Mounier Kuhn syndrome presenting with recurrent atelectasis.

Objective and importance Mounier Kuhn syndrome is usually diagnosed in adulthood, and only a few cases have been described in childhood. Clinical presentation We present the case of a seven-year-old boy suffering from recurrent pneumonia and atelectasis. Intervention Previously performed chest X-rays showed bilateral hyperinflation and tracheobronchomegaly. Chest computed tomography (CT) confirmed the presence of distal enlargement of trachea and bronchi. Tracheobronchomegaly associated with recurrent respiratory tract infections is consistent with Mounier Kuhn syndrome. Pseudomonas aeruginosa was isolated from the sputum of the patient. He was then treated according to the guidelines for P. aeruginosa management in cystic fibrosis patients considering the similarities in clinical presentations and pathophysiology of both diseases. Antibiotic treatment resulted in a remarkable reduction of events of pulmonary exacerbation and hospitalizations. There are no specific guidelines for treatment options in case of pulmonary exacerbation of Mounier Kuhn syndrome. Case reports discussing the choice and efficiency of antibiotic treatment are random. Conclusion headings We share our experience of treating pulmonary exacerbation caused by P. aeruginosa in a patient with Mounier Kuhn syndrome suggesting a possible treatment option of pseudomonas infections in this syndrome.

Adherence to asthma treatment in childhood and adolescence - a narrative literature review.

OBJECTIVES: We provide a narrative literature review on surveys used to assess the level of medication adherence in children and adolescents with asthma, the attitudes of these patients and their parents toward asthma therapy, and their expectations concerning asthma and available treatment. METHODS: A PubMed search and manual selection of the retrieved papers was conducted to identify studies using surveys or interviews that addressed one of the three topics of interest. RESULTS: Adherence to asthma medication varies across age groups and with the type of measurement used. Levels of 49-71% were observed in children and adolescents by objective measurements. Subjective measurements overestimate the level of adherence compared to objective measurements. A considerable percentage of parents expressed fear of side effects of inhaled corticosteroids, although the impact of these concerns on adherence is unclear. Many adolescents and parents adapt inhaled corticosteroids use according to the prevalence of asthma symptoms, by reducing or eliminating controller medication in the absence of symptoms. Pediatric asthma patients and their parents tend to overestimate the level of asthma control, either by underestimating asthma severity or by assuming that a better control is not possible. The knowledge of parents and adolescents concerning asthma management is suboptimal; moreover, insufficient knowledge about inhaled corticosteroids was linked to poor adherence. CONCLUSION: Medication adherence is crucial for a good control of asthma symptoms. Additional research concerning the triggers of non-adherence is still needed. Educating both the patients and their parents on proper asthma care might improve adherence.

Predicting quality of life in pediatric asthma: the role of emotional competence and personality.

PURPOSE: The present study examined the predictive value of emotional competence and the five-factor model of personality on the quality of life of children with asthma. METHODS: Participants were 90 children (M age = 11.73, SD = 2.60) having controlled and partly controlled asthma, undergoing everyday treatment. Children filled in questionnaires assessing emotional competence and quality of life. Parents completed questionnaires assessing the personality of their child. RESULTS: Results showed that two emotional competences, bodily awareness and verbal sharing of emotions, were related to the quality of life of children with asthma. Moreover, one personality trait, benevolence, was associated with children's quality of life. Regression analyses showed that the predictive value of these three dimensions remained significant over and above asthma control and socio-demographic variables frequently associated with the quality of life of children with asthma (age, gender, and educational level of parents). CONCLUSIONS: These findings emphasize the importance of alerting the clinician who works with children with asthma to observe and assess the child's expression of emotions, attention to bodily sensations, and benevolence.

Emotional competence and quality of life of children with asthma: The mediating effect of coping strategies.

This study explored the mediating effect of coping strategies on the relationship between emotional competence (EC) and quality of life (QOL) among children with asthma. Participants were 87 children (M age = 11.72, SD = 2.58) with controlled and partially controlled asthma, undergoing everyday treatment. They filled in questionnaires assessing EC, coping strategies and QOL. Results showed that the association between some ECs and the QOL of children with asthma was fully mediated by two maladaptive cognitive coping strategies. Among children with asthma, a greater ability to differentiate their emotions, a reduced attention to bodily signals of emotions and a reduced analysis of their current emotional state were related to decreased engagement in two coping strategies ('Ignoring Asthma' and 'Worrying about Asthma'), which in turn increased their QOL. These findings show that EC has an indirect effect on QOL through very specific coping strategies. They also emphasise the importance of screening EC in children with asthma and the importance of developing and using multidisciplinary interventions for them.

Psychometric properties of the emotion awareness questionnaire for children in a French-speaking population.

In this study, we investigated the psychometric properties of the French version of the Emotion Awareness Questionnaire (EAQ30; Rieffe et al., 2008). The EAQ30 was administered to 707 French-speaking children aged 8 to 16 years old. The original 6-factor structure was replicated in our data. The internal consistency coefficients of the EAQ30 subscales were satisfactory. We found small significant differences for gender and age. Regarding convergent validity, we found positive correlations between EAQ30 scores and emotional intelligence and negative correlations between EAQ30 scores and alexithymia. There was preliminary evidence of discriminant validity, with EAQ30 scores being weakly related to school performance, and concurrent validity, with EAQ30 scores being negatively related to somatic complaints, depression, and anxiety. Finally, except for 1 dimension, EAQ30 scores were not susceptible to social desirability. Although some weaknesses of the scale remain to be addressed, these findings support the use of the EAQ30 for research and clinical purposes.

Submandibular lymphadenitis caused by Mycobacterium interjectum: contribution of new diagnostic tools.

Mycobacterium interjectum is a rare causative agent of cervical lymphadenitis. We describe a 2-year-old girl with suspected tuberculous cervical lymphadenitis.Sequencing of the 16S rRNA gene allowed the correct identification of Mycobacterium interjectum. As yet, only nine case reports of infections due to M. interjectum in children have been reported in the literature, and in all of them a correct identification could only be obtained using gene sequencing.

Prediction of Lyme meningitis based on a logistic regression model using clinical and cerebrospinal fluid analysis: a European study.

BACKGROUND: A prediction model based on clinical and cerebrospinal fluid (CSF) analysis has been proposed for the differentiation of Lyme meningitis (LM) from non-Lyme aseptic meningitis (NLAM) in the United States. No similar model has ever been proposed for European patients. The objective of our study was to develop a prediction model to differentiate LM from NLAM based on clinical and CSF biologic data. METHODS: The medical charts of all children older than 2 years of age admitted to our hospital from 1996 through 2006 with a definite diagnosis of LM were analyzed and compared retrospectively with those having a diagnosis of NLAM. Chart review included the duration of symptoms, the presence of cranial neuropathy, and CSF analysis. RESULTS: A total of 93 patients were included (LM: 26 patients; NLAM: 67 patients) in the study. Patients with LM had statistically more frequent cranial neuropathy (73% vs. 4%), displayed a longer duration of symptoms before admission (8.8 vs. 1.8 days), had a higher CSF protein (71 vs. 38 mg/d), and had a lower percentage of neutrophil cells in the CSF (3.4% vs. 51%) than patients with NLAM. A predicted probability was derived from these 4 variables. At a cutoff point of >0.432, the model had a negative predictive value of 100% and a positive predictive value of 92.3%, with a sensitivity of 100% and a specificity of 97%. CONCLUSIONS: We report the first European prediction model for LM. Owing to its high negative predictive value, this model may assist physicians in managing aseptic meningitis (AM) while awaiting serologic tests, especially in Lyme endemic regions.

Severe ostial stenosis of the left coronary artery in a 12-year-old girl as the first manifestation of Takayasu's arteritis.

We report the case of a 12-year-old girl who presented with isolated chest pain on minimal exertion over the last 2 months. A coronary angiography revealed severe narrowing of the left coronary artery ostium and increased thickness of the ascending aortic wall was demonstrated by transoesophageal echocardiography, suggesting the diagnosis ofTakayasu's arteritis. The patient was successfully treated with corticosteroids and coronary artery bypass graft surgery.

Intrathoracic lymphadenitis caused by Mycobacterium avium and Mycobacterium tuberculosis in an immunocompetent child.

By means of a DNA probe assay (INNO-LiPA) we identified 2 different mycobacterial strains (Mycobacterium avium and Mycobacterium tuberculosis complex) from a mediastinal lymph node biopsy obtained from an apparently immunocompetent 7.5-year-old girl, whereas culture grew only M. avium. Clinicians should be aware of the possible occurrence of mixed infection involving both nontuberculous mycobacteria and M. tuberculosis.

Exercise-induced respiratory symptoms are poor predictors of bronchoconstriction.

Exercise-induced asthma (EIA) is a possible cause of poor physical performance in children. No data are available on the value of respiratory symptoms to discriminate children with bad physical fitness from children with EIA. We evaluated respiratory symptoms in school-age children during and after exercise in relation to EIA. The population of 149 primary schools (849 classes with 15,241 children) was enrolled in the study. EIB was assessed using the 6-min free-running-test (6MFRT) in 15,241 children. At the end or at premature arrest of the 6MFRT, signs reported by the children and clinical symptoms observed by supervising physicians were recorded. Peak flow measurements were obtained before and 5 and 10 min after the 6MFRT, a decrease of 15% or more being defined as significant. The 6MFRT was positive in 7.4% of primary schoolchildren. Girls were more likely to have a positive test than boys (8.5% vs. 6.4%, P < 0.001), and children living in urban areas more than those living in rural areas (8.9% vs. 7.0%, P < 0.01). Premature arrest was seen in 3.5% (i.e., n = 353) of the children. The main reasons for premature arrest were dyspnea and chest pain. Among them, only 21% had a positive 6MFRT, while 89% with a positive 6MFRT could terminate the test. Premature arrest, breathlessness, chest tightness, wheezing, and cough had a positive predictive value to detect a EIA of 21.9%, 20.8%, 36.4%, 41.2%, and 28.3%, respectively, and a sensitivity of 10.8%, 29.5%, 14.9%, 4.8%, and 13.0%, respectively. In conclusion, EIA was detected in 7.4% of schoolchildren. A slightly higher incidence was observed in girls and children from an urban area. Neither premature arrest nor clinical signs and symptoms were good predictors of EIA in primary schoolchildren.

Successful use of intralesional and intravenous cidofovir in association with indole-3-carbinol in an 8-year-old girl with pulmonary papillomatosis.

We report the history of an 8-year-old girl who was treated for suspected lung lesions of respiratory papillomatosis with Indole-3-Carbinol, local and intravenous injections of Cidofovir for 27 months. This is the first report where a complete cure of the lung lesions occurred in a child, and was sustained for at least 24 months.

Pneumococcal psoas pyomyositis associated with complement deficiency.

A 4.5-year-old boy with complement deficiency developed infection of the psoas caused by Streptococcus pneumoniae. Pyomyositis of the psoas muscle is uncommon but should be included in the differential diagnosis of fever and lameness. The most useful diagnostic test is computed tomography guided needle aspiration, and underlying conditions should be sought.

Retropharyngeal and mediastinal abscess following adenoidectomy.

Adenoidectomy is one of the most common surgical procedures in children. On rare occasions it can be complicated by a retropharyngeal abscess (RA). We report the case of a 9-year-old girl with an RA extending to the mediastinum following adenoidectomy. The mediastinal collection was successfully treated with computed tomography-guided percutaneous drainage to avoid extensive surgical procedure. Surgeons should be aware of these dramatic complications and should avoid overzealous surgery in the removal of the adenoid tissue.

Clinical data and cerebrospinal fluid findings in Lyme meningitis versus aseptic meningitis.

The purpose of this study was to characterise Lyme meningitis (LM) in a Belgian paediatric population and to suggest findings that could allow early distinction from aseptic meningitis (AM). The medical records of patients hospitalised between 1993 and 2000 and with a discharge diagnosis of LM (n=14) or AM (n=16) were retrospectively reviewed. The Kruskal-Wallis test was used to compare data. Of the patients, 16 were identified with AM and 14 with LM, amongst which 7 presented with isolated LM and 7 with associated peripheral facial palsy (PFP). Patients with AM, when compared with LM, complained of more pronounced signs and symptoms of meningitis (fever, headaches, and neck stiffness); they statistically displayed a shorter period of symptoms before admission (1.6 vs 15 days), higher neutrophilic component (mean 56% vs 2.4%), and lower protein levels (mean 0.39 vs 1.12 g/l) on cerebrospinal fluid analysis. In the neuroborreliosis group, the duration of symptoms was shorter and the cerebrospinal protein level was lower in cases of LM associated with PFP compared to isolated LM (mean 1.3 vs 15 days; mean 0.55 g/l vs 1.12 g/l). Conclusions. Our results suggest that some clinical data and laboratory findings may help the physician to diagnose aseptic or Lyme meningitis before completion of serologic testing. LM should be suspected in cases of meningitis with very low CSF neutrophilic counts and high protein levels associated with prolonged duration of symptoms, low grade fever, and absence of pronounced signs of meningitis.