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Objective: This study was aimed to investigate the complications arising during follow-up and the reasons for hospitalization in pediatric patients who were operated on for esophageal atresia (EA) and tracheoesophageal fistula (TEF). Materials and Methods: Between 2007 and 2019, all patients operated for EA and TEF were evaluated in terms of age, gender, age at diagnosis, post-op, and complications in follow-up. Results: In the study, 28 of 50 patients were operated on for EA and TEF, 14 for isolated EA, and 8 for isolated TEF. The mean age of the patients was 4.9 ± 4.4 years, and 18 (36%) of them were female. The median age of diagnosis was 1 (IQR: 1-3) day and the mean follow-up duration was 4.6 ± 4.1 years. During follow-up, 84% of patients had recurrent pneumonia, 60% gastroesophageal reflux (GER), 34% growth retardation, 30% restrictive lung disease, and 18% scoliosis. Postoperative pneumonia, hospitalization, development of stricture and growth retardation were more frequent in patients with GER (P < .05). Patients with scoliosis had more frequent pneumonia and hospitalization rates (P < .05). Conclusion: Hospital admissions of the patients with EA and TEF were higher due to GER, recurrent pneumonia, restrictive lung disease, and scoliosis in the follow-up. Pneumonia, hospitalization, stricture in the esophagus, and growth retardation were observed more frequently in patients with GER. Patients should be followed up by the pediatric pulmonology, gastroenterology, and orthopedic departments.

Objective Patients with primary ciliary dyskinesia (PCD) may present with different clinical findings at different ages, and age at diagnosis may differ. We aimed to review clinical factors that affected age at diagnosis of patients with PCD. Study Design All 70 patients with PCD who were followed in our pediatric pulmonology department were included. Demographic features, clinical findings, PrImary CiliAry DyskinesiA Rule (PICADAR) scores and pulmonary function tests of patients were recorded and clinical factors that affected age at diagnosis were evaluated. Results The mean age at diagnosis was 8.3 +/- 4.6 years. Most of patients (95.7%) had a persistent wet cough. The mean PICADAR score was 6.5 +/- 3.2, and there was a negative correlation between PICADAR and age at diagnosis (r = -0.271, p = .023). The mean ages at diagnosis of patients with situs abnormality and recurrent wheezing were earlier than in patients without situs abnormality and recurrent wheezing (6.7 +/- 4.3 and 6.8 +/- 4.3, p = .002 vs. 9.8 +/- 4.3 and 9.0 +/- 4.6 years, p = .040, respectively). The mean age at diagnosis of patients with bronchiectasis was later than in patients without bronchiectasis (10.8 +/- 3.9 and 6.9 +/- 4.4 years, p = .001). Other clinical features were not statistically significant according to age at diagnosis (p > .05). There was no statistically significant relation between age at diagnosis and sex, sibling or relative with PCD and parental consanguinity (p > .05). Conclusion Although most patients diagnosed with PCD had symptoms, the diagnosis may be delayed. High PICADAR score is a useful guide to evaluate PCD. Situs abnormality and recurrent wheezing could be clues for early diagnosis of PCD. Early diagnosis of PCD may prevent bronchiectasis.

© 2022 John Wiley & Sons Ltd.Aims and Objectives: The aim of this study was to evaluate the clinical features of children with tracheostomy and the predictors of psychological status of their primary caregivers in order to determine the associations between the children's clinical course with their caregivers' psychological status. Background: The caregivers of children with tracheostomy are responsible for providing basic tracheostomy care at home. All these responsibilities may be associated with significant changes in family members' lifestyles, daily routines and family dynamics. Design: This study is a cross sectional study. Methods: Data of the family's socioeconomic status and clinical status of children with tracheostomy were noted in four paediatric pulmonology centers. The Beck Depression Inventory, Maslach Burnout Inventory, Zarit Caregiver Burden Scale, and Rosenberg Self-esteem Scale were used for psychological measurements of the caregivers. The STROBE checklist was used for this study. Results: Eighty-five children and their primary caregivers were enrolled in the study. The children's median age was 4.1 years. Thirty-eight of them were dependent on home ventilators. Twenty-one had bacterial colonisation. All children's primary caregivers were their mothers. Beck Depression Inventory scores of mothers of children with colonisation were higher. Number of hospitalizations in previous 6 months was related to mothers' emotional exhaustion and depersonalization scores. Duration of children's hospitalizations in previous 6 months was positively correlated to mothers' emotional exhaustion, depersonalization, and Beck Depression Inventory scores. Conclusions: Mothers of children with tracheostomy may experience psychological conditions, such as high levels of depression, burnout, burden and low levels of self-esteem. Frequent and long-term hospitalizations of children correlated with mother's depression and burnout. Therefore, primary caregivers should be evaluated and supported psychologically. Relevance to Clinical Practice: Preventing mothers of children with tracheostomy from experiencing psychological conditions such as depression, burnout, burden and low self-esteem can also increase the quality of care for children.

Thoracic air leak syndromes (TALS) are very rare among the noninfectious pulmonary complications (PCs). They can either be idiopathic or have several risk factors such as allogeneic hematopoietic stem cell transplantation (allo-HSCT), graft versus host disease and rarely pulmonary aspergillosis. We present a 14-year-old girl with hypoplastic myelodysplastic syndrome who developed graft versus host disease on day 60, TALS on day 150, bronchiolitis obliterans syndrome on day 300, pulmonary aspergillosis on day 400 and COVID-19 pneumonia on day 575 after allo-HSCT. This is the first report of a child who developed these subsequent PCs after allo-HSCT. Therefore, the manifestations of these unfamiliar PCs like TALS and COVID-19 pneumonia, and concomitant pulmonary aspergillosis with management options are discussed.

AIM: We aimed to investigate sleep disturbances in children with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) and typically developing (TD) children during the COVID-19 pandemic. METHODS: Primary care givers of children with CF and PCD aged 3-16 years were asked to enrol in the study. Primary care givers of TD children were included as control group. The Sleep Disturbance Scale for Children (SDSC) was used, and questions related to sleep habits during the pandemic were asked. Results of the three groups were compared. RESULTS: Primary care givers of 33 children with CF, 16 children with PCD and 66 TD children were included in the study. There were no differences in terms of age and gender between the three groups. Changes in sleep patterns during the pandemic were more common among TD children and their families, with 75% of the children and 80% of their families sleeping later than before. The sleep initiation and maintenance disorder scores were higher in TD children (P = 0.001), whereas the sleep breathing disorder scores were higher in children with PCD (P = 0.001), and the sleep hyperhidrosis scores were higher in children with CF and PCD (P = 0.011). No relationships were found between sleep parameters and clinical findings of children with lung disease. CONCLUSIONS: Children's sleep habits have changed during the pandemic. Children with chronic lung diseases and even TD children may experience sleep disturbances during this period.

We aimed to evaluate cutoff values of immunoreactive trypsinogen (IRT)/IRT and determine relationship between IRT values and clinical characteristics of children with cystic fibrosis (CF). This study is cross-sectional study. Data of children with positive newborn screening (NBS) between 2015 and 2021 were evaluated in three pediatric pulmonology centers. Age at admission, sex, gestational age, presence of history of meconium ileus, parental consanguinity, sibling with CF, and doll-like face appearance, first and second IRT values, sweat chloride test, fecal elastase, fecal fat, biochemistry results, and age at CF diagnosis were recorded. Sensitivity and specificity of IRT cutoff values were evaluated. Of 815 children with positive NBS, 58 (7.1%) children were diagnosed with CF. Median values of first and second IRT were 157.2 (103.7-247.6) and 113.0 (84.0-201.5) μg/L. IRT values used in current protocol, sensitivity was determined as 96.6%, specificity as 17.2% for first IRT, and 96.6% sensitivity, 20.5% specificity for second IRT. Positive predictive value (PPV) was determined as 7.1%. When cutoff value for first IRT was estimated as 116.7 μg/L, sensitivity was 69.0% and specificity was 69.6%, and when cutoff value was set to 88.7 μg/L for second IRT, sensitivity was 69.0% and specificity was 69.0%. Area under curve was 0.757 for first and 0.763 for second IRT (p 0.001, p 0.001, respectively). PPV was calculated as 4.3%. Conclusion: Although sensitivity of CF NBS is high in our country, its PPV is significantly lower than expected from CF NBS programs. False-positive NBS results could have been overcome by revising NBS strategy. What is Known: • Although immunoreactive trypsinogen elevation is a sensitive test used in cystic fibrosis newborn screening, its specificity is low. • In countries although different algorithms are used, all strategies begin with the measurement of immunoreactive trypsinogen in dried blood spots. What is New: • In our study, it was shown that use of the IRT/IRT protocol for cystic fibrosis newborn screening is not sufficient for the cut-off values determined by the high number of patients. • Newborn screening strategy should be reviewed to reduce false positive newborn screening results.

AIM In this study, we aimed to determine the psychosocial status of primary care givers of children with cystic fibrosis (CF) and its relationship with the clinical scores of children with CF. METHODS Thirty-six patients with CF and their primary care givers were assessed. A personal information form, as well as the Parent Attitude Research Instrument, Maslach Burnout Inventory, Beck Depression Scale and modified Shwachman-Kulczycki Score, were used for data collection. RESULTS All the CF children's primary care givers were their mothers. The mothers' occupation and educational level affected their child-rearing attitudes (P

Background Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. Methods In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). Results In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). Conclusions Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.

© 2022 Wiley Periodicals LLC.Background: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. Methods: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. Results: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). Conclusions: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.

Introduction: The COVID-19 pandemic can increase anxiety levels of children with chronic respiratory disease and their primary caregivers. Aims: To evaluate the anxiety of children with primary ciliary dyskinesia (PCD) and their families during COVID-19 pandemic Methods: A total of 29 patients and 105 healthy children and their mothers were included in this questionnaire-based study conducted via teleconference. Their demographic characteristics, clinical information, and information about caregivers’ exercise at home during pandemic were recorded. State and trait anxiety levels of children’s and mothers’ were evaluated and compared between two groups. The anxiety levels of patients’ mothers were evaluated according to the children’s clinical features. Mothers’ knowledge about COVID-19 was evaluated, and the effect of teleconference on their anxiety was assessed. Results: The median age of patients was 13 years. State anxiety of children in patients aged 13–18 years and trait anxiety levels of their mothers were lower than control group (P Conclusions: Children with PCD were less anxious than healthy children during pandemic, and their mothers exhibited lower trait anxiety than healthy children’s mothers. Obtaining information about COVID-19 and exercising during pandemic may have decreased anxiety levels of PCD patients’ mothers.

BACKGROUND Cystic fibrosis, a pulmonary disease which is an autosomal recessive, inherited, multisystemic genetic disease commonly seen in the Caucasian race, is the most frequent cause of mortality and morbidity. So far, more than 2000 disease-causing gene variants have been found and this number has been increasing with the studies conducted. Although there is not yet enough data that include the Turkish population, the recent increase of studies is noteworthy. AIMS To discover the genetic variation in patients diagnosed with cystic fibrosis in the Central Anatolian region. STUDY DESIGN Cross-sectional study. METHODS The study was carried out in the Central Anatolian region in 3 pediatric pulmonology departments (Kayseri, Konya, and Ankara) in Turkey between July 2014 and December 2017. The Sanger and Next Generation Sequence analyses were used for exon and exon-intron boundaries in the cystic fibrosis transmembrane conductance regulatory (CFTR) gene, and in selected patients, mutation analysis was performed using the Multiplex Ligation-dependent Probe Amplification technique for large deletions and duplications. RESULTS CFTR gene analysis was performed for 316 patients and 215 of them were genetically diagnosed with cystic fibrosis. Sixtythree different variants were defined in these patients and 7 of these were large deletions/duplications detected with the MLPA method. The most frequent variants were F508del (29.6%), G85E (8.2%), N1303K (8.2%), Y515* (7.5%), and G542* (3.4%). CONCLUSION Using sequencing and Multiplex Ligation-dependent Probe Amplification methods, the identification of seven new mutations that were not previously reported in the literature contributes to a better understanding of the heterogeneous nature of CFTR mutations in the Turkish population. When no mutations are detected (pathogenic/probably pathogenic) in clinically compatible cases, Multiplex Ligationdependent Probe Amplification analysis contributes significantly to the diagnosis.

Introduction: Pulmonary hypoplasia can cause decreased lung function and respiratory failure depending on its severity. Aims: To evaluate clinical characteristics of patients with pulmonary hypoplasia. Methods: Gender, age, complaints, age at diagnosis, diagnostic methods, parental consanguinity, presence of operation, age at operation and long-term complications of all patients with pulmonary hypoplasia were evaluated. Results: Of 18 patients with pulmonary hypoplasia, 44% were female and median age was 10.5 (min:0.9; max:192) months. The complaints were respiratory distress and cough. Median age at diagnosis was 0.41 (min:0; max:36) months. Median duration of follow-up was 8 (min:0.5; max:184) months. Parental consanguinity was present in 33.3% of patients. 66.6% of patients were diagnosed by CT, 22.2% by chest radiography and 11.2% by prenatal ultrasonography. 7 had accompanying diaphragma hernia (DH) and 7 had congenital heart disease. Others had PCD, Jeune syndrome, MPS and Fraser syndrome. Median age at diagnosis of patients with DH was 0.03 (min:0; max:1) months, and earlier than others (p=0.007). Patients born with DH were hospitalized due to respiratory distress. Median age at operation of 9 patients was 2 (min:1; max:150) days. Most common cause of hospitalization in follow-up was pneumonia. There was correlation between age at diagnosis and number of pneumonia (r=0.654; p=0.026). 3 operated patients had chest deformity, 2 had scoliosis, and 2 had diaphragm eventration. Conclusions: Pulmonary hypoplasia can be detected with respiratory distress symptoms in neonatal period, or it may occur incidentally in later life. Pulmonary infections can be reduced in patients who are diagnosed and treated early.

Introduction: Pulmonary nodules detected by thoracic imaging in children can be seen as a systemic disease finding or incidentally. Aims: To evaluate children referred to pediatric pulmonology department with pulmonary nodules Methods: Gender, age at admission, symptom, pulmonary function tests, radiologic results, diameters and locations of nodules of all children with pulmonary nodules were analyzed. Results: The mean age of all 45 patients was 12.3±3.8 years and 62.2% were female. The mean follow-up duration was 12.9±19.9 months. 26 (57.8%) patients had symptoms at admission and 46.2% of symptoms were cough. 24 (53.3%) patients had comorbid diseases, 7 (29.2%) had rheumatological, and others had gastroenterological, nephrological, dermatological and cardiological diseases. Chest radiographs were mostly normal (57.1%), while the most common finding on thoracic CT was nodule with fibrotic thickening (87.5%). The mean diameter of nodule was 3.2±2.6 mm. Solitary nodule was found in 31 (68.9%) patients and 21 of them had symptoms at admission. Presence of symptoms was more common in patients with solitary nodules than those without (p=0.045). Nodules were most common in the lower lobes of the lungs (77.1%) and 50.0% of the nodules were only in the right lung. 25 (55.6%) patients had pulmonary function tests and mean FEV1, FVC, FEV1/FVC, and MEF25–75 values were 100.4±12.1%, 96.8±13.2%, 103.8±9.6, and 98.9±28.5%, respectively. There was no significant difference between diameter and location of nodules and gender (p>0.05). The diameter and location of nodules were not correlated with age at admission and symptoms. Conclusions: Nonspecific pulmonary nodules in children can be seen and may have no effect on pulmonary function test of children.

Introduction: Non-cystic fibrosis (CF) bronchiectasis is a chronic inflammatory lung disease that causes persistent productive cough, recurrent pneumonia and obstructive pulmonary disease. Aims: To evaluate etiology and clinical features of children with non-CF bronchiectasis. Methods: All children with non-CF bronchiectasis were included in the study. Patients’ gender, parental consanguinity, age at diagnosis, complaints, duration of complaints, etiology of bronchiectasis, radiologic findings, pulmonary function test (PFT) results and operations were evaluated. Results: Of the 77 patients with non-CF bronchiectasis, 47.4% were female and mean age at diagnosis was 10.3±4.0 years. The median duration of complaints was 78 (IQR:12-120) months. Parental consanguinity was present in 59.4% of patients. Most frequent symptoms were chronic productive cough, sputum expectoration, shortness of breath, wheezing, hemoptysis and weight loss. Common causes were post-infectious complication in 50.6%, PCD in 33.7%, immunodeficiency in 9.0%, history of tuberculosis in 3.8%, history of foreign body aspiration in 1.3% and other anomalies in 1.6% of patients. Pulmonary lobe was involved in 60.8% left lower lobe, 50.9% right lower lobe, 40% right middle lobe, 25.6% right upper lobe, and 14.2% left upper lobe. Fifty-one patients were able to perform PFT. Mean of FEV1, FVC, FEV1/FVC, FEF25-75 were 81.8±21.4%, 82.3±19.7%, 87.0±22.2, 83.5±27.8% respectively. Lobectomy was performed in 5 patients with localized bronchiectasis causing recurrent pneumonia. Conclusions: Although infections are most common causes of non-CF bronchiectasis, other underlying diseases should be investigated.

We aim to evaluate the anxiety levels of caregivers of children with tracheostomy during the COVID-19 (coronavirus disease 2019) pandemic. Caregivers of 31 children with tracheostomy and 105 healthy children (control group) were included. The State-Trait Anxiety Inventory was administered via teleconference in order to investigate how participants describe how they feel at a particular moment (State) and how they generally feel (Trait). The trait anxiety levels of caregivers of children with tracheostomy were significantly higher ( P = .02). Their state anxiety levels were similar. The state and trait anxiety levels of caregivers of children with tracheostomy correlated ( r = 0.70, P < .001). At the end of the teleconference, caregivers of children with tracheostomy experienced greater anxiety relief than controls ( P < .001). Trait anxiety scores were higher among caregivers of children with tracheostomy, but their state anxiety levels were comparable to those of controls. Caregivers with high trait anxiety also exhibited high state anxiety. Informing caregivers of children with tracheostomy about COVID-19 via teleconference can reduce their anxiety during such stressful times.

Objective To objectively investigate the effect of passive smoking on pneumonia and disease severity in children aged less than 5 years by using cotinine as an indicator of passive smoking. Methods Between December 2015 and April 2016, children aged less than 5 years with pneumonia and age-matched healthy controls were included in this study, which was conducted at three tertiary pediatric pulmonology centers. A questionnaire was given to the parents regarding demographic data and smoking status at home. Urinary cotinine/creatinine ratio (CCR) was measured. The data from the pneumonia and control groups, as well as children with mild and severe pneumonia within the pneumonia group, were compared. Results A total of 227 subjects were included in the study; there were 74 children in the pneumonia group and 153 in the control group. The mean age of all the children was 33.4 ± 1.28 months. Of all subjects, 140 were male and 102 were exposed to passive smoking by their parents at home. There were statistically significant differences in age, number of people in the home, and mother’s and father’s age between the control and pneumonia groups (p 0.05). Age and urinary CCR were significantly different between children with mild and severe pneumonia (p Conclusion We showed that passive smoking exposure was associated with the development of severe pneumonia in children. Further studies are needed to examine the underlying cause in detail.

The pathophysiological mechanisms of primary ciliary dyskinesia (PCD) may affect many functions, including respiratory, physical, and health status. This study aimed to compare respiratory muscle strength, inspiratory muscle endurance, muscle strength, exercise capacity, physical activity levels, and quality of life in PCD patients and controls.Twenty-seven patients and 28 controls were included. Respiratory muscle strength (maximal inspiratory (MIP) and maximal expiratory (MEP) pressures), inspiratory muscle endurance (incremental threshold loading test), muscle strength (quadriceps femoris, shoulder abductor, elbow flexor, handgrip), exercise capacity (6-min walk test (6MWT)), physical activity and quality of life (QOL-PCD) were evaluated.MIP, inspiratory muscle endurance, quadriceps femoris, and handgrip muscle strength, 6MWT distance, total energy expenditure; childrens' (6-12 years) and their parents' physical function, upper, lower respiratory, and hearing symptoms and treatment burden QOL-PCD subscales scores were significantly lower in patients compared with controls (p 0.05). The 66.7% of patients did not meet the optimal number of steps. MEP, shoulder abductor, and elbow flexor muscle strength, active energy expenditure, physical activity duration, average metabolic equivalents, number of steps, lying time, and sleep duration; childrens' and their parents' other subscales and adolescents' all QOL-PCD subscales scores were similar between groups (p 0.05).Inspiratory muscle strength and endurance, lower extremity and total muscle strength, exercise capacity, total energy expenditure, and childrens' quality of life are impaired compared to healthy controls. Decreased physical activity level is prevalent in these patients. Effects of pulmonary rehabilitation on these impaired outcomes for PCD patients should be investigated.Clinicaltrials.gov: NCT03370029; December 12, 2017.

Objectives To evaluate the risk factors of recurrent pulmonary exacerbation and poor prognosis in children with idiopathic pulmonary hemosiderosis (IPH). Methods In this multicenter study, 54 patients with a diagnosis of idiopathic pulmonary hemosiderosis were included. Medical records were retrospectively reviewed from three tertiary care hospitals between 1979 and 2019. Also, current information and the long-term progress of patients was determined by contacting the families by telephone. Results A total of 54 children were included. The median age of onset of symptoms was 4.5 years (3 months to 15.8 years). The median time from onset to diagnosis was 0.9 years (0.25 months to 12 years). The mean number of recurrent episodes per child in the recurrence-positive group was 3.55 (1-15). Univariate analysis demonstrated that patients presenting with hypoxia or requiring transfusion at the time of presentation had significantly more recurrence episodes (P=0.002). Multivariate analysis showed that the presence of hypoxia at the time of initial presentation was a significant independent predictor of recurrent episodes (P=0.027). The median follow-up was 3.3 years (0.75 months-27 years). There was a significant relationship between the presence of hypoxia, transfusion history, ANA positivity, and elevated transaminases at the time of initial evaluation and treatment response. Conclusions The present study provides substantial information regarding factors that may affect recurrent exacerbations and prognosis in children with idiopathic pulmonary hemosiderosis. Demonstrating hypoxia as an independent risk factor in recurrence episodes could guide physicians in the planning of treatment strategies. This article is protected by copyright. All rights reserved.

© 2021 The Author(s) [2021]. Published by Oxford University Press. All rights reserved.Mycobacterium abscessus appears to be increasing cause of pulmonary infection in children with underlying risk factors including cystic fibrosis, chronic lung disease and immunodeficiency syndromes. We present a case of pulmonary M. abscessus infection in a pediatric patient with primary ciliary dyskinesia and he was successfully treated with parenteral amikacin, linezolid and oral clarithromycin combined with inhaled amikacin. Clinical improvement was observed after adding inhaled amikacin to the treatment.

Background: Niemann-Pick disease (NPD) is caused by abnormal storage of sphingomyelin. NPD may affect the pulmonary system and cause hypoxia. In the present case, both hepatopulmonary syndrome (HPS) and pulmonary arteriovenous fistulas (PAVFs) developed in a child with NPD and were successfully treated with repeated embolization. Case Presentation: We have reported the case of a 16-year-old-girl with NPD who suffered severe hypoxia, dyspnea, fatigue, had multiple PAVFs, and was diagnosed with type 2 HPS. To improve oxygenation, 10 PAVFs were embolized. She needed re-embolization after 9 months because of hypoxia redevelopment. Conclusions: Pulmonary involvement, HPS, and/or PAVFs could be responsible for hypoxemia in patients with NPD, who should, therefore, be investigated for HPS and PAVFs. Embolization could be beneficial. Some patients may need repeated embolization.

Background Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical courses beyond infancy have rarely been assessed. Methods Patients included in the Kids Lung Register diagnosed with PTI as infants and now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry and clinical outcome were analyzed. Results Thirty-five children older than 5 years with PTI diagnosed as infants were analyzed. At the age of 5 years, 74% of the patients were reported as asymptomatic and did not develope new symptoms during the observational period at school-age (mean, 3.9 years; range, 0.3-6.3). At the age of about 10 years, none of the symptomatic children had abnormal oxygen saturation during sleep or exercise anymore. Lung function tests and breathing frequency were within normal values throughout the entire observational period. Conclusions PTI is a pulmonary disease that can lead to respiratory insufficiency in infancy. As at school age most of the previously chronically affected children became asymptomatic and did not develop new symptoms. We conclude that the overall clinical course is favorable.

Objective: Primary ciliary dyskinesia (PCD) is an inherited, chronic respiratory disease with defective mucociliary clearance. There is no gold standart diagnostic test for PCD. Up-to-date 43 different genes described as related with PCD however only 65-70% of patients could be diagnosed with genetic analysis. We reported two siblings diagnosed as PCD with a novel mutation. Case 1: Thirteen year-old, girl patient had chronic rhinits and wet cough after the birth. She had recurrent pneumonia. Nasal polip was present in physical examination. His brother had situs anomaly. Heterozygous DNAH5 (c.5563dupA p.(Ile1855Asnfs*6) mutation and a novel heterozygous c.2511dupC p.(Thr838Hisfs*15) mutation were detected in genetic analysis. Case 2: Six year-old male patient had chronic rhinitis after the birth and dextrocardia was detected in neonatal period. After the PCD diagnosis of her sister he was evaluated and atelectasis was present on chest X-ray. The same mutations with his sister were detected. Sanger confirmation and family segregation studies were performed for both patients and the mutations were found to be compound heterozygous. Method: PCD has different clinical findings. All findings that may be associated with PCD should be questioned in pateints and also their families. However, c.5563dupA is a known mutation in PCD patients, c.2511dupC mutation is a novel mutation and was not reported before. Mutation analysis is important in terms of genetic counseling, determining the genotype phenotype relationship and potential new drugs that may be developed in the future.

Objective: Knowledge about the lung disease caused by ABCA3 mutation is limited. There are no clinical trials for treatment. We reported a patient with progressive lung disease due to homozygous ABCA3 mutation. Case presentation: A 2.5 month-old boy patient had tachypnea, growth failure, cyanosis and needed 10 l/min oxygen supplementation. Common ground glass opacities were present on the chest CT. Lung biopsy was compatible with chronic pneumonitis of infancy with focal pulmonary alveolar proteinosis consistent with surfactant metabolism disorders and a new homozygous mutation in ABCA3 at p.Q1045R was detected. Prednisolone, azithromycin and hydroxychloroquine treatments were started and his oxygen demand decreased to 1.5 l/min. Prednisolone treatment was stopped after the second year due to clinical improvement. On the fourth year of the treatment he had an exacerbation and he was hospitalized. Oxygen demand increased from 1.5 l/min to 10-15 l/min. Chest CT was compatible with progression of the disease. Steroid treatment was started again. He had no improvement and cyclosporin A (CsA) treatment was started. But no clinical healing was observed with CsA treatment and CsA started to tapered and was stopped on the 3rd month of treatment. Z score of bone mineral densities was -3.9. He had cushingoid appearance, hirsutism and needed 10 L/min oxygen supplementation. Steroid was gradually reduced due to side effects. Oxygen need decreased to 8 l/ min and cushingoid appearance disappeared. Conclusion: The definitive treatment for ChILD are unknown. Patients should be closely monitored for their clinical status, effects and side effects of the drugs, and treatment should be planned specifically for the each patient.

Objective: Primary Ciliary Dyskinesia (PCD) is an inherited disease with defective mucociliary clearance. Patients with PCD may present with different clinical findings besides classic findings. We aimed to review the clinical features and factors that affect the diagnose age of patients with PCD. Material and Method: Between 2007-2019, all 74 patients who were followed up with PCD in pediatric pulmonology department were included in this study. Demographic features, age of diagnosis, clinical findings, PICADAR (PrImary CiliAry DyskinesiA Rule) score and pulmonary function test (PFT) of the patients were evaluated. Results: The mean age of diagnosis was 98.7±55.2 months and the mean duration of complaints was 62.0±4.7 months. Most of the patients (95.9%) had persistent wet cough, 37.8% had a history of hospitalization in neonatal intensive care unit, 43.2% had a respiratory symptom in newborn period, 81.1% had history of pneumonia, 35.1% had bronchiectasis and 50% had situs anomalies. The mean PICADAR score was 6.5±3.1. The mean age of diagnosis of patients with situs anomaly was earlier than the others and PFT values of them were lower than those without (p Method: Primary Ciliary Dyskinesia can cause different clinical signs at different ages. Although most of the patients have prominent findings related with PCD they are often diagnosed lately. Better PFTs and clinical findings may cause delayed diagnose. PCD should be kept in mind in differential diagnosis in children with recurrent respiratory complaints.

Background: Cystic Fibrosis Registry of Turkey shows various CFTR mutations due to the geographical location and historical background of our country, and also high prevalence of consanguineous marriages. Method:All mutations detected in the Cystic Fibrosis Registry of Turkey 2017 (CFRT2017) data were screened in CFTR1 and CFTR2 databases. Mutations which were not found in both were identified and characteristics of these patients were compared with F508del homozygous patients. Results: Among 1170 registered patients, 978 were genotyped and 200 different mutations were shown in 1270 alleles.29 mutations were not reported in both databases; 58 mutations have been reported in CFTR1 but not in CFTR2. Demographic and phenotypic characteristics of the 112 patients with 87 different alleles those were not previously reported in the CFTR2 database (nonCFTR2 group) were compared with F508del homozygous 103 patients in CFRT2017. In the nonCFTR2 group, mean age was younger (5.81 vs 7.69; p:0.015), mean age at diagnosis was older (1.88 vs 1.08; p:0.041), sweat test was lower (75.12 vs 98.28; p Conclusion: We suggest that patients in the nonCFTR2 group have a mild clinical course, but in some patients, further investigations and functional studies are required for the exact diagnosis.

Objectives: Biological agents are frequently used in inflammatory diseases in childhood. We aim to determine the incidence of latent tuberculosis infection (LTBI) and tuberculosis disease (TB) during follow-up due to use of biological agents. Methods: Between 2008-2019, all 194 patients who were followed up in pediatric pulmonology department in terms of LTBI and TB disease occurance while using biological agents were included in the study. Tuberculin skin test (TST), interferon gamma release assay (IGRA) tests and lung imaging (chest X-ray and/or tomography) before and during the treatment with biological agents were evaluated. Results: The mean duration of biological agent treatment was 35.5±25.6 months and 156 (80.4%) patients had rheumatological diseases. In the pretreatment evaluation 26 (13.4%) patients had positive TST and all these patients were given isoniazid (INH) prophylaxis after excluding TB disease. During follow-up, TST of 5 (2.6%) patients became positive and they received INH prophylaxis. TB disease was occured in follow up of one of the TST-negative patients. IGRA became positive in the second year of biological agent, sputum culture grew M. tuberculosis and the chest CT showed tree-in-bud pattern consistent with pulmonary tuberculosis. Conclusions: There is a risk of LTBI and TB disease under biological agent treatment in children. Care should be taken that TB can be seen during treatment even in patients who did not have LTBI in pretreatment evaluation of biological agents and patients should be controlled frequently in terms of developing LTBI and TB disease.

Introduction: Bronchiolitis obliterans (BO) is as a rare pulmonary complication of Stevens Johnson Syndrome (SJS). We present a patient who was thought to have BO after SJS but there was no radiological finding compatible with BO on the first thorax CT. However one month later thorax CT findings became consistent with BO. Case Report: A 12-year-old female patient was diagnosed with SJS after using oseltamivir, ibuprofen and trimethoprim sulfometaxazole and was treated with IVIG and steroids. One month later, she had cough, shortness of breath. Physical examination revealed bilateral decreased breath sounds, common rhonchi and crackles on both lungs. Oxygen saturation was 95% and respiratory rate was 20/min. Obstructive and restrictive patterns were detected in pulmonary function test (PFT). There was no findings compatible with BO on thorax CT in the expiratory phase. Steroid, azithromycin and chest physiotherapy were started due to the clinical findings and PFT results suggesting BO. In the second month control, bilateral patchy air trapping and peribronchial thickening were detected in thorax CT in the expiratory phase, which are thought to be compatible with BO. Steroid therapy was tapered and stopped due to significant improvement in the patient9s clinical findings. Method: Bronchiolitis obliterans secondary to SJS is generally progressive and has poor prognosis. Close monitoring of patients with SJS is important for timely detection of BO. BO is mainly diagnosed by thorax CT however radiological findings may occur in the late period.

Objective: Pulmonary hypertension (PH) is a rare condition associated with diverse cardiac, pulmonary, and systemic diseases in childhood. We aimed to detect the frequency of PH due to pulmonary diseases and clinical features of them in a pediatric pulmonology department. Method: Between 2008-2019, all patients with PH who were followed in pediatric pulmonology department were evaluated in terms of age of diagnosis, cause of PH, treatments and outcome. Results: A total of 41 patients were followed up with PH, 53% of them were female. Mean age of diagnosis was 4.07±5.1 years. Mean age of diagnosis was 3.14±4.85 years in patients with cardiac or pulmonary anomaly and 6.01±5.28 years in patients with idiopathic PH (p>0.05). Of these, 19 patients (46%) had cardiac, 20% had pulmonary disease related with PH, 3% had genetic disorder and 31% had idiopathic PH. Seven of the patients with cardiac anomaly had accompanying Down syndrome. Four of 19 patients with cardiac anomaly had accompanying pulmonary disorders. In pulmonary disease group, 6 patients had bronchopulmonary dysplasia, one had bronchiolitis obliterans, one had cystic fibrosis, and one patient had surfactant protein C deficiency. Patients were treated with endothelin receptor antagonists and/or phosphodiesterase inhibitor and/or furosemide and/or digital and/or angiotensin converting enzyme blockers and 11 of all them needed oxygen supplementation. In the follow up four patients were died. Method: PH is a serious disease in childhood with high mortality. Cardiac, pulmonary, genetic and systemic diseases should be investigated in patients with PH. Patients may have more than one systemic cause of PH even they have a cardiac anomaly related with PH.

Introduction: Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar hemorrhage in children with unknown underlying pathophysiology. The prognosis of the disease is extremely different between patients. There is limited study evaluated prognostic factors in IPH. Methods: We performed a multi-center, retrospective study in three tertiary care hospital between 1979-2019. Results: A total of 54 children included to the study. The mean age of onset of symptoms was 62.72 ± 45.8 months. The mean age at diagnosis was 80,2 months. The average time from onset to diagnosis was 18 months. The mean follow-up period was 56.7 months (0.75months to 27years). Patients are categorized into three groups according to treatment response; good, partial, and poor. Thirty-seven patients with long term follow-up or current prognosis information present were included in this grouping. Six of the patients were in a good response group, 18 of the patients were in partial response groups, and 13 of the patients were in the poor response group. There was no significant difference in terms of the age of diagnosis, sex, onset time of symptoms, consequently, initial signs, radiologic findings, and PFT. There was a significant relationship between the presence of hypoxia, transfusion history, ANA positivity, and elevated liver enzyme at the time of initial evaluation and treatment response. Conclusions: We evaluate a large pediatric cohort who were under follow-up for a long time. To the best of our knowledge, this association of hypoxia and ANA positivity and poor prognosis never been reported. This information would be useful for clinicians to decide treatment strategies.

BACKGROUND AND OBJECTIVES Hypersensitivity pneumonia is a complex condition due to exposure time, intensity, different clinical presentation, and treatment practices. We aimed to evaluate the patients that were diagnosed with hypersensitivity pneumonia (HSP) due to exposure to pigeons and a review of the literature for diagnosis and treatment of Pigeon Breeder`s Disease (PBD) in children. METHOD Between the years of 2009-2018, patients who were diagnosed with HSP due to PBD were included in the study in a pediatric pulmonology department. Findings of our patients, treatments, and prognoses were compared with 17 articles in the literature about PBD in children. RESULTS In a 9 year-period, 6 patients were diagnosed as HSP due to PBD. The mean age of the patients was 8.8 ± 5.4 years and the average duration of pigeon exposure was 60.1 ± 6.5 days. Precipitating antibodies were positive in 3 patients. In four cases, symptoms were resolved with only prevention of pigeon exposure. Two patients who had close contact with pigeons needed oxygen supplementation and steroid therapy. CONCLUSION Hypersensitivity pneumonia should be considered for the differential diagnosis of patients that present with respiratory distress, cough, fever, and weight loss. Prolonged exposure and close contact may worsen the clinical symptoms. In most cases, only exposure prevention is enough, while steroid therapy, oxygen support, and intensive care monitoring may be required in severe cases.

Background We aimed to evaluate anxiety among children with cystic fibrosis (CF) and their mothers related to the COVID‐19 pandemic. Methods A total of 45 patients with CF and their mothers were enrolled in the study together with 90 age‐matched healthy children and their mothers as a control group. The State and Trait Anxiety Inventory (STAI) was administered by teleconference with children aged 13–18 years old and their mothers. The STAI for children was administered with children aged 9–12 years. Results were compared with age‐matched healthy children and their mothers. The relationship between anxiety scores of children with CF and their mothers was evaluated by comparing with clinical data of children with CF. At the conclusion of the teleconference, mothers were asked whether their anxiety had changed as a result of the interview. Results It was found that healthy children aged 13–18 years had higher state anxiety scores than age‐matched children with CF. Mothers of children with CF had higher trait anxiety scores, especially those of children aged 0–12 years, than mothers of healthy children (p

Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Dogru, Deniz/0000-0001-9931-9473; Aslan, Ayse Tana/0000-0002-5360-8517; COBANOGLU, NAZAN/0000-0002-3686-2927; ORHAN, FAZIL/0000-0002-4850-932X WOS: 000530442700001 PubMed: 32364312 Background Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. Methods The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. Results Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. Conclusion PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.

Kistik fibrozis ile ilişkili diyabet (KFİD), kistik fibrozisin (KF) yaygın bir komplikasyonudur. Kistik fibrozis ile ilişkili diyabetin temel nedeni hastalarda yıllar içerisinde pankreas hasarının artması ile beraber pankreas adacık hücrelerinin kaybı ve insülin eksikliğinin gelişmesidir. Tedavideki amaç çocuk ve adolesanlarda normal büyüme ve gelişmenin ve kan glukoz regülasyonunun sağlanmasıdır. Tedavinin temelini insülin tedavisi ve tıbbi beslenme tedavisi oluşturur. İnsülin tedavisi, KFİD hastalarında akciğer fonksiyonunu stabilize eder ve beslenme durumunu iyileştirir. Kistik fibrozis ile ilişkili diyabetli hastalara yüksek enerji, yüksek yağ ve yüksek tuz içeren bir beslenme planı önerilmektedir. Karbonhidrat sayımı, insülin tedavisi alan hastalarda öğün öncesi insülin dozunun belirlenmesinde yararlıdır. Bu yazıda kliniğimizde izlenen KFİD’li bir çocuk olgunun medikal ve tıbbi beslenme tedavisi literatür derlemesi eşliğinde sunulmuştur.

Asthma and postinfectious bronchiolitis obliterans (PIBO) have similar clinical findings, and PIBO may be misdiagnosed with asthma. This study aimed to determine the clinical features of PIBO in children and the causes of delay in its diagnosis.We retrospectively evaluated all patients diagnosed with PIBO in four pediatric pulmonology centers between 2007 and 2018. In total, 64 PIBO patients were retrospectively reviewed. We compared the clinical and laboratory differences between PIBO patients who had initially been misdiagnosed with asthma and correctly diagnosed with PIBO.Of the 64 patients, 22 (34.4%) had initially been misdiagnosed with asthma. Adenovirus was the most common infectious agent in children. The age upon diagnosis was older, and the symptom duration was significantly longer in patients misdiagnosed with asthma (P .05). There were no statistical differences in terms of sex, history of prematurity, duration of hospitalization, treatment, history of oxygen or mechanical ventilation support, pulmonary function test (PFT) results and asthma-predisposing findings between the two groups (P .05).Patients with PIBO who had initially been misdiagnosed with asthma were correctly diagnosed at older ages and had longer symptom duration. Asthma may mask PIBO diagnosis by the similarity of symptoms and the clinical response to inhaled β2-agonist or steroid treatment. PFTs may not help clinicians because of the age of children. The delay in the diagnosis of PIBO is probably attributable to the fact that some clinicians fail to include PIBO in the differential diagnosis when there is no clinical response to asthma medication.

Harmanci, Koray/0000-0002-8494-648X; Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Aslan, Ayse Tana/0000-0002-5360-8517 WOS: 000535323700001 PubMed: 32453906 Background A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. Methods Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. Results Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). Conclusions The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.

Introduction No clear information exists about the factors affecting pleural thickening following parapneumonic effusion in children. We aimed to investigate factors that affect the resolving time of pleural thickening after parapneumonic effusion. Methods Between the years of 2007-18, 91 patients, which were followed due to diagnosis of pleural thickening after parapneumonic effusion, were assessed. Ages, complaints, physical examination findings, laboratory results, chest x-ray and ultrasonography findings, treatments, duration of treatment and recovery time of the patients were examined terms in of pleural thickening resolving time. Results The mean age of patients was 7.5 ± 5.0 years. Pleural thickening resolving time was 151 ± 6.8 days. The resolving time for pleural thickening was delayed with older ages, longer duration of complaints, fever before hospital admission and treatment, lower oxygen saturation at the time of admission, crackles in the physical examination, higher white blood cell count and pleural fluid density (p = 0.018, p = 0.001, p = 0.021, p = 0.020, p = 0.024, p = 0.025, p = 0.021, p = 0.019). In addition, the amount of effusion measured by thorax ultrasonography, fibrinolytic usage, and complications had a role in the delayed resolving time (p = 0.034, p = 0.001, p = 0.034). Pleural thickening resolved in 80% of the patients. Conclusion In this report, 80% of pleural thickening, following parapneumonic effusion resolved within 5 months. Patients who do not have a complication during follow-up are not required to monitor with frequent chest x-ray. Patients with a higher amount of pleural effusion, complications and need for fibrinolytic treatment should be followed more carefully.

BACKGROUND Primary ciliary dyskinesia (PCD) patients have higher incidence of sleep disordered breathing which lead neurocognitive impairments such as attention-deficit/hyperactivity disorder (ADHD). It may effect academic performance of children and may cause impairment in emotional relationships. This study aim to evaluate hyperactivity and attention deficiency in PCD patients and investigate the relationship between sleep and hyperactivity and attention deficiency in PCD patients. METHOD Fifteen PCD patients aged 8-18 years and 31 age-matched healthy controls were compared. Ear, nose, and throat examination and home sleep testing were performed in PCD patients. Pediatric sleep questionnaire, Conners' Parents and Teacher scale and Stroop test were applied in both groups in order to investigate the relation between sleep disordered breathing and ADHD in PCD children. RESULTS PCD patients had chronic rhinosinusitis (100%), tonsillar hypertrophy (80%) and adenoidal hypertrophy (60%). FEF25-75 was low in pulmonary function test. Sixty percent of the PCD patients had mild obstructive sleep apnea syndrome in home sleep testing. Mean AHI was 1.54 ± 0.27. Compared with the controls PCD patients had higher PSQ scores. Hyperactivity scores on Conners' Parents scale and inattention findings in Stroop test were higher in PCD patients than the healthy controls (P

Nocardia spp is a gram-positive aerobic filamentous bacteria that causes pulmonary and systemic infections, especially in patients with immunosuppression or chronic lung diseases. It is rarely reported in children with cystic fibrosis. Macrophage activation syndrome is a life-threatening disease with an excessive inflammatory response usually triggered by infections. There are few reports in cystic fibrosis related to macrophage activation syndrome. Herein we report a child with cystic fibrosis who had macrophage activation syndrome due to Nocardia infection.

Background/aim: Airway compression (AC) by vascular structures is an important complication of congenital heart disease (CHD) that often goes unrecognized. It is not easy to identify whether CHD patients require additional invasive examinations or not. Therefore, the present study aims to develop an AC diagnostic algorithm for CHD patients. Materials and methods: CHD patients with persistent respiratory symptoms that were treated between January 2007 and December 2015 were retrospectively reviewed. The following data were recorded for all CHD patients with AC: age, cardiac anomalies, the compressed structure, the airway diameter ratio (ADR), the compressing structure(s), treatment, and follow-up.Results: During the 8-year study period, 62 of 253 CHD patients had persistent respiratory symptoms, of which 11 cases were diagnosed as AC via bronchoscopy and/or thoracic computed tomography angiography. The most frequently affected structures were the left main bronchus and trachea, and the most common compressing structure was the right pulmonary artery. The ADR was near total compression in 3 patients and >0.50 in 3 patients. During follow-up, 5 of the 11 patients with AC underwent surgery, 2 died, and 4 were followed clinically. Patients with ADR of >0.50 did not require surgery and were followed clinically. Conclusion: CHD patients with persistent respiratory symptoms associated with lower respiratory airway obstruction should be evaluated via invasive examination. An AC diagnostic algorithm for pediatric CHD patients was developed.

Background: Knowledge of pulmonary complications (PCs) in children after hematopoetic stem cell transplantation (allo-HSCT) is limited; most data are from adult studies. Case: We describe a 8 year old girl with high risk acute myeloid leukemia who developed graft versus host disease (GVHD) on Day 20, Cytomegalovirus (CMV) pneumonia on Day 50 and Cryptogenic organizing pneumonia (COP) on Day 170 after allo-HSCT. Discussion: Cryptogenic organizing pneumonia is a rare noninfectious PCs that can be idiopathic or have several risk factors as a secondary causes, such as viral respiratory infections, drugs, GVHD and allo-HSCT. Viral respiratory infections and alloimmune lung syndromes have been reported in a few patients who have undergone transplantation. Conclusion: Transplant physicians should be kept in mind for the development of alloimmune lung syndrome in the form of COP following CMV pneumonia in patients after allo- HSCT