Systematic skin and nasal decolonization lowers Staphylococcus infection in pediatric cardiac surgery.

BACKGROUND: Postoperative infections occur in approximately 10% of pediatric cardiac surgeries, involving Staphylococcus species in most cases. Nasal decontamination of Staphylococcus with mupirocin has been reported to reduce postoperative Staphylococcus infections after cardiac surgery in adults, but the effect of preoperative decontamination in children undergoing cardiac surgery has not been sufficiently studied to reach consensus. METHODS: We conducted a single-center retrospective study to evaluate the impact of systematic preoperative decolonization with intranasal mupirocin application and skin-washing with chlorhexidine soap on postoperative Staphylococcus infection in children undergoing cardiac surgery. Our population was divided into three groups according to decolonization protocol (group N: no decolonization; group T: targeted decolonization in Staphylococcus aureus [SA] carriers only; and group S: systematic decolonization). RESULTS: A total of 393 children were included between October 2011 and August 2015 (122 in group N, 148 in group T, and 123 in group S). The Staphylococcus infection rate significantly decreased in group S compared to group N (0.8% vs. 7.7%; p < 0.05) and tended to decrease in group S compared to group T (0.8% vs. 4.7%; p = 0.06). Systematic decontamination also significantly reduced the rate of infections starting from the skin (including surgical site infections and bloodstream infections) compared to targeted decolonization or lack of decolonization, but had no effect on the rate of pulmonary infections. CONCLUSION: The results of our study suggest that systematic preoperative skin and nasal decontamination, regardless of SA carriage status, could reduce the rate of postoperative Staphylococcus infections after cardiac surgery in children.

[Cardiomyopathy induced by frequent premature ventricular contractions]. / Myocardiopathie rythmique induite par des extrasystoles ventriculaires chez un enfant.

We report on the rare case of 7-year-old child presenting with frequent and apparently benign premature ventricular complexes (VPC) and left ventricular dysfunction. No structural disease of the heart was seen at cardiological evaluation. Eighteen months after the introduction of an antiarrhythmic treatment with low-dose bisoprolol, Holter monitoring showed 70,000 VPC in 24h with left ventricular function remaining decreased. Six months after increasing the dose of bisoprolol, the VPC were less frequent and left ventricular function was completely normalized. This case was characteristic of a cardiomyopathy induced by frequent premature ventricular contractions.

[Heart murmurs in asymptomatic children: When should you refer?]. / Souffle cardiaque chez l'enfant asymptomatique : quand demander un avis cardiologique ?

Heart murmurs are common in children and adolescents. Although most are innocent, an isolated heart murmur in asymptomatic children may be the sole finding indicating serious heart disease. Historical elements of familial heart disease, cardiovascular symptoms and a well-conducted medical examination can identify children with an increased risk of heart disease. The distinction between an innocent heart murmur and a pathologic heart murmur is not always easy for primary care physicians because most of them have little experience with auscultation searching for congenital heart malformation. Echocardiography provides a definitive diagnosis of heart disease but is not required in case of innocent murmur. Inappropriate pediatric cardiologist and echocardiographic referral leads to useless and expensive examinations, resulting in a work overload for pediatric cardiologists. The objective of this review is to provide the keys to differentiate innocent and pathologic murmurs, and to help physicians decide on the optimal diagnostic strategy.

Characteristics of endobronchial primitive tumors in children.

Primary endobronchial tumors are rare in children and they include a broad spectrum of lesions. The aim of this study was to determine the characteristic features, treatments and outcomes of these tumors. We report a retrospective analysis of all patients treated for endobronchial tumor in nine French hospitals between 1990 and 2010 and a comparison of the results with those reported in the medical literature. Twelve tumors were reported: five low grade muco epidermoid carcinomas, two inflammatory myofibroblastic tumors, two hemangiomas, one anaplastic large cell lymphoma, one carcinoid tumor, and one juvenile xanthogranuloma. The mean age of the patients was 7.5 ± 3.5 years. The most common sign revealing the disease was persistent atelectasis or recurrent pneumonia (eight cases). The other revealing signs were a persistent bronchospasm (three cases) and hemoptysis (one case). The clinical presentation, biology, serum tumor markers, and chest X-ray abnormalities were not specific to a particular histological diagnosis. Chest CT scan revealed the presence of an endobronchial tumor in 11 cases. Nine tumors could be diagnosed from a biopsy obtained by video endoscopy. Complete surgical resection was performed in seven patients. Bronchoscopic removal was performed in five cases and was successful in three. There were no deaths. Endobronchial tumors are rare in childhood and their histology is diverse. Chest CT scan and per-endoscopic endobronchial biopsies are required for diagnosis, when possible. Surgical or endoscopic treatment should be discussed by a multidisciplinary team. Despite the multiple etiologies, the prognosis of these tumors is good if diagnosis is early and if resection is complete. Long-term recurrences have been described, so long-term follow-up of these children is recommended.

[Donohue syndrome or leprechaunism]. / Le syndrome de Donohue ou lepréchaunisme : à propos d'un cas.

Donohue syndrome or leprechaunism is a severe congenital insulin-resistance syndrome. It is characterized by intra-uterine and neonatal growth retardation, typical dysmorphic features, and metabolic abnormalities with hyperinsulinism and hyperandrogenism. Problems in energy metabolism and loss of glucose homeostasis are responsible for early death in the first year of life. We describe a case with a novel homozygote mutation in the insulin receptor gene. This patient had hypertrophic cardiomyopathy with heart failure and bronchial compression leading to clinical deterioration over 5 days and subsequently death. A treatment with recombinant IGF-1 was tried without efficacy.

[Atrioventricular block after transcatheter atrial septal defect closure using the Amplatzer septal occluder(®)]. / Bloc auriculo-ventriculaire après fermeture d'une communication interauriculaire avec un système Amplatzer septal occluder(®)

Transcatheter closure of atrial septal defect (ASD) in children is now an accepted treatment as an alternative to surgery. We report a case of complete atrioventricular block (AVB) observed over several days after closure of an ASD with an Amplatzer Septal Occluder(®) in a 13-year-old child. The spontaneous outcome was good with regression of the complete AVB, but 1 year later, a first-degree AVB was still present. The use of large devices in young children and a small postero-inferior rim seem to be risk factors of AVB. Transcatheter closure of ASD requires close monitoring of cardiac rhythm.

[Evaluation of an educational program for oral anticoagulation in children with the INR home-monitoring CoaguChek XS(®)]. / Évaluation de l'éducation au traitement anticoagulant et au système d'auto-mesure de l'INR CoaguChek XS(®) chez l'enfant.

OBJECTIVE: To assesses the results of our education program relative to anticoagulation therapy and self-monitoring of INR. MATERIALS AND METHODS: All children treated with oral anticoagulation therapy and followed using INR self-monitoring in Tours and Nantes, France, were included. A questionnaire on the treatment and its management was sent to the patients and their family. We analyzed the quality of anticoagulation using the proportion of INR within the target range. RESULTS: Thirty-three children were included, with a mean age of 10.9years. Thirty-one questionnaires could be analyzed. Insufficient knowledge on the treatment objective and risks, the INR target range, and the interpretation of the INR was observed. We found 65.8% of INRs within the target range. The proportion of INRs within the target range was better with the INR self-testing than with the laboratory test (69.7% vs. 49.1%, P=0.003). There was no major complication observed during the study period. A moderate correlation (K=0.57) was noted between the INRs measured with the point-of-care monitor and the laboratory test on the same day. CONCLUSION: However, home-monitoring of oral anticoagulation therapy increases the quality of anticoagulation. Both education and the training program need improvement in order to provide patients and their families with better knowledge on anticoagulation therapy.

[Atrial chaotic tachycardia during a respiratory tract infection due to NL63 coronavirus]. / Tachycardie atriale chaotique au cours d'une infection respiratoire à coronavirus NL63.

We report the case of a 3-month-old boy hospitalized with acute bronchiolitis. Respiratory distress was associated with cardiogenic shock caused by chaotic atrial tachycardia. The cause of bronchiolitis was a coronavirus NL63 viral infection, confirmed in nasopharyngeal aspirations. The patient required intensive care including diuretics (furosemide), anti-arrhythmic drugs (amiodarone and digoxin), and inotropic drugs (milrinone and levosimendan) associated with mechanical ventilation. The outcome was favorable in 10 days and the sinusal cardiac rhythm was completely restored at discharge.

[Benefits of continuous subcutaneous insulin infusion in children with type 1 diabetes mellitus]. / Bénéfices de l'insulinothérapie par pompe chez les enfants diabétiques de type 1.

BACKGROUND: The Diabetes Control and Complications Trial clearly demonstrated the benefits of blood glucose control, especially in children and adolescents, in the prevention of long-term complications of type 1 diabetes (T1D). This can be achieved with intensive insulin treatment with either multiple daily insulin injections (MDI) or continuous subcutaneous insulin infusion (CSII), also known as insulin pump. The aim of this study was to compare glycemic control of T1D children treated with either CSII or MDI. PATIENTS AND METHODS: Thirty-eight T1D children treated with CSII were compared to 38 children treated with MDI, matched for age, gender, and duration of diabetes. Collected data, including daily doses of insulin in IU/kg/d, HbA1c levels, body mass index expressed in standard deviation/age, number of severe hypoglycemia episodes and of admissions related to T1D expressed in events/patient/year, were retrospectively collected every 3 months. RESULTS: There was no difference between the 2 groups at baseline. During the 3 years of follow-up, patients treated with CSII had lower daily doses of insulin (0.78 ± 0.19 vs. 0.87 ± 0.22 IU/kg/d, p<0.05), significantly lower levels of HbA1c (7.5 ± 0.6 vs. 8.0 ± 1.3 %, p<0.05), and a decreased number of admissions related to T1D (0.07 ± 0.14 vs. 0.17 ± 0.22 events/patient/year, p<0.05) than children treated with MDI. In contrast, body mass index and number of severe hypoglycemic episodes did not differ between the two groups. No diabetic ketoacidosis episode was recorded in either group. CONCLUSION: The results from this study suggest that treatment with CSII provided better metabolic control than treatment with MDI, in spite of lower daily doses of insulin and without increasing acute complications, in children with T1D.

[Evolution of prenatal diagnosis of congenital heart diseases in Indre-et-Loire (France) over a period of 15 years]. / Évolution du diagnostic anténatal des cardiopathies congénitales entre 1991 et 2005 dans une population limitée au département d'Indre-et-Loire.

UNLABELLED: The value of prenatal diagnosis of major congenital heart diseases (CHDs) has already been proved. In this study, we observed the evolution of the detection rate of CHDs and the quality of the diagnoses over a 15-year period in the Indre-et-Loire department of France. METHOD: Retrospective analysis of the quantitative and qualitative data of prenatal diagnosis between 2000 and 2005 (period 3) and comparison with studies conducted from 1991 to 1994 (period 1) and from 1995 to 1999 (period 2). The CHDs considered to be detectable are the major CHDs with neonatal symptoms. In order to analyze the quality of prenatal diagnosis, each diagnosis was classified as correct, false, or incomplete during period 3. RESULTS: Period 3: 65.7% detectable CHDs were screened. Using period 1 criteria for prenatal diagnosis, the percentage of CHDs detected for periods 1, 2, and 3 was 43.2, 66.7, and 78%, respectively. Between periods 2 and 3, the detection rate increased from 52 to 74% for CHDs diagnosed with the visualization of the outflow tract. It increased from 63 to 71% for CHDs diagnosed with a four-chamber view. During period 3, diagnoses made by a pediatric cardiologist were correct and complete in 80% of cases. It was less than 25% otherwise. CONCLUSION: The improvement of prenatal diagnosis over time results from technological progress, the greater experience of ultrasonographers, and the systematic visualization of the outflow tract in addition to the four-chamber view. Despite the improvement in prenatal diagnosis, one-third of major CHDs were not screened before birth. Pediatricians still need to diagnose CHDs after birth.

[Clinical variability and diagnosis steps in childhood mitochondrial disease]. / Variabilité clinique et conduite diagnostique des cytopathies mitochondriales : à propos d'une série de 18 cas pédiatriques.

OBJECTIVES: Mitochondrial respiratory chain deficiencies are known for their high clinical variability. Difficult to diagnose, the prevalence of these diseases is probably underestimated. METHODS: We report 18 children diagnosed with respiratory chain deficiency at the Tours University Hospital over the past 10 years. RESULTS: Three clinical profiles can be distinguished depending on the age at onset of the first symptoms: the neonatal period (4 cases), between 1 month and 2 years of age (10 cases), and after 10 years (4 cases). However, no clinical feature appears specific of any age group. In contrast, respiratory chain analysis on liver biopsy was very informative for all our patients at any age and with any clinical presentation, even with predominant neurological symptoms. CONCLUSIONS: These biochemical analyses support the diagnosis of mitochondrial disorders in view of molecular analysis, which nevertheless frequently remains inconclusive. These investigations should benefit from the new molecular screening technologies based on DNA chips that can identify the genomic mutations responsible for these severe and relatively frequent diseases.

[Enzyme replacement therapy in a boy with infantile Pompe disease: cardiac follow-up]. / Enzymothérapie substitutive chez un nourrisson atteint de maladie de Pompe : évolution cardiologique.

Pompe disease is an autosomal recessive glycogen storage disorder caused by acid-alpha-glucosidase deficiency. The infantile form is usually fatal by 1 year of age in the absence of specific therapy. We report the cardiac follow-up of a 4-month-old boy treated with enzyme replacement therapy (ERT) for 8 months. The patient had no cardiac failure at the age of 1 year. Before starting ERT, ECG showed a shortened PR interval, with huge QRS complexes and biventricular hypertrophy; echocardiography demonstrated major hypertrophic cardiomyopathy. The QRS voltage (SV1+RV6) decreased from 13 to 2.9 mV after 32 weeks of ERT, suggesting a progressive reduction of cardiac hypertrophy and intracellular glycogen excess. The PR interval increased from 60 to 90 ms. A block of the right bundle branch appeared after 13 weeks of treatment. The indexed left ventricular mass decreased from 240 to 90 g/m2 after 30 weeks of ERT. The left ventricular ejection fraction decreased transitorily between the 5th and the 15 th weeks of treatment. In summary, ERT is an efficient therapeutic approach for the cardiomyopathy of infantile Pompe disease. However, the possible occurrence of a right bundle branch block and a transitory alteration in the ejection fraction highlight the importance of cardiac follow-up.

[The role of aortic morphology in the occurrence of hypertension in patients undergoing surgery for coarctation of the aorta before the age of 1 year]. / Rôle de la morphologie aortique dans la survenue d'une hypertension artérielle chez des malades opérés d'une coarctation de l'aorte avant l'âge de 1 an.

OBJECTIVE: Hypertension on effort can be observed following surgical treatment of coarctation of the aorta even if the operation has been performed early and has been judged to be satisfactory. The pathophysiology of this hypertension has not been completely elucidated. The aim of our study was to look for a residual morphological anomaly of the aortic arch which might be responsible. METHODS: This was a retrospective study of patients aged over 10 years who had undergone surgery for coarctation of the aorta before the age of 1 year, consecutively between 1979 and 1993, and free from re-coarctation clinically or with Doppler. All of them had a blood pressure effort test on a cycling machine, echocardiography and an MRI of the thoracic aorta. RESULTS: 61 patients were included. Their median age was 15 years, 59% were male, 15% were hypertensive at rest and 56% on effort. With univariate analysis, only the average blood pressure (p=0.03) and the resting pulse pressure (p=0.001) were associated with hypertension on effort. With multivariate analysis the only factors that correlated independently with maximum arterial pressure on effort were the height of the patient (p=0.02) and the pulse pressure (p<0.0001). The surgical technique and the age at intervention were not associated with hypertension on effort (p=0.96 and 0.69 respectively). The diameter of the horizontal aorta and the aorta at the site of repair, measured on MRI, added to the diameter of the descending aorta at the diaphragm were not lower in patients with hypertension on effort (p=0.77 and 0.38). There were proportionally more Roman type aortic arches than Gothic types in patients with hypertension on effort, but this difference was not significant (p=0.18). CONCLUSION: In our study, the patients with hypertension on effort following correction of coarctation of the aorta did not display any residual obstruction at the level of the aortic cross on MRI. However, they had a resting pulse pressure that was significantly higher than the non hypertensive patients, reflecting an increase in the aortic wall rigidity.

[Quality of life of patients with total cavopulmonary anastomosis]. / Qualité de vie des patients avec une dérivation cavo-pulmonaire totale.

INTRODUCTION: Total cavo-pulmonary connection (TCPC) represents the current surgery standard for congenital univentricular heart disease (CUHD) patients. We studied the health related quality of life (HRQOL) expressed by TCPC operated patients. PATIENTS AND METHODS: This transversal study is based on 24 children and 10 adult TCPC patients aged from 5 to 41 years old. The HRQOL evaluation relies on generic self report instruments such as the PedsQL 4.0 for the children and their parents, and the Duke Health profile for the adult patients. Additional questions were specifically designed for this study as well. RESULTS AND DISCUSSION: The estimated HRQOL is 68.15 for the children and 68.71 for their parents. In these young cases, the global HRQOL or some specific score are altered when any comorbidity or scholar difficulties are present, or when TCPC was performed more than 10 years ago. The estimated HRQOL is 74.67 in adulthood. In these patients, the young age, the lifestyle, the onset of more than 2 early complications ans the necessity of anti-arrhythmia therapy decrease various HRQOL scores. The physical handicap is considered as the major limitation for children and adult patients. Thanks to adaptative and cognitive compensatory mechanisms, such as resilience, both groups express a satisfying HRQOL assessment. CONCLUSION: Our study indicates that TCPC provides a good HRQOL in CUHD patients despite the complex management and follow-up of these diseases, the risk of complication onset and the physical handicap usually observed.

Permanent junctional reciprocating tachycardia in children: a multicentre study on clinical profile and outcome.

OBJECTIVES: To investigate the clinical profile, natural history, and optimal management of persistent or permanent junctional reciprocating tachycardia (PJRT) in children. METHODS AND RESULTS: 85 patients meeting the ECG criteria for PJRT were enrolled in a retrospective multicentre study. Age at diagnosis varied from birth to 20 years (median 3 months). Follow up ranged from 0.1 to 26.0 (median 8.2) years. At the time of referral, 24 of 85 patients (28%) had congestive heart failure that was resolved with medical treatment in all patients. Eighty three patients received drug treatment initially. Amiodarone and verapamil were the most effective with a success rate of 84-94% alone or in association with digoxin. Radiofrequency ablation of the accessory pathway was performed in 18 patients. There was a trend for a relation between age at ablation and the result of the procedure, failures being more common in younger patients (three of six procedures in younger and 15 of 18 in older children were successful; p = 0.14). Two patients with persistent left ventricular dysfunction on echocardiography but with no symptoms of congestive heart failure died suddenly one month and three years after diagnosis. PJRT resolved spontaneously in 19 patients (22%). Age at diagnosis of PJRT was not a predictor of spontaneous resolution. CONCLUSIONS: PJRT is a potentially lethal arrhythmia in children with tachycardia induced cardiomyopathy. Spontaneous resolution of tachycardia is not uncommon. Antiarrhythmic treatment is often effective. Radiofrequency ablation should be performed in older children or when rate is not controlled, especially in patients with persistent left ventricular dysfunction.