Introduction: Protracted bacterial bronchitis (PBB) is defined as the presence of more than 4 weeks of chronic wet cough that resolves with appropriate antibiotic therapy, in the absence of alternative diagnoses. Microbiological confirmation of PBB poses a significant challenge as affected children rarely expectorate sputum. The gold standard method of sampling the lower airways in young children is flexible bronchoscopy with bronchoalveloar lavage (FB-BAL) The objective of this study was to analyze the bronchoscopic findings of children suspected with PBB. Methods: We retrospectively reviewed 31 children investigated for suspected PBB between January 2011 and December 2014 at Hacettepe University Pediatric Pulmonology Department. Results: The median age at bronchoscopy was 7(2.5–14) years and the median duration of cough was 11(6–48) months. On physical examination 23 patients have no findings. Positive cultures were obtained from 12/31 (38.7%). Bronchoscopy revealed out purulent secretion in 16 patients, bronchomalasia in 2 patients and tracheomalasia in 1 patient. The commonest organisms isolated were Streptococcus pneumoniae (7 patients) and Haemophilus influenzae (5 patients). Conclusion: In conclusion, a substantial number of children with chronic wet cough can be found to have a bacterial infection of the lower airway. Bacterial distribution in the lungs of children with PBB is heterogeneous and organisms may therefore be missed. Presence of purulent bronchitis during bronchoscopy is strongly associated with bacterial lower airway infection in this population of patients. The predominance of airway malacia in these patients suggests an etiologic role for those airway anomalies.
Tracheobronchomegaly is a rare disorder characterized by marked dilatation of trachea and major bronchi. We report a 10-year-old child with tracheobronchomegaly presenting with localized bronchiectasis and mimicking foreign body aspiration. The diagnosis was confirmed via flexible bronchoscopy. We believe that flexible bronchoscopy can provide useful information in children with a prolonged history of respiratory symptoms and a large trachea in whom the diagnosis of tracheobronchomegaly is considered.
Introduction: Filamin A (FLNa) is the first actin filament cross-linking protein identified in non-muscle cells. Mutations in the X-linked gene encoding filamin A (FLNA) cause cerebral periventricular nodular heterotopia, cardiac valvular disease and skeletal anomalies to a variable degree; recently lung involvement has been defined and emphysematous lesions in lung parenchyma are the characteristic findings of this mutation. Case Report: Here we present the clinical, radiological, and pathological features of five children that we think clinical findings may be associated with FLNA mutation. All presented with cough at ages of five, six, ten and eight years. One patient had exercise induced dyspnea. Chest X-ray and chest CT showed multiple lung cysts in three patients and emphysematous changes in one patient. One patient had intracranial cyst and two patients had sensorineural hearing defect. Joint hypermobility was positive in two patients. Lung biopsy has been performed and emphysematous changes revealed out in this patient. Genetic analysis is being processed now. Conclusion: We think that these severe lung manifestations may have resulted from FLNA mutation. Extra-neurological features have already been described in patients with X-linked periventricular nodular heterotopia, and lung manifestations may be another expression of this multisystem disorder. The occurrence of emphysema, aortic aneurysm, joint hypermobility, skeletal dysplasia, otopalatodigital spectrum disorders, epilepsia, dyslexia should be considered that FLNA can cause this phenotype.
This study aimed to examine early motor repertoire using Prechtl General Movement Assessment (GMA) and later developmental functioning of infants with cystic fibrosis (CF).Early motor repetoire was evaluated using Prechtl GMA, and developmental functioning was assessed using Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III) in infants with CF and their peers who were neurotypical, as the control group.Twelve infants with CF clinically stable and 12 infants who were neurotypical, with respective median post-term ages of 14 and 13 weeks, were assessed using GMA. At 24 to 36 months, the Bayley-III was applied to the CF group (median post-term age = 27.5 months) and the control group (median post-term age = 27.0 months). Fidgety movements were absent in 5 infants with CF, whereas all infants who were neurotypical had normal fidgety movements. The Motor Optimality Score was significantly lower in the CF group (median = 18.5) compared with the control group (median = 26). The CF group had significantly lower composite scores in the Bayley-III cognition, language, and motor domains compared with the control group.Cognitive, language, and motor development was delayed in infants with CF. Developmental functioning of infants with CF should be assessed as early as possible and monitored, and age-specific early intervention programs should be considered when necessary.Infants with CF may have motor, cognitive, and language developmental delays compared with peers who are neurotypical during early childhood, and hospitalization was negatively correlated with motor development at 24 to 36 months of age. This study highlights the importance of early assessment of developmental functioning and age-specific, early intervention programs when necessary in infants with CF.It is important to assess developmental functioning as early as possible in infants with CF and to consider age-specific early intervention programs when necessary.
Background: Considering COVID-19 pandemic, self-isolation is recommended for preventive strategy in cystic fibrosis (CF) patients. Long-term social isolation and school closure may have adverse effects on physical health. Aim: This study aimed to examine the effect of a 6-week online exercise protocol on peripheral muscle function of pediatric CF patients. Methods: Thirty CF patients (age=11.16±3.02 years) were included in the study. Patients were allocated to either the telerehabilitation or control group. Exercise protocol was included exercises such as posture exercises, squat, crunch, oblique crunch, lunge. Online supervised exercise training was applied three times per week for six weeks in the telerehabilitation group. Same exercises were sent as an exercise brochure to the control group, and phone contacts were applied once a week to learn their session number. One session was lasted 30-45 minute. All patients were assessed for peripheral muscle function with the number of crunch repetitions, push-ups and squats before after the exercise training. Results: Peripheral muscle function parameters were increased each group. Patients who had an online exercise training improvements in crunch, push-up and squat were increased higher than the control group (p=0.008, p=0.015, p=0.002, respectively). Conclusions: Exercise training with online-supervised or follow-up is a beneficial effect on peripheral muscle function in CF patients. But this benefical effect is more in online-supervised exercise training. Exercise training programs can be applied with telerehabilitation approaches to decreased immobility due to social isolation in CF patients.
Background: Allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) is characterized by destructive changes in the airways. Long-term treatment with oral corticosteroids is often required for repeated exacerbations. Because elevated total IgE is a cardinal abnormality of ABPA, omalizumab has been used sporadically to decrease corticosteroid dose or totally replace corticosteroids. Objective: The aim of this report is to describe our experience with omalizumab treatment in patients with CF and ABPA. Methods: We conducted a review of 6 CF patients with ABPA receiving omalizumab. All patients were treated with oral prednisolone and itraconazole. Omalizumab was started if the patient was not responding to steroid treatment, which was determined according to serum IgE levels and/or clinical findings or depending on if there were side effects caused by steroid treatment. Results: The mean age of patients at the beginning of omalizumab treatment was 16.1 years. One patient had a new diagnosis of ABPA; however, the others had the first to third exacerbation when treated with omalizumab. The mean duration of ABPA by the time that treatment with omalizumab started was 13 ± 12.4 months (range = 2-29 months). With omalizumab treatment, IgE levels were decreased in all patients, and Aspergillus-specific IgE levels were decreased in 4 patients; however, FEV 1 (% predicted) improved only in 2 patients who had mild disease. Corticosteroids were reduced in the first, second, and third months of omalizumab treatment in 2, 1, and 3 patients, respectively. In 2 patients, steroid treatment was stopped. None of the patients suffered from side effects of omalizumab. The mean duration of omalizumab treatment was 12.5 months (range = 6-18 months). Conclusions: This study showed steroid-sparing effect, decreasing IgE levels, and improvement in respiratory symptoms in 6 CF patients with omalizumab treatment. Although this is a small sample of the population, omalizumab may be an alternative therapy for ABPA in CF patients who fail to respond to systemic corticosteroids or have serious adverse effects.
We reviewed our experience on tracheomalacia (TM) and bronchomalacia (BM) in children who presented with chronic respiratory problems and evaluated their clinical and radiologic characteristics and their associations with other disorders. There were 26 males and 8 females with a median age of 9 months. The main symptoms were wheezing, persistent or recurrent pneumonia, and chronic cough. Atelectasis on chest radiograph was the most common sign. Of 23 children with TM, 1 had a double aortic arch, 1 had tracheoesophageal fistula, and 1 other had associated laryngomalacia. BM was found in 27 children and was predominantly seen on the right side. TBM was found in 16 cases (in an infant TBM was accompanied by pharyngeal dyskinesia and in another by laryngomalacia). Malacia disorders were associated with gastroesophageal reflux, cardiovascular anomalies, and tracheoeosophageal fistula. TM and BM should be considered in the differential diagnosis of children with chronic and recurrent respiratory symptoms. Early diagnosis of malacia disorders will prevent unnecessary use of antibiotics or antiasthmatic drugs, which are often abused to treat these children. In these patients, treatment for associated diseases should also be considered.
n the immediate vicinity of the cells have a direct effect on cells’ behaviour and subsequently on clinical outcomes. Physical, chemical , and biological control of cell microenvironment are of crucial importance for the ability to direct and control cell behaviour in 3-dimensional tissue engineering scaffolds spatially and temporally. In this review, we will focus on the different aspects of cell microenvironment such as surface micro-, nanotopography, extracellular matrix composition and distribution, controlled release of soluble factors, and mechanical stress/strain conditions and how these aspects and their interactions can be used to achieve a higher degree of control over cellular activities. The effect of these parameters on the cellular behaviour within tissue engineering context is discussed and how these parameters are used to develop engineered tissues is elaborated. Also, recent techniques developed for the monitoring of the cell microenvironment in vitro and in vivo are reviewed, together with recent tissue engineering applications where the control of cell microenvironment has been exploited. Cell microenvironment engineering and monitoring are crucial parts of tissue engineering efforts and systems which utilize different components of the cell microenvironment simultaneously can provide more functional engineered tissues in the near future.
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