Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children.

BACKGROUND: The prevalence of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and developing countries in recent years. Although the main features remain similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of our recent non-CF bronchiectasis patients and to compare these with our historical cohort in 2001. METHODS: One hundred four children with non-CF bronchiectasis followed between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months. RESULTS: Mean FEV1 and FVC %predicted at presentation improved compared to historical cohort (76.6 ± 17.1 vs. 63.3 ± 22.1 and 76.6 ± 15.1 vs. 67.3 ± 23.1, respectively; p <  0.001). There was a significant decrease in pulmonary exacerbation rate from 6.05 ± 2.88 at presentation to 3.23 ± 2.08 during follow-up (p <  0.0001). In 80.8% of patients, an underlying etiology was identified. There was an increase in primary ciliary dyskinesia (PCD) (32.7% vs. 6.3%; p = 0.001), decrease in idiopathic cases (19.2% vs. 37.8%; p = 0.03) with no change in postinfectious and immunodeficiencies as underlying etiology. Sputum cultures were positive in 77.9% of patients which was 46.9% in the historical cohort (p = 0.001). CONCLUSION: Baseline pulmonary function tests were better and distribution of underlying etiology had changed with a remarkable increase in diagnosis of PCD in the recent cohort.

Intrinsic endobronchial obstructions in children from Turkey: evaluation of 2,555 flexible bronchoscopic procedures.

BACKGROUND: Endobronchial obstructions are rarely seen in children and are often misdiagnosed resulting in delay of definitive treatment. A variety of diseases can cause endobronchial obstructions in childhood, but data is limited as to the frequency, distribution and clinical characteristics of endobronchial obstructions diagnosed with flexible bronchoscopy (FB). OBJECTIVE: To document endobronchial obstructions detected by FB. METHODS: FB results from three pediatric pulmonology centers in Istanbul were evaluated. RESULTS: A total of 2,555 children underwent an FB procedure during the study period. Endobronchial obstructions were detected in 10% (n = 256) of the patients. Among FB in patients who had endobronchial obstructions, the four most common indications for bronchoscopy were persistent infiltrations (30%, n = 72), persistent wheezing (28%, n = 70), chronic cough (26%, n = 66) and atelectasis (23%, n = 59). The most common endobronchial obstructions detected in the patients were aspirated foreign bodies (35.9%, n = 92), endobronchial tuberculosis (31.6%, n = 81), mucous plugs occluding airway (16.7%, n = 43) and granulation scars (6%, n = 16). Other pathologies included hydatid cysts (n = 5), hemangiomas (n = 5), tumors (n = 5), submucosal nodules (n = 5) and polyps (n = 4). Endobronchial obstructions were most commonly located in the right bronchus (51%, n = 130) followed by the left bronchus (33%, n = 85), bilaterally (8%, n = 21) and trachea (8%, n = 20). CONCLUSIONS: Endobronchial obstructions can be caused by a number of different diseases which require various medical or surgical treatments. In the presence of clinical or radiological findings suggesting an endobronchial obstruction, FB should be performed promptly.

Bronchoscopic evaluation of unexplained recurrent and persistent pneumonia in children.

BACKGROUND: Persistent or recurrent pneumonia in children can pose a significant challenge to paediatricians and respiratory physicians. AIM: The aim of this study is to determine the role of flexible bronchoscopy (FB) in evaluation of recurrent or persistent pneumonia that remain otherwise unexplained by non-invasive diagnostic tests in children. METHODS: Retrospective evaluation of patients who underwent FB with an indication of recurrent or persistent pneumonia from 1997 to 2011. RESULTS: Among 2600 FB procedures, 434 (17%) were performed with the indication of recurrent or persistent pneumonia. There were 237 (54%) boys. Median age at presentation was 84 months, and median duration of symptoms was 9 months. FB led to specific diagnosis in 33% of the cases. The most common diseases diagnosed by FB were malacia disorders (n: 32, 7%), aspirated foreign body (n: 30, 7%), endobronchial tuberculosis (n: 20, 5%), congenital airway anomalies (n: 14, 3%), mucus plugs (n: 14, 3%), pulmonary haemosiderosis (n: 12, 3%) and middle lobe syndrome (n: 11, 3%). During FB, only 6% of the patients had minor complications such as transient hypoxia, stridor and tachycardia. CONCLUSIONS: In our study, FB proved to be a safe and effective tool in evaluation of children with persistent or recurrent pneumonia. FB is indicated for children with recurrent or persistent pneumonia where the underlying diagnosis remains unclear even after non-invasive diagnostic tests.

Clinical and Radiological Evaluation and Follow-Up of Patients with Noncardiac Plastic Bronchitis.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease characterized by obstruction of the airway by fibrinous mucus plugs. The etiology can be idiopathic or secondary to systematic diseases such as congenital heart diseases. Definitive diagnosis is made by pathological examination of the sputum or bronchial sample taken by bronchoscopy. In this study, the clinical status and treatment status of patients with PB were evaluated. MATERIALS AND METHODS: Medical records of the patients diagnosed as PB were reviewed ret- rospectively. Age, gender, clinical symptoms, radiology, bronchoscopic findings, and pathology results were documented. RESULTS: Six patients with PB were included in this study (female:male, 2:4). The median age of the diagnosis was 45 months. The most common symptoms are persistent wet cough and short- ness of breath. The duration of symptoms ranged from 30 to 90 days. Atelectasis was the most common radiological finding. Diagnosis was made with pathological examination of the mucus in all patients. All of the patients were treated with bronchoscopic removal of the mucus, and 4 patients required oral prednisolone therapy. Symptoms and radiological findings resolved completely in all patients. CONCLUSION: Although PB is a rare disease, it should be kept in mind in relation to patients with persistent radiological and clinical respiratory symptoms.

Evaluation of diagnostic components and management of childhood pulmonary tuberculosis: a prospective study from Istanbul, Turkey.

INTRODUCTION: The diagnosis of childhood tuberculosis is difficult and most of the patients are diagnosed clinically. The objective of this study is to reveal the diagnostic and therapeutic components of childhood pulmonary tuberculosis and to analyze the changes that occurred in our country over the years. METHODOLOGY: All patients diagnosed with tuberculosis between 2006 and 2016 were included. Demographic characteristics, diagnostic and treatment outcomes were recorded and patients were followed up prospectively. RESULTS: A total of 492 patients were included in the study. 97% had Bacillus Calmette-Guerin vaccine, 36% were diagnosed with microbiologically-confirmed tuberculosis and 64% were diagnosed with clinically-proven tuberculosis. 94% of the patients had symptoms consistent with tuberculosis, all patients had radiologic findings, 74% had a history of tuberculosis contact and 63% had tuberculin skin test positivity. The diagnoses included primary tuberculosis in 62%, secondary tuberculosis in 21%, progressive primary tuberculosis in 13% and miliary tuberculosis in 4%. 48% of the patients received a treatment regimen containing three drugs as the initial treatment, and drug-related side effects developed in 12%. Isoniazid resistance was detected in 13% of the patients and rifampicin resistance was detected in 8%. None of the patients died due to tuberculosis. In the last 50 years in Turkey, the rates of Bacillus Calmette-Guerin vaccination and diagnosis of tuberculosis cases have increased and the mortality rates have decreased over the years. CONCLUSIONS: Our study is one of the few prospective studies and revealed the differences between the recent data and the past 50 years in childhood tuberculosis in Turkey.

The effect of peripheral blood eosinophilia on inflammatory markers in asthmatic patients with lower respiratory tract infections.

BACKGROUND: To investigate the behaviour of the inflammatory marker neutrophil-to-lymphocyte ratio (NLR) in the presence of peripheral blood eosinophilia (PBE) in paediatric asthma patients with lower respiratory tract (LRT) infections. METHODS: This retrospective study enrolled consecutive patients aged ≥5 years who were diagnosed with asthma and whose haemogram values were available. The patients were further subdivided based on the presence or absence of LRT infections and allergies. NLR and C-reactive protein (CRP) were evaluated in relation to the presence or absence of PBE (≥4% eosinophils). RESULTS: A total of 991 patients were enrolled in the study. Patients with LRT infections had significantly higher leucocyte and neutrophil counts, a greater NLR and a higher level of CRP compared with patients without LRT infections. Overall, patients with PBE had significantly lower NLRs and CRP regardless of the presence or absence of an LRT infection. The PBE percentage showed moderate inverse correlations with NLR ( r = -0.34) and CRP ( r = -0.20). CONCLUSION: The presence of PBE was significantly associated with lower NLR and CRP regardless of the presence or absence of an infectious condition.

Tracheobronchial calcification associated with Keutel syndrome.

Tracheobronchial cartilage calcification is an unusual radiologic finding in infants and children under 15 years old. Keutel syndrome is a rare, autosomal recessive disorder characterized by diffuse cartilage calcification, brachytelephalangia, pulmonary stenosis and midfacial hypoplasia. We report two children in whom abnormal tracheobronchial calcification was associated with Keutel syndrome. Keutel syndrome should be considered in the differential diagnosis of children with tracheobronchial calcification.

Classic kaposi sarcoma with pulmonary involvement mimicking endobronchial tuberculosis in a child.

Kaposi' sarcoma (KS) is a low-grade vascular neoplasm and classic KS, a subtype of KS, is extremely rare in children. Childhood pulmonary involvement in classic KS has not been reported in the literature. We describe an HIV-seronegative pediatric case with a fulminant course of classic KS with pulmonary involvement mimicking endobronchial tuberculosis.

Bronchoscopic evaluation in childhood pulmonary tuberculosis: risk factors of airway involvement and contribution to the bacteriologic diagnosis.

We aimed to describe the risk factors of airway involvement and to investigate the contribution of bronchoscopy in the bacteriologic diagnosis of tuberculosis. Airway involvement was more often present in patients with resistance to tuberculosis therapy than in the patients having bronchoscopy performed at initial presentation. Addition of bronchoalveolar lavage to the diagnostic workup increased the mycobacteriologic yield statistically.

Relapsing Polychondritis in a Nine-Year-Old Child Presenting with Subglottic Stenosis.

Relapsing polychondritis (RP) is a rare multisystem disorder characterized by inflammation and destruction of cartilaginous tissue. Atypical initial symptoms and signs may cause difficulties in the diagnosis of RP for pediatricians. In this article, we report a 9-year-old male who presented with stridor and hoarseness indicating upper respiratory tract involvement in the setting of chronic polyarthralgia. Flexible bronchoscopy revealed subglottic stenosis. Increasing awareness of early respiratory manifestations of RP will prevent life-threatening complications.

Upper airway abnormalities detected in children using flexible bronchoscopy.

OBJECTIVES: Rapid anatomical evaluation is essential to establish the severity of cases with upper respiratory obstruction and to define the degree of respiratory distress. Detailed airway endoscopy is required in most patients, not only for diagnosis, but also to treat the condition. In this study, as two of the largest paediatric pulmonology centres in Turkey, we reviewed the data of our bronchoscopy patients, and aimed to document the upper airway abnormalities that we detected during these procedures. PATIENTS AND METHODS: A retrospective analysis was made of the records of 1076 paediatric cases with pulmonary/airway disease who had undergone flexible bronchoscopy between 2007 and 2011. RESULTS: Upper airway malacia disorders were the most common (79.6%, n=259) bronchoscopic findings detected in the patients. The other most common pathologies were laryngeal edema (12.9%, n=42), external tracheal compression (12.3%, n=40), subglottic stenosis (4.0%, n=13), tracheal stenosis (2.8%, n=9), and vocal cord paralysis/irregularity (2.8%, n=9). The mean duration of symptoms was shortest in patients with vocal cord paralysis, and longest in patients with tracheal nodules (p<0.001). CONCLUSION: Paediatricians should keep in mind the possibility of malacia disorders and other congenital and acquired upper airway abnormalities in children with chronic respiratory problems. Diagnosis of underlying diseases, as soon as possible, permits the withdrawal of antibiotics or antiasthmatic drugs often used unnecessarily for long periods to treat these children.

Endobronchial findings of hydatid cyst disease: a report of five pediatric cases.

Hydatid disease is still an important public health problem throughout the world. Diagnosis of the disease is generally based on clinical and radiological findings. Evaluation of pulmonary disorders by flexible bronchoscopy (FOB) is a rapidly developing facility, but diagnostic and therapeutic FOB for pulmonary hydatid cysts is still controversial. This study examines the findings of endobronchial hydatid cyst disease in five pediatric patients from Turkey, and clinical experience about this subject is reviewed. All our patients presented with unusual symptoms of the disease, and for all of them, diagnosis had been delayed using current diagnostic methods. As a result of our experience, it can be reported that the endobronchial appearance of the hydatid cyst membrane is whitish-yellow, and it is difficult to differentiate it radiologically from some other common causes of endobronchial lesions in childhood, such as endobronchial tuberculosis, foreign body aspirations, mucous plaques, and granulation scars. The findings of these cases show that, hydatid cyst should also be kept in mind in differential diagnosis of endobronchial lesions. In the diagnosis of pulmonary hydatid cyst in children without typical clinical and radiological findings of the disease, FOB examination is a valuable diagnostic procedure.