Implementation of a high fidelity simulation based training program for physicians of children requiring long term invasive home ventilation: a study by ISPAT team.

Introduction: The number of children requiring long-term invasive home ventilation (LTIHV) has increased worldwide in recent decades. The training of physicians caring for these children is crucial since they are at high risk for complications and adverse events. This study aimed to assess the efficacy of a comprehensive high-fidelity simulation-based training program for physicians caring for children on LTIHV. Methods: A multimodal training program for tracheostomy and ventilator management was prepared by ISPAT (IStanbul PAediatric Tracheostomy) team. Participants were subjected to theoretical and practical pre-tests which evaluated their knowledge levels and skills for care, follow-up, and treatment of children on LTIHV. Following the theoretical education and hands-on training session with a simulation model, theoretical and practical post-tests were performed. Results: Forty-three physicians from 7 tertiary pediatric clinics in Istanbul were enrolled in the training program. Seventy percent of them had never received standardized training programs about patients on home ventilation previously. The total number of correct answers from the participants significantly improved after the theoretical training (p < 0.001). The number of participants who performed the steps correctly also significantly increased following the hands-on training session (p < 0.001). All of the 43 participants who responded rated the course overall as good or excellent. Conclusion: The knowledge and skills of clinicians caring for children on LTIHV can be enhanced through a comprehensive training program consisting of theoretical training combined with hands-on training in a simulation laboratory.

Is the Pediatric Sleep Questionnaire sensitive for sleep-disordered breathing in children with complex chronic disease?

PURPOSE: Sleep-disordered breathing (SDB) is a disease defined by breathing or breathing irregularities while asleep. The current study examines the association between results of polysomnography (PSG) and the Pediatric Sleep Questionnaire (PSQ), and the specificity and sensitivity of the PSQ for obstructive sleep apnea (OSA) in patients with chronic illnesses. METHODS: Demographic and clinical attributes, in addition to PSQ and PSG outcomes were examined retrospectively among patients who underwent polysomnography (PSG) at our facility between 2012 and 2021. RESULTS: Of 745 patients included in the study, 462 (62%) were male. The median age was 81 months (34-151 months). 117 of the patients (15/8%) had chronic lung disease, and 80 (10.7%) had cerebral palsy. The most common indications for PSG were symptoms of OSA (n = 426; 57.1%). According to obstructive apnea-hypopnea index (AHI), 361 patients (48.5%) had normal PSG. The median PSQ score was 0.40 (0.22-0.57). The sensitivity and specificity of the PSQ were 71.8% and 40.4%, respectively, for individuals aged 2 to 18 years. Among the disease subgroups, the cerebral palsy group had the highest sensitivity of PSQ (88.8%) for diagnosis of OSA. CONCLUSION: Questionnaires for evaluating SDB are not sensitive or specific for identification of OSA in children with chronic conditions, and PSG remains the best method.

Long-term outcomes of standardized training for caregivers of children with tracheostomies: The IStanbul PAediatric Tracheostomy (ISPAT) project.

BACKGROUND AND OBJECTIVES: Children with tracheostomies are at increased risk of tracheostomy-related complications and require extra care. Standardized training programs for caregivers can improve tracheostomy care and reduce complications. In this study, we compared caregiver knowledge and skill scores after a standardized theoretical and practical training program on tracheostomy care (IStanbul PAediatric Tracheostomy (ISPAT) project) immediately and 1 year post-training and evaluated how this training affected the children's clinical outcomes. MATERIALS AND METHODS: We included 32 caregivers (31 children) who had received standardized training a year ago and administered the same theoretical and practical tests 1 year after training completion. We recorded tracheostomy-related complications and the number and reasons for admission to the healthcare centers. All data just before the training and 1 year after training completion were compared. RESULTS: After 1 year of training completion, the median number of correct answers on the theoretical test increased to 16.5 from 12 at pretest (p < 0.001). Compared with pretest, at 1-year post-training practical skills assessment scores, including cannula exchange and aspiration, were significantly higher (both p < 0.001) and mucus plug, bleeding, and stoma infection reduced significantly (p = 0.002, 0.022, and 0.004, respectively). Hands-on-training scores were better than pretest but declined slightly at 1 year compared to testing immediately after training. Emergency admission decreased from 64.5% to 32.3% (p = 0.013). Hospitalization decreased from 61.3% to 35.5% (p = 0.039). CONCLUSION: Our findings indicate that caregiver training can lead to a persistent increase in knowledge and skill for as long as 1 year, as well as improvements in several measurable outcomes, although a slight decrease in scores warrants annual repetitions of the training program.

The ISPAT project: Implementation of a standardized training program for caregivers of children with tracheostomy.

BACKGROUND: Tracheostomy-related morbidity and mortality mainly occur due to decannulation, misplacement, or obstruction of the tube. A standardized training can improve the skills and confidence of the caregivers in tracheostomy care (TC). OBJECTIVE: Our primary aim was to evaluate the efficiency of standardized training program on the knowledge and skills (changing-suctioning the tracheostomy tube) of the participants regarding TC. MATERIALS AND METHODS: Sixty-five caregivers of children with tracheostomy were included. First, participants were evaluated with written test about TC and participated in the practical tests. Then, they were asked to participate in a standardized training session, including theoretical and practical parts. Baseline and postintervention assessments were compared through written and practical tests conducted on the same day. RESULTS: A significant improvement was observed in the written test score after the training. The median number of correct answers of the written test including 23 questions increased 26%, from 12 to 18 (p < .001). The median number of correct steps in tracheostomy tube change (from 9 to 16 correct steps out of 16 steps, 44% increase) and suctioning the tracheostomy tube (from 9 to 17 correct steps out of 18 steps, 44% increase) also improved significantly after the training (p < .001, for both). CONCLUSION: Theoretical courses and practical hands-on-training (HOT) courses are highly effective in improving the practices in TC. A standardized training program including HOT should be implemented before discharge from the hospital. Still there is a need to assess the impact of the program on tracheostomy-related complications, morbidity, and mortality in the long term.

Efficacy of standardized tracheostomy training with a simulation model for healthcare providers: A study by ISPAT team.

INTRODUCTION: Tracheostomy care in children may be challenging, due to lack of knowledge of healthcare providers (HCPs). The aim of this study was to determine the level of knowledge of HCP who follow patients with tracheostomy and to increase this level with theoretical training and training in a simulation laboratory. MATERIALS AND METHODS: ISPAT (IStanbul PAediatric Tracheostomy), a multidisciplinary team for tracheostomy care was established and a training program was prepared. Participants were subjected to theoretical and practical pretests which evaluated their knowledge levels and skills for care, follow-up, and treatment of a patient with tracheostomy. After the theoretical and practical training given to the participants with a simulation model, theoretical and practical posttests were applied. RESULTS: Fifty-one HCP from nine tertiary pediatric clinics in Istanbul were enrolled in the training program. Only six (11.8%) of them had received standardized training programs previously. Regarding the theoretical tests, seven of the 33 questions were indicated as essential. The knowledge level of the participants based on the essential questions significantly increased after the training (p < 0.05 for all of the essential questions). The total number of correct answers and correct answers of three subheadings also significantly increased after the practical training (p < 0.001 for all). Ninety-five percent of the participants assessed the course as good or excellent in general. CONCLUSION: Training in a simulation laboratory in combination with theoretical education can improve the knowledge and skills of the HCP enabling improved care of children with a tracheostomy.

Pediatric flexible bronchoscopy in the intensive care unit: A multicenter study.

INTRODUCTION: Flexible bronchoscopy (FB) is frequently used for assessment and treatment of patients with respiratory diseases. Our aim was to investigate the contribution of FB to diagnosis and therapy in children admitted to the intensive care units (ICU) and to evaluate the safety of FB in this vulnerable population. METHODS: Children less than 18 years of age who underwent FB in the five neonatal and pediatric ICUs in Istanbul between July 1st, 2015 and July 1st, 2020 were included to the study. Demographic and clinical data including bronchoscopy indications, findings, complications, and the contribution of bronchoscopy to the management were retrospectively reviewed. RESULTS: One hundred and ninety-six patients were included to the study. The median age was 5 months (range 0.3-205 months). The most common indication of FB was extubation failure (38.3%), followed by suspected airway disease. Bronchoscopic assessments revealed at least one abnormality in 90.8% patients. The most common findings were airway malacia and the presence of excessive airway secretions (47.4% and 35.7%, respectively). Positive contribution of FB was identified in 87.2% of the patients. FB had greater than 1 positive contribution in 138 patients and 80.6% of the patients received a new diagnosis. Medical therapy was modified after the procedure in 39.8% and surgical interventions were pursued in 40% of the patients. Therapeutic lavage was achieved in 18.9%. There were no major complications. CONCLUSION: Flexible bronchoscopy is a valuable diagnostic and therapeutic tool in neonatal and pediatric ICUs and is not associated with major complications.

The effect of nebulized antibiotics in children with tracheostomy.

INTRODUCTION: Children with tracheostomy have an increased risk of bacterial colonization and infection of the lower respiratory tracts. This study aimed to investigate the effects of nebulized antibiotics on the bacterial load, the need for oral antibiotics, the number of hospitalizations, and the length of stay in the intensive care unit in tracheotomised children with persistent colonization. METHODS: Children with tracheostomy and persistent bacterial colonization who were started on nebulized antibiotic therapy after a lower respiratory tract infection were included in the study. Nebulized gentamicin or colistin were used according to the results of the tracheal aspirate cultures. Demographic and clinic characteristics were recorded from one year prior until one year after initiation of nebulized antibiotic treatment. RESULTS: Nebulized antibiotic treatment was initiated in 22 patients. Nebulized gentamicin was administered to 14 patients (63.6%) and colistin to 8 patients (36.4%). The median duration of treatment was 3 months (range 2-5 months). Following nebulized antibiotic treatment, median number of hospitalizations decreased from 2 (range 1.0-3.5) to 1 (range 0.0-1.5) (p = 0.04). The median length of stay in the intensive care unit reduced significantly from 89.5 days (range 43.0-82.5) to 25 days (range 7.75-62.75) after starting nebulized antibiotics (p = 0.028). Following nebulized antibiotic treatment median bacterial colony count also decreased (from 105 CFU/ml (range 105-106) to 6 × 104 CFU/ml (range 104-105); p = 0.003). There were no significant side effects during nebulized antibiotic therapy. CONCLUSIONS: The use of nebulized antibiotics reduced the number of hospitalizations, length of stay in the intensive care unit, and bacterial load in tracheotomised children with persistent airway colonization without significant side effects. The use of nebulized antibiotics showed a statistically significant decrease in the measures studied. Use of nebulized antibiotics may help to decrease the health care burden of these children, families and health care system. Further studies are needed to determine the indications and optimal duration of long-term nebulized antibiotic treatment in children with tracheostomy.

Depression and anxiety in mothers of home ventilated children before and during COVID-19 pandemic.

BACKGROUND: Due to advances in technology, home ventilation in children has increased in recent years. The provision of proper care for a home-ventilated (HV) child can have a strong impact on the lifestyle of caregivers. The aim of this study was to evaluate the depression and anxiety levels of the mothers of HV children during the current COVID-19 pandemic and compare them to those of mothers of healthy peers. METHODS: This cross-sectional study was conducted on HV children (n = 21) and a control group of healthy peers (n = 32) by means of a questionnaire completed by the mothers of the children of both groups. Psychometric scales, such as the Beck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), and the State-Trait Anxiety Inventory-Trait (STAI-T), were used to assess participants. RESULTS: During the pandemic signs of depression were present in 8 (38.1%) of the case group and 8 (25%) of the healthy control group. Comparison of the BDI scores from before and during the pandemic showed no difference between mothers of the HV children (p = .09). Scores for BDI and STAI-T were higher in the case group than in the control group, whereas there was no significant difference in STAI-S scores. CONCLUSION: Depression and anxiety levels of mothers of HV children were found to be higher during the COVID-19 pandemic. Economic, medical, and social support resources are needed to reduce levels of depression and anxiety and help mothers of those children dependent on technology.

Non-invasive Ventilation for Children With Chronic Lung Disease.

Advances in medical care and supportive care options have contributed to the survival of children with complex disorders, including children with chronic lung disease. By delivering a positive pressure or a volume during the patient's inspiration, NIV is able to reverse nocturnal alveolar hypoventilation in patients who experience hypoventilation during sleep, such as patients with chronic lung disease. Bronchopulmonary dysplasia (BPD) is a common complication of prematurity, and despite significant advances in neonatal care over recent decades its incidence has not diminished. Most affected infants have mild disease and require a short period of oxygen supplementation or respiratory support. However, severely affected infants can become dependent on positive pressure support for a prolonged period. In case of established severe BPD, respiratory support with non-invasive or invasive positive pressure ventilation is required. Patients with cystic fibrosis (CF) and advanced lung disease develop hypoxaemia and hypercapnia during sleep and hypoventilation during sleep usually predates daytime hypercapnia. Hypoxaemia and hypercapnia indicates poor prognosis and prompts referral for lung transplantation. The prevention of respiratory failure during sleep in CF may prolong survival. Long-term oxygen therapy has not been shown to improve survival in people with CF. A Cochrane review on the use NIV in CF concluded that NIV in combination with oxygen therapy improves gas exchange during sleep to a greater extent than oxygen therapy alone in people with moderate to severe CF lung disease. Uncontrolled, non-randomized studies suggest survival benefit with NIV in addition to being an effective bridge to transplantation. Complications of NIV relate mainly to prolonged use of a face or nasal mask which can lead to skin trauma, and neurodevelopmental delay by acting as a physical barrier to social interaction. Another associated risk is pulmonary aspiration caused by vomiting whilst wearing a face mask. Adherence to NIV is one of the major barriers to treatment in children. This article will review the current evidence for indications, adverse effects and long term follow up including adherence to NIV in children with chronic lung disease.

Comparison of conventional chest physiotherapy and oscillatory positive expiratory pressure therapy in primary ciliary dyskinesia.

BACKGROUND: Chest physiotherapy (CP) is a recommended treatment modality in primary ciliary dyskinesia (PCD). OBJECTIVE: Primary aim was to compare the efficacy and safety of the conventional chest physiotherapy (CCP) and oscillatory positive expiratory pressure therapy (OPEPT). Secondary aims were to compare the exacerbation rate, time until the first exacerbation, patient compliance and comfort between the two CP methods. METHODS: This is a 6 month randomized, controlled crossover trial. Patients >6 years of age with PCD were randomized into two groups, first group was assigned to OPEPT (Acapella®) for 3 months while second group was assigned to CCP. Groups were crossed over to the other modality after a 15-day washout period. Pulmonary function tests (PFTs) and compliance were monitored by monthly clinic visits. RESULTS: There was a significant increase in FEV1 , FEF25-75 , and PEF values (p = .018, p = .020, and p = .016, respectively) in the OPEPT group and in FVC values (p = .007) in CCP group compared to baseline. However PFT increase at 3rd month was not superior to each other with both physiotherapy methods. Median acute pulmonary exacerbation rate and time period until the first exacerbation were similar in both groups (p = .821, p = .092, respectively). Comfort and effectiveness of OPEPT was higher than CCP according to patients (p = .029 and p = .042, respectively). There were no adverse effects with either therapy. CONCLUSIONS: OPEPT was as effective as CCP in PCD patients. OPEPT was more comfortable and effective than CCP according to patients. OPEPT might be an efficient alternative method for airway cleareance in PCD patients.

An Adolescent Presented With Hemoptysis: Pulmonary Artery Aneurysm in Pediatric Behçet's Disease.

Behçet's disease (BD) is a rare autoimmune and chronic inflammatory vasculitis, characterized by relapsing episodes of oral aphthous and genital ulcers, skin lesions, ocular lesions and vascular involvement. Pulmonary artery involvement is rare in BD but it carries a high mortality risk. In this article, we report a 15-year-old male patient presented with a two-month history of hemoptysis, cough, fewer and weight loss. On physical examination, auscultation revealed decreased breath sounds at left lung base. Bronchoscopy showed narrowed left lower lobe bronchus due to the external compression. Computed tomography angiography revealed multiple bilateral pulmonary artery aneurysms. Pathergy test was positive and he was diagnosed with BD. BD should be considered in the differential diagnosis of childhood hemoptysis.

Health-related quality of life in patients with bronchiolitis obliterans.

INTRODUCTION: Bronchiolitis obliterans (BO) is mainly caused by infections and hematopoietic stem cell transplantation (HSCT). This study aimed to investigate the health-related quality of life (HRQOL) of children with BO compared to the healthy children and also to assess the HRQOL according to the etiology. METHODS: Postinfectious (group 1) and post-HSCT BO (group 2) patients and healthy children were included in the study. HRQOL was assessed by the Short Form-36 (SF-36) and St George's Respiratory Questionnaire (SGRQ). Correlations between demographic and clinical characteristics, pulmonary function tests, high-resolution chest tomography scores, and HRQOL were assessed. RESULTS: Thirty-seven postinfectious and post-HSCT BO patients and 34 healthy children were included in the study. Mean age was 13.8 ± 0.7 years. Mean forced vital capacity and forced expiratory volume1 were 60.7 ± 2.7% predicted, and 49.8 ± 3.1% predicted, respectively. The SF-36 scores were lower in BO patients compared to healthy children (P < .01). Patients with better lung functions had higher SF-36 scores, but lower SGRQ. The number of inhaled therapies, acute exacerbations, hospitalizations were inversely correlated with SF-36. A positive correlation was found between these parameters and total SGRQ scores (r = .507, P = .02; r = .409, P = .12; r = .326, P = .049, respectively). SF-36 scores were better in group 1 for subscales of physical role functioning and social role functioning compared to group 2. (P = .01, P = .01, respectively). CONCLUSION: The HRQOL of patients with BO measured by SF-36 was low compared to healthy children. SF-36 scores were more affected in post-HSCT BO patients. HRQOL of children with chronic lung disease should be taken into consideration in the management of these patients.

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.

Novel mutations and deletions in cystic fibrosis in a tertiary cystic fibrosis center in Istanbul.

BACKGROUND: Cystic fibrosis (CF) genotyping has garnered increased attention since the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 led to the identification of over 1700 mutations on chromosome 7. Yet, little is known about the genetic profile of CF patients in Turkey. This study sought to determine the mutation distribution among CF patients seeking care at Marmara University. METHODS: Two hundred fifty previously diagnosed CF patients were included in the study. CFTR gene exons 1 to 27 were amplified by a polymerase chain reaction and whole DNA sequencing was performed. Duplications and deletions were investigated by the multiplex ligation-dependent probe amplification (MLPA) technique in patients with one or two unidentified mutations in sequence analysis. RESULTS: CFTR mutation analysis revealed 80 mutations and five large deletions were present in our study population. The five most common mutations were (delta) F508 (c.1521-1523delCTT) (28.4%), 1677delTA (c.1545-1546delTA) (6.4%), 2789 + 5G- > A (c.2657 + 5G > A) (5.8%), N1303K (c.3909C > G) (2.4%), and c.2183AA- > G (c.2051-2052delAAinsG) (4.0%). Large deletions were found in 16 patients. Four novel mutations and two novel deletions were detected in this study. CONCLUSIONS: We have identified four novel mutations and two novel deletions using next-generation DNA sequencing and the MLPA technique and obtained an overall mutation detection rate of 91.4%. Detection of novel variants in CF patients will assist in genetic counseling and in determining appropriate patients for new therapies.

Adolescente con fibroelastosis pleuroparenquimatosa idiopatica: A propòsito de un caso / An adolescent with idiopathic pleuroparenchymal fibroelastosis: Case report

La fibroelastosis pleuroparenquimatosa idiopàtica (FEPPI) es un trastorno raro incluido recientemente en las neumonías intersticiales idiopáticas según la actualización de la clasificación de la Sociedad Torácica Estadounidense (American Thoracic Society) y la Sociedad Respiratoria Europea (European Respiratory Society). La FEPPI se caracteriza por fibrosis parenquimatosa pleural y subpleural que produce reducción del volumen, sobre todo en los lóbulos pulmonares superiores. La edad al momento de la aparición varía, aunque la FEPPI ocurre principalmente entre los 30 y los 50 años de edad. En este artículo, presentamos el caso de un paciente de 16 años con antecedentes de disnea de esfuerzo, tos seca y pérdida de peso en los últimos dos años. Durante el examen físico, con la auscultación se detectó disminución de los ruidos respiratorios en los lóbulos superiores. En la radiografía de tórax se observó engrosamiento pleural apical y volumen reducido. En la tomografia computarizada (TC) del tórax se observaron densidades en vidrio esmerilado y bronquiectasia tubular predominantemente en ambos lóbulos superiores, con engrosamiento septal interlobulillar de la pleura y adenopatía mediastínica. Se realizó una biopsia pulmonar por toracoscopia y en el examen histológico se observaron fibrosis subpleural y tinción de las fibras elásticas que demostraba depósito de fibras elásticas en el área subpleural y el parénquima pulmonar adyacente, lo que sugería FEPPI. Hasta donde sabemos, este es el primer caso durante la niñez. Por lo tanto, los pediatras deben estar atentos a esta enfermedad para realizar un diagnóstico y tratamiento adecuados.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss. On physical examination, auscultation revealed diminished breath sounds on the upper lobes.Chest radiograph showed apical pleural thickening and volume loss. Computerized tomographic scan (CT) of chest revealed ground glass densities and tubular bronchiectasis predominantly in upper lobes bilaterally, with interlobular septal thickening of the pleura and enlarged mediastinal lymph nodes. Thoracoscopic lung biopsy was performed and histological evaluation showed subpleural fibrosis and elastic staining demonstrated fragmented elastic fiber deposition in the subpleural area and adjacent pulmonary parenchyma suggesting IPPFE. To our knowledge this is the first case in childhood. Therefore, pediatricians should be aware of this disease for the diagnosis and appropriate management.

An adolescent with idiopathic pleuroparenchymal fibroelastosis. Case report. / Adolescente con fibroelastosis pleuroparenquimatosa idiopatica. A propòsito de un caso.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss. On physical examination, auscultation revealed diminished breath sounds on the upper lobes. Chest radiograph showed apical pleural thickening and volume loss. Computerized tomographic scan (CT) of chest revealed ground glass densities and tubular bronchiectasis predominantly in upper lobes bilaterally, with interlobular septal thickening of the pleura and enlarged mediastinal lymph nodes. Thoracoscopic lung biopsy was performed and histological evaluation showed subpleural fibrosis and elastic staining demonstrated fragmented elastic fiber deposition in the subpleural area and adjacent pulmonary parenchyma suggesting IPPFE. To our knowledge this is the first case in childhood. Therefore, pediatricians should be aware of this disease for the diagnosis and appropriate management.

La fibroelastosis pleuroparenquimatosa idiopàtica (FEPPI) es un trastorno raro incluido recientemente en las neumonías intersticiales idiopáticas según la actualización de la clasificación de la Sociedad Torácica Estadounidense (American Thoracic Society) y la Sociedad Respiratoria Europea (European Respiratory Society). La FEPPI se caracteriza por fibrosis parenquimatosa pleural y subpleural que produce reducción del volumen, sobre todo en los lóbulos pulmonares superiores. La edad al momento de la aparición varía, aunque la FEPPI ocurre principalmente entre los 30 y los 50 años de edad. En este artículo, presentamos el caso de un paciente de 16 años con antecedentes de disnea de esfuerzo, tos seca y pérdida de peso en los últimos dos años. Durante el examen físico, con la auscultación se detectó disminución de los ruidos respiratorios en los lóbulos superiores. En la radiografía de tórax se observó engrosamiento pleural apical y volumen reducido. En la tomografia computarizada (TC) del tórax se observaron densidades en vidrio esmerilado y bronquiectasia tubular predominantemente en ambos lóbulos superiores, con engrosamiento septal interlobulillar de la pleura y adenopatía mediastínica. Se realizó una biopsia pulmonar por toracoscopia y en el examen histológico se observaron fibrosis subpleural y tinción de las fibras elásticas que demostraba depósito de fibras elásticas en el área subpleural y el parénquima pulmonar adyacente, lo que sugería FEPPI. Hasta donde sabemos, este es el primer caso durante la niñez. Por lo tanto, los pediatras deben estar atentos a esta enfermedad para realizar un diagnóstico y tratamiento adecuados.