two groups, based on the pathophysiology of the CHD: CHDs with left-to-right shunt (n = 212) and CHDs with right outflow tract obstruction (n = 113). RESULTS: Current wheezing episodes were observed in 15% of the children with isolated CHD and left-to-right shunt, and 11% of the children with isolated CHD and right outflow tract obstruction (not significant). Total lung capacity (TLC) was the only respiratory function parameter that significantly differed between the two groups. It was lower in children with left-to-right shunt (88.72 ± 0.65% predicted) than in those with right outflow tract obstruction (91.84 ± 0.96, p = 0.006). In multivariate analysis, CHD with left-to-right shunt (coeff. [95% CI]: -3.17 [-5.45; -0.89]) and surgery before the age of 2 months (-6.52 [-10.90; -2.15]) were identified as independent factors associated with significantly lower TLC values. CONCLUSION: Lower TLC remains a long-term complication in CHD, particularly in cases with left-to-right shunt and in patients requiring early repair. These findings suggest that an increase in pulmonary blood flow may directly impair lung development.

Vaccine coverage in CF children: A French multicenter study.

BACKGROUNDS: Recent reports have pointed the low vaccine coverage in patients with chronic diseases. Data are lacking in patients with cystic fibrosis (CF). Gaining more information on coverage both for mandatory vaccines and those more specifically recommended would help to optimize care of these patients. METHODS: Data were extracted from the "MucoFlu" study, which was a prospective study performed in 2009 in the 5 cystic fibrosis centers of the Paris metropolitan area. Data on mandatory and recommended vaccines in CF were collected in the health booklet and compared to the coverage of the general population. RESULTS: A total of 134 CF children were included. Vaccination coverage for mandatory vaccines was insufficient (DTPCaHi, conjugate pneumococcal, BCG, MMR and hepatitis B) at 1year of age with no catching-up with age in contrast to the general population. Approximately 66% of the children had immunization for seasonal influenza and 91% for 2009 pandemic flu. Coverage for vaccines specifically recommended in CF was low for hepatitis A, non conjugate pneumococcal and varicella. CONCLUSION: This study shows a defect in vaccine coverage for both routine immunization and vaccines more specifically recommended in CF.

[National French guidelines for management of infants with cystic fibrosis]. / Recommandations nationales pour la prise en charge du nourrisson dépisté atteint de mucoviscidose. Consensus de la fédération des centres de ressources et de compétences de la mucoviscidose.

These guidelines aim to standardize the care of infants diagnosed with a typical form of cystic fibrosis (CF) at neonatal screening. They have been implemented by the National Working Group for Neonatal Screening of the French Federation for CF and have been validated using the Delphi methodology by a large group of clinicians involved in the care of CF infants. These guidelines encompass management and organization of care at diagnosis and describe nutritional, digestive, and respiratory monitoring and treatment during the first 2 years of life.

[Anti Pseudomonas aeruginosa antibiotic therapy in cystic fibrosis (exclusion of macrolides)]. / Stratégie antibiotique contre Pseudomonas aeruginosa dans la mucoviscidose (macrolides exclus).

Antibiotherapy is one of the main treatment in cystic fibrosis. Pseudomonas aeruginosa infection is one of the main causes of pulmonary degradation. The chronic sputum colonisation is characterized by the emergence of the mucoid phenotype, the formation of biofilm and the induction of excessive inflammatory response and consecutive tissue lesion. The choice of antibiotics depends on quantitative and qualitative analysis of sputum, bacteria resistance phenotypes and severity of infection. Treatment of P. aeruginosa is different in case of first colonization or chronic infection. In the first case, parenteral antibiotherapy (beta-lactams-aminoglycosids) followed by inhaled antibiotherapy may eradicate the germ. In the other case, superinfections can be treated with parenteral biantibiothérapy (beta-lactams or quinolons and aminoglycosides) during 15 to 21 days. This is associated with a better nutritional and respiratory status and a prolonged survival. Inhaled antibiotics between the courses have decreased the number of superinfections. This prolonged antibiotherapy must be monitored because of possible induction of bacterial resistance, nephrotoxicity and ototoxicity of aminosids and allergy to beta-lactams.

Follow-up of 452 totally implantable vascular devices in cystic fibrosis patients.

The use and complications of totally implantable vascular access devices (TIVADs) were examined during multiple courses of antibiotics in cystic fibrosis (CF) patients. This retrospective study involved 36 CF centres. Risk factors for removal and septicaemia were sought by survival analysis of censored data. Multivariate Cox models were constructed with removal or septicaemia as the event and the characteristics of TIVADs as explanatory variables. TIVADs (n = 452) were implanted in 315 patients. The mean functional time per device was 32 +/- 25 months. Long-term complications occurred with 188 devices (42%); they consisted mainly of occlusion (21%, requiring removal in 77%), infection (9.3%, requiring removal in 851%; septicaemia in 7.3%; rate 0.3 per 1,000 days, Candida in 66%), and vascular thrombosis (4.7%, removal in 58%). Multivariate survival analysis showed that removal, whatever the reason, was associated with polyurethane (versus silicone) and routine use of the device for blood sampling (versus never). No risk factors, including heparin lock, were identified for septicaemia or for removal for obstruction. Totally implantable venous access devices appear to be safe and reliable for long-term intermittent venous access. Although retrospective, this study suggests that the characteristics of the material and blood sampling are risk factors for removal.

Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization.

Chronic infection by Pseudomonas aeruginosa (PA) in patients with cystic fibrosis (CF) is preceded by a period of colonization and acute infection. Early aggressive antibiotic treatment of initial colonisation may prevent or at least delay chronic pulmonary infection. We initiated treatment with a combination of IV beta-lactam tobramycin, followed by nebulized colistin when PA was first isolated from patients with CF. Subsequent serial PA isolates obtained from these colonized CF patients were characterized by means of molecular methods to determine whether they were genetically related to the initial strain. Initial colonization was eradicated in all 19 patients. All patients reacquired PA within 3-25 months during the 3 years of follow-up. Fourteen patients acquired a new PA strain with a distinct genotypic profile, suggesting a new source of contamination. Five patients had two PA isolates with identical genotypes, suggesting either previous undetected respiratory tract colonization or a persistent environmental source of contamination.

Role of embolisation in the treatment of bronchopulmonary sequestration.

BACKGROUND: Sequestrations represent bronchopulmonary malformations that are increasingly diagnosed antenatally. After birth, the therapeutic approach in asymptomatic children is debated, as some may spontaneously regress. OBJECTIVE: To evaluate the efficacy of embolisation of the feeding systemic artery in the treatment of bronchopulmonary sequestration. MATERIALS AND METHODS: Sixteen children with bronchopulmonary sequestration were treated by endovascular embolisation of the feeding systemic artery. RESULTS: Ten patients were considered cured by embolisation alone. One patient was operated on after unsuccessful embolisation, three had partial regression of the lung mass and two are still under follow-up. CONCLUSIONS: Our experience indicates that bronchopulmonary sequestrations in children can be treated by embolisation alone.

[Subacute extrinsic allergic alveolitis with lesion edema]. / Alvéolite allergique extrinsèque suraiguë avec oedéme lésionnel.

BACKGROUND: Extrinsic allergic alveolitis may have a chronic, subacute or acute course. Its fulminant form with interstitial edema is unusual. CASE REPORT: An 11-year-old girl was referred to our intensive care unit because she suffered from high fever. Chest X-rays showed bilateral alveolitis. History revealed home exposure to several birds for many years, and episodes of dyspnea and fever for a few months. Diagnosis of extrinsic allergic alveolitis was confirmed by strongly positive results of precipitins to avian antigens. In addition, a paramyxovirus influenzae was recovered from broncho-alveolar lavage. Treatment, including high dose steroids, hydroxychloroquine, and suppression of antigenic exposure allowed weaning from mechanical ventilation after 50 days. Severe pulmonary restriction was disclosed by lung function testing following weaning and slow improvement occurred 5 months later, despite persistent interstitial lesions on chest X-rays. CONCLUSION: Extrinsic allergic alveolitis may mimic an acute respiratory distress syndrome. Documenting bird exposure in such a case helps to achieve appropriate diagnosis which requires early administration of steroids in order to improve vital and functional prognosis.

[Value of ipratropium bromide in asthma crisis in children]. / Intérêt du bromure d'ipratropium dans la crise d'asthme de l'enfant.

Ipratropium bromide is a synthetic derivative of atropine with little absorption when used in inhalation, and therefore little secondary effects. The authors review its pharmacological properties and therapeutic efficacy in the treatment of asthma in children. Combined nebulized inhalation of ipratropium bromide and beta 2 sympathomimetic results in a more efficient and more sustained bronchodilatation than beta 2 sympathicomimetic alone in the treatment of acute asthma in children. Ipratropium bromide should be usefully introduced in the therapeutic scheme of acute asthma in children. Further studies will be necessary in order to determine its efficacy and tolerance in infants.

[Plummer-Vinson syndrome or related syndrome in 3 black African women]. / Syndrome de Plummer-Vinson ou syndrome apparenté chez trois africaines de race noire.

The Plummer-Vinson syndrome or "sideropenic dysphagia" is exceptional among Blacks. One case was recently reported in a female patient from Guadeloupe. This study pertains to three cases observed in Senegalese Black women aged 28, 27, and 41 years. These three women were admitted for a dysphagia, in fact in evidence 10, 4, and 7 years respectively before the diagnosis was made. A clinical anemia was noted twice in addition to mucocutaneous disorders (cases 1 and 2). The laboratory tests showed in all three cases a hypochromic microcytic sideropenic anemia (serum iron levels at 32, 14, and 31 mcg 100 ml respectively). Barium swallow films showed esophageal rings in front of C5-C6 (case 1) of T2-T3 (case 2) and a web of fine mucosal folds in front of C5-C6 (case 3). These films were confirmed cineradiographically by esophagoscopy. The treatment consisted of blood transfusions (cases 1 and 2) and administration of iron by injections and or per os. The endoscopic exams were repeated two or three times. Medical treatment rapidly changed the course of disease for the better. No cause for bleeding was found. A chemical achlorhydria (case 1), a provoked hypoachlorhydria (cases 2 and 3) can be retained as associated factors. In light of the frequency of esophageal membranes in the general population and the incidence of sideropenic anemias among African women, the Plummer-Vinson syndrome should be more often detected in Black Africa.