Background: Mediastinal tumors in children comprise a heterogeneous group of lesions that have a range of embryonic origins. They may present as benign cysts, as well as malignant lesions. Objective: To describe the diagnostic procedures, the treatments and outcomes of a group of children and adolescents with mediastinal tumors. Method: A retrospective analysis of twenty children and adolescents with mediastinal tumors who were treated at the Hospital de Clínicas de Porto Alegre from July, 1996 to July, 2002. All patients were submitted to some kind of surgical procedure: diagnostic, therapeutic, or both. Results: Twelve boys and eight girls were studied. Mean age at diagnosis was 6 years and 8 months (ranging from 3 months to 16 years). Fourteen tumors (70%) were located at the anterior, and six (30%) at the posterior mediastinum. Hodgkin and non-Hodgkin lymphomas were the most common tumors found in anterior mediastinum, whereas neuroblastoma was the most common among posterior malignancies. The most used surgical procedure for anterior tumors was Chamberlain anterior thoracotomy; posterolateral thoracotomy was usually performed for posterior tumors. Six patients died during the follow-up period but none of the deaths was considered related to the surgical procedure. Conclusion: Mediastinal tumors in children and adolescents represent an important cause of morbidity/mortality. The most common tumors at the anterior mediastinum were lymphomas, whereas at the posterior mediastinum the most common were neurogenic tumors. Surgery is an important step for the diagnosis and treatment of such lesions.

Keywords: Tumors. Mediastinum. Child. Lymphoma/surgery.

Sclerosing mediastinitis is a rare disorder characterized by an extensive fibrotic reaction involving the mediastinum. Due to the compression or invasion of mediastinal structures, the disorder mimics neoplasia. We present three cases of superior vena cava syndrome in which sclerosing mediastinitis was confirmed. The pathophysiological process is related to enlarged mediastinal lymph nodes, fibroblast proliferation and collagen deposition. The main causes of sclerosing mediastinitis are histoplasmosis and tuberculosis, both of which are prevalent in Brazil. It is difficult to make an accurate histopathological diagnosis using minimally invasive methods, and there is no effective treatment for this condition. In order to make a definitive diagnosis and resolve the aerodigestive tract obstruction, exploratory surgery is indicated.

Keywords: Mediastinitis/physiopathology; Mediastinum; Pulmonary fibrosis; Mediastinal neoplasms; Diagnosis, differential; Case reports [Publication type].

Objectives: Thoracoscopic management of mediastinal tumors is still subject to analysis. Seventy-three patients were submitted to thoracoscopy for the treatment of mediastinal masses and were analyzed retrospectively, in order to evaluate the effectiveness and complications of the procedure. Methods: Between 1983 and 1999, 21 conventional thoracoscopies and 52 video-assisted thoracic surgeries were performed (33 for diagnostic purposes and 40 for therapy). Patient ages ranged from two to 81 years (mean 43.8) with a slight predominance of females over males (41 versus 32). All underwent general anesthesia using simple (22) or double lumen (51) intubation. Results: The histological type of tumor was identified in all patients. For therapeutic purposes, conversion to thoracotomy was necessary in nine patients. The reasons were tumor size and invasion of nearby structures, difficulty to continue dissection, to perform an upper lobectomy, and to suture the iatrogenic diaphragm lesion. Four patients died during the first 30 postoperative days as a consequence of their primary pathology. Conclusions: Thoracoscopy was confirmed to be an effective diagnostic and therapeutic alternative for the treatment of mediastinal disorders.

Keywords: Thoracoscopy, thoracic surgery, mediastinal neoplasms, mediastinal cysts.

The presence of mediastinal lymph node metastasis in lung cancer has a great influence on treatment selection and prognosis, and the diagnosis of lymphatic metastasis must be accurate. CT chest scans and mediastinoscopy are the diagnostic tools available to detect mediastinal lymph node metastases and to establish preoperative staging. Lymph node size evaluated by CT scans is not an acceptable criterion in the patients studied here. The aim of this study was to find out a representative pattern of size to indicate the possibility of neoplastic invasion in the population studied. Fifty consecutive patients were operated on and were submitted to pulmonary resection and mediastinal dissection to treat lung cancer. Seventy-four percent of the lymph nodes with transverse section of more than 3 cm had no neoplastic invasion. CT scans and mediastinoscopy have similar sensitivity (close to 65%), but mediastinoscopy has higher specificity (100%). This means that the authors must have histologic confirmation of any suspected lymph node to achieve a correct staging.

Keywords: Carcinoma, non-small cell. Lung neoplasias. Lymph nodes. Lymphatic metastasis. Mediatinoscopy. Neoplasm, staging. CT scanning.

A 27-year-old male patient presented with respiratory symptoms, bilateral enlargement of the cervical lymph nodes and enlarged liver. In the imaging studies, bilateral enlargement of the hilar nodes was observed, together with pulmonary infiltrate. The patient was submitted to lung and liver biopsies, which revealed noncaseating granulomas. The clinical, radiological and histopathological findings were consistent with sarcoidosis and lymph node amyloidosis. The combination of sarcoidosis and amyloidosis has rarely been reported.

Keywords: Amyloidosis; Sarcoidosis; Lymph nodes.

The growth of children with asthma has attracted great interest because it is a chronic respiratory illness whose prevalence in childhood has increased during the last decades. The relationship between asthma and growth has been studied for a long time, but the great variability of diagnosis, therapy, and different methods of investigation make it difficult to disentangle the factors that are responsible for the growth retardation detected by some authors. As a result of different observations, there remain many doubts about the impact of asthma on child and adolescent growth. The aim of this paper is to provide a general view on the issue.

Objective: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive diagnostic test with a high diagnostic yield for suspicious central pulmonary lesions and for mediastinal lymph node staging. The main objective of this study was to describe the diagnostic yield of EBUS-TBNA for mediastinal lymph node staging in patients with suspected lung cancer. Methods: Prospective study of patients undergoing EBUS-TBNA for diagnosis. Patients ≥ 18 years of age were recruited between July of 2010 and August of 2013. We recorded demographic variables, radiological characteristics provided by axial CT of the chest, location of the lesion in the mediastinum as per the International Association for the Study of Lung Cancer classification, and definitive diagnostic result (EBUS with a diagnostic biopsy or a definitive diagnostic method). Results: Our analysis included 354 biopsies, from 145 patients. Of those 145 patients, 54.48% were male. The mean age was 63.75 years. The mean lymph node size was 15.03 mm, and 90 lymph nodes were smaller than 10.0 mm. The EBUS-TBNA method showed a sensitivity of 91.17%, a specificity of 100.0%, and a negative predictive value of 92.9%. The most common histological diagnosis was adenocarcinoma. Conclusions: EBUS-TBNA is a diagnostic tool that yields satisfactory results in the staging of neoplastic mediastinal lesions.

Keywords: Lung neoplasms; Bronchoscopy; Endosonography; Neoplasm staging.

Objective: Evaluation of enlarged mediastinal lymph nodes is crucial for patient management. Malignant lymphoma and sarcoidosis are often difficult to differentiate. Our objective was to determine the diagnostic accuracy of MRI for differentiating between sarcoidosis and malignant lymphoma. Methods: This was a retrospective study involving 47 patients who underwent chest MRI and were diagnosed with one of the diseases between 2017 and 2019. T1, T2, and diffusion-weighted signal intensity were measured. Apparent diffusion coefficients (ADCs) and T2 ratios were calculated. The diagnostic performance of MRI was determined by ROC analysis. Results: Mean T2 ratio was significantly lower in the sarcoidosis group than in the lymphoma group (p = 0.009). The T2-ratio cutoff value that best differentiated between lymphoma-related and sarcoidosis-related enlarged lymph nodes was 7.1, with a sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of 58.3%, 95.6%, 76.5%, 93.3%, and 68.7%, respectively. The mean ADC was significantly lower in the lymphoma group than in the sarcoidosis group (p = 0.002). The ADC cutoff value that best differentiated between lymphoma-related and sarcoidosis-related enlarged lymph nodes was 1.205, with a sensitivity, specificity, positive predictive value, negative predictive value and accuracy of 87.5%, 82.6%, 85.1%, 84.0% and 86.3%, respectively. No significant differences were found between the two groups regarding T1 signal intensity, T2 signal intensity, and lymph node diameter. Conclusions: MRI parameters such as ADC, diffusion, and T2 ratio can be useful in the differentiation between sarcoidosis and lymphoma in the evaluation of enlarged lymph nodes.

Keywords: Diffusion magnetic resonance imaging; Lymphoma; Lymph nodes; Sarcoidosis.

Objective: Although EBUS-TBNA combined with EUS-FNA or EUS-B-FNA stands as the primary approach for mediastinal staging in lung cancer, guidelines recommend mediastinoscopy confirmation if a lymph node identified on chest CT or showing increased PET scan uptake yields negativity on these techniques. This study aimed to assess the staging precision of EBUS/EUS. Methods: We conducted a retrospective study comparing the clinical staging of non-small cell lung cancer patients undergoing EBUS/EUS with their post-surgery pathological staging. We analyzed the influence of histology, location, tumor size, and the time lapse between EBUS and surgery. Patients with N0/N1 staging on EBUS/EUS, undergoing surgery, and with at least one station approached in both procedures were selected. Post-surgery, patients were categorized into N0/N1 and N2 groups. Results: Among the included patients (n = 47), pathological upstaging to N2 occurred in 6 (12.8%). Of these, 4 (66.7%) had a single N2 station, and 2 (33.3%) had multiple N2 stations. The adenopathy most frequently associated with upstaging was station 7. None of the analyzed variables demonstrated a statistically significant difference in the occurrence of upstaging. PET scan indicated increased uptake in only one of these adenopathies, and only one was visualized on chest CT. Conclusions: Upstaging proved independent of the studied variables, and only 2 patients with negative EBUS/EUS would warrant referral for mediastinoscopy. Exploring other noninvasive methods with even greater sensitivity for detecting micrometastatic lymph node disease is crucial.

Keywords: Carcinoma, non-small cell lung; Neoplasm staging; Endoscopic ultrasound-guided fine needle aspiration.

The saccharin test measures nasal mucociliary clearance. It is simple to perform, inexpensive, reproducible and is a screening test for the diagnosis of primary ciliary dyskinesia. Study objective - To establish the normal range of the ST in a group of presumed normal Brazilian children, and compare the results with those from the literature. Setting - The study was conducted in 2 schools in São Paulo, Brazil. Participants - Two hundred and thirty eight schoolchildren, 134 boys and 104 girls, age range 10.8-16.8 years, were studied. Information on respiratory diseases and smoking habits of parents were obtained from a questionnaire completed by the parents. Results - The questionnaires were analyzed and the children were then divided into 5 groups.The mean ST value in the group of normal children was 7.5 ± 2.8 minutes, comparable to the literature. The mean ST value in children with parental smoking, upper respiratory tract infection (URTI) within the preceding week, or allergy and URTI was not significantly different from the normal group. However, there was a significant difference between the normal group and those with allergy. As the standard deviation was greater in the groups with URTI, the population was reorganized into 2 groups, the criteria being the presence or not of URTI within the preceding week. Again, the mean ST values were not significantly different, but the variability was significantly different in the group with URTI. In 99% of the children studied the test took less than 30 minutes. Conclusions - Saccharin test values over 30 minutes should be considered abnormal and retested. We recommend that the test should not be performed during or within one month following an URTI.

Keywords: Saccharin test. Nasal mucociliary clearance.

Interstitial lung diseases (ILDs) in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences and research in adults. However, pediatric pulmonologists have observed that the clinical spectrum is broader in children than in adults, and that many of these disorders have different courses and treatment responses. In addition, probably due to the various stages of lung development and maturation, new clinical forms have been described, particularly in infants. This has broadened the classification of ILDs in this age bracket. The understanding that neither the usual definition nor the standard classification of these disorders entirely apply to children has prompted multicenter studies designed to increase knowledge of these disorders, as well as to standardize diagnostic and therapeutic strategies. We have reviewed the conceptualization of ILDs in children, taking into consideration the particularities of this group of patients when using the criteria for the classification of these diseases in adults. We have also made a historical review of several multicenter studies in order to further understanding of the problem. We have emphasized the differences in the clinical presentation, in an attempt to highlight knowledge of newly described entities in young children. We underscore the need to standardize management of laboratory and radiological routines, as well as of lung biopsy processing, taking such knowledge into account. It is important to bear in mind that, among the recently described disorders, genetic surfactant dysfunction, which is often classified as an idiopathic disease in adults, should be included in the differential diagnosis of ILDs.

Keywords: Lung diseases, interstitial; Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/therapy; Child.

Objective: To determine the rate at which satisfactory spirometry results are obtained (spirometry success rate) in preschool children. Methods: We analyzed the spirometry results of children ≤ 6 years of age. All tests were conducted between June of 2009 and February of 2010 in the Pulmonary Function Laboratory of the Hospital Infantil Jeser Amarante Faria, located in the city of Joinville, Brazil. The spirometry program employed features an animated incentive (soap bubbles). The procedures were performed by a pediatric pulmonologist, in accordance with the reproducibility and acceptability criteria recommended by the American Thoracic Society. We attempted to achieve an expiratory time of at least 1 s. The following parameters were measured: FVC, FEV0.5, FEV1, and the FEV1/FVC ratio. Results: Our sample comprised 74 children. The spirometry success rate was 82%. Although the performance improved with age, the difference between younger and older children was not significant (p > 0.05). An average of 6.6 attempts/test were needed in order to achieve acceptable, reproducible curves. All 61 successful tests produced satisfactory FEV0.5 and FEV1 values. By calculating Z scores, we found that 21.6% of the children presented with an obstructive pattern. Conclusions: In our sample, the spirometry success rate was high, showing that spirometry is a valid method for assessing pulmonary function in preschool children. The high success rate in our sample might be attributable to the use of an incentive and to the fact that the tests were performed by professionals specializing in pediatrics.

Keywords: Spirometry; Feasibility studies; Respiratory function tests.

Objective: To generate reference values for spirometry in Brazilian children 3-12 years of age and to compare those values with the values employed in the equations currently in use in Brazil. Methods: This study involved healthy children, 3-12 years of age, recruited from 14 centers (primary data) and spirometry results from children with the same characteristics in six databases (secondary data). Reference equations by quantile regressions were generated after log transformation of the spirometric and anthropometric data. Skin color was classified as self-reported by the participants. To determine the suitability of the results obtained, they were compared with those predicted by the equations currently in use in Brazil. Results: We included 1,990 individuals from a total of 21 primary and secondary data sources. Of those, 1,059 (53%) were female. Equations for FEV1, FVC, the FEV1/FVC ratio, FEF between 25% and 75% of the FVC (FEF25-75%) and the FEF25-75%/FVC ratio were generated for white-, black-, and brown-skinned children. The logarithms for height and age, together with skin color, were the best predictors of FEV1 and FVC. The reference values obtained were significantly higher than those employed in the equations currently in use in Brazil, for predicted values, as well as for the lower limit of normality, particularly in children with self-reported black or brown skin. Conclusions: New spirometric equations were generated for Brazilian children 3-12 years of age, in the three skin-color categories defined. The equations currently in use in Brazil seem to underestimate the lung function of Brazilian children 3-12 years of age and should be replaced by the equations proposed in this study.

Keywords: Spirometry; Reference values; Child; Child, preschool; Respiratory function tests.

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a new method for the diagnosis and staging of mediastinal lymph nodes. The objective of this study was to evaluate the preliminary results obtained with EBUS-TBNA in the diagnosis of lesions and mediastinal lymph node staging. Methods: We evaluated patients with tumors or mediastinal adenopathy, diagnosed with or suspected of having lung cancer. The procedures were performed with the patients under sedation or under general anesthesia. Material was collected by EBUS-TBNA, after which it was prepared on slides, fixed in either absolute alcohol (for cytology) or formalin (for cell-block analysis). Results: We included 50 patients (30 males). The mean age was 58.3 ± 13.5 years. We performed 201 biopsies of 81 lymph nodes or mediastinal masses (mean of 2.5 punctures/biopsy). The quantity of material was considered sufficient for cytology in 37 patients (74%), 21 (57%) of whom were thus diagnosed with malignancy. Of the remaining 16 patients, 1 was diagnosed with tuberculosis, 6 entered clinical follow-up, and 9  underwent further investigation (2 diagnosed with neoplasm-false-negative results). The yield was higher when the procedure was performed for diagnostic purposes, as well as being higher in patients with lesions in multiple stations and in biopsies involving the subcarinal lymph node station. One patient had endobronchial bleeding, which was resolved with local measures. There were no deaths among the patients evaluated. Conclusions: This preliminary experience shows that EBUS-TBNA is a safe procedure. Our diagnostic yield, although lower than that reported in the literature, was consistent with the learning curve for the method.

Keywords: Ultrasonography, interventional; Biopsy, fine-needle; Neoplasm staging; Lung neoplasms; Bronchoscopy.