Pediatric Pulmonology Department Research Articles

Changing clinical characteristics of pediatric inpatients with pneumonia during COVID-19 pandamic: a retrospective study

BackgroundThe COVID-19 pandemic have impacts on the prevalence of other pathogens and people’s social lifestyle. This study aimed to compare the pathogen, allergen and micronutrient characteristics of pediatric inpatients with pneumonia prior to and during the COVID-19 pandemic in a large tertiary hospital in Shanghai, China.MethodsPatients with pneumonia admitted to the Department of Pediatric Pulmonology of Xinhua Hospital between March-August 2019 and March-August 2020 were recruited. And clinical characteristics of the patients in 2019 were compared with those in 2020.ResultsHospitalizations for pneumonia decreased by 74% after the COVID-19 pandemic. For pathogens, virus, mycoplasma pneumoniae (MP) and mixed infection rates were all much lower in 2020 than those in 2019 (P < 0.01). Regarding allergens, compared with 2019, the positive rates of house dust mite, shrimp and crab were significantly higher in 2020 (P < 0.01). And for micronutrients, the levels of vitamin B2, B6, C and 25-hydroxyvitamin D (25(OH)D) in 2020 were observed to be significantly lower than those in 2019 (P < 0.05). For all the study participants, longer hospital stay (OR = 1.521, P = 0.000), milk allergy (OR = 6.552, P = 0.033) and calcium (Ca) insufficiency (OR = 12.048, P = 0.019) were identified as high-risk factors for severe pneumonia by multivariate analysis.ConclusionsThe number of children hospitalized with pneumonia and incidence of common pathogen infections were both reduced, and that allergy and micronutrient status in children were also changed after the outbreak of the COVID-19 pandemic.

Hyperventilation syndrome in children with asthma

Background Hyperventilation syndrome (HVS) may be associated with asthma. In the absence of a gold standard diagnosis for children, its impact on asthma has been rarely assessed. Objective To assess the impact of HVS on the symptoms and lung function of children with asthma and determine the diagnostic value of the Nijmegen questionnaire in comparison to a hyperventilation test (HVT). Methods Data from asthmatic children followed in the department of Pediatric Pulmonology of Necker Hospital and explored for HVS were retrospectively analyzed. HVS was diagnosed by a positive HVT. Asthma exacerbations, control and lung function were assessed in children with or without a positive HVT. The sensitivity and specificity of the Nijmegen questionnaire were determined relative to the positivity of a HVT. The Nijmegen questionnaire threshold was ≥23. Results Data from 112 asthmatic children, median age 13.9 years [11.6–16], were analyzed. Twenty-eight children (25%) had mild or moderate asthma and 84 (75%) severe asthma. The HVT was performed on 108 children and was negative for 34 (31.5%) and positive for 74 (68.5%). The number of asthma exacerbations in the past 12 months, Asthma Control Test (ACT) score, and lung function did not differ between children with a positive HVT and a negative HVT. The Nijmegen questionnaire was administered to 103 children. Its sensitivity was 56.3% and specificity 56.3%. Conclusion The symptoms and lung function of adolescents with asthma are not affected by the presence of HVS. The sensitivity and specificity of the Nijmegen questionnaire are low.

Connective tissue dysplasia in children with broncho-obstructive syndrome

BACKGROUND: Changes in the respiratory system in children with connective tissue dysplasia (CTD) have not been studied enough.&#x0D; AIM: To study the features of CTD in patients of the pediatric pulmonology department with broncho-obstructive syndrome and chronic inflammation of the bronchi, to assess complaints and the nature of the disease.&#x0D; MATERIALS AND METHODS: Patients admitted to the department were evaluated according to the table of diagnostic signs of CTD. 380 children were examined, aged 2 to 17 years 11 months.&#x0D; RESULTS: Out of 380 children, the following were identified: with bronchial asthma 309, with obliterating bronchiolitis 18, with bronchiectasis 11, with recurrent bronchitis 42. Three groups were distinguished: 1st patients with CTD (172; 45%), 2nd with increased dysplastic stigmatization (111; 29%), 3rd children without signs of CTD (97; 26%). Most patients admitted to the pulmonology department have signs of CTD or dysplastic stigmatization (74%). Children with CTD often complain of feeling unwell, asthenia, joint pain, they are more likely to have orthopedic pathology. Patients of the 1st group often complain of articular syndrome 19.2%, group 2 13.5%, children without CTD 4.1 % (p 0.001). Complaints about feeling short of breath were presented by 32.6% of patients with CTD, 21.6% with dysplastic stigmatization, and 7.2% in the comparison group (p 0.001). Children with CTD and dysplastic stigmatization more often had chest deformities.&#x0D; CONCLUSIONS: Patients with CTD are characterized by asthenic physique, changes in the axial skeleton chest deformities of II and III degrees, scoliotic deformity of the spine of II and III degrees, which may contribute to the development of the pathology of the respiratory system. CTD is a comorbid condition that significantly affects all organs and systems. Timely diagnosis and an integrated approach to these patients are required. Diseases proceed with severe clinical symptoms, require more active examination and aggressive treatment.

SARS-CoV-2 B.1.1.529 (Omicron) Variant Causes an Unprecedented Surge in Children Hospitalizations and Distinct Clinical Presentation Compared to the SARS-CoV-2 B.1.617.2 (Delta) Variant.

BackgroundIn the midst of successive waves of SARS-CoV-2 variants, the B.1.1.529 (omicron) variant has recently caused a surge in pediatric infections and hospitalizations. This study aimed to describe and compare the symptoms, explorations, treatment and evolution of COVID-19 in hospitalized children during the successive B.1.617.2 (delta) and B.1.1.529 (omicron) waves.MethodsThis observational study was performed in the Pediatric Pulmonology Department of a University Hospital in Paris, France. All hospitalized children aged between 0 and 18 years who tested positive for SARS-CoV-2 using reverse transcription polymerase chain reaction (RT-PCR) in nasopharyngeal swabs from July 15th to December 15th 2021 (delta wave), and from December 15th 2021 to February 28th 2022 (omicron wave) were included.ResultsIn total, 53 children were included, 14 (26.4%) during the delta wave and 39 (73.6%) during the omicron wave (almost three times as many hospitalizations in half the time during the latter wave). During the omicron wave, hospitalized patients were mostly aged < 5 years (90 vs. 71% of all the children during omicron and delta waves, respectively), and tended to have fewer underlying conditions (56 vs. 79% during omicron and delta waves, respectively, p = 0.20). The omicron variant was also responsible for a different clinical presentation when compared to the delta variant, with significantly higher and often poorly tolerated temperatures (p = 0.03) and increased digestive symptoms (p = 0.01). None of the three patients who were older than 12 years were fully vaccinated.ConclusionThe dramatic increase in the hospitalization of children with COVID-19 and the modification of the clinical presentation between the latest delta and omicron waves require pediatricians to remain vigilant. It should also encourage caregivers to ensure vaccination in children older than 5 years, for whom the BNT162b2 COVID-19 vaccine has been deemed safe, immunogenic, and effective.

The Importance of Flexible Bronchoscopy in Difficult-to-treat Asthma from a Pediatric Pulmonology Perspective.

ObjectiveAsthma is the most common chronic lung disease in childhood. Difficult-to-treat asthma is defined as the continuation of symptoms or attacks of patients despite step 4 or 5 of Global Initiative for Asthma therapy. In the differential diagnosis of these patients, flexible fiberoptic bronchoscopy is recommended to exclude other lung diseases. In this study, we aimed to examine the clinical and radiologic features and flexible fiberoptic bronchoscopy findings of patients referred to our pediatric pulmonology department due to difficult-to-treat asthma and determine the effects of flexible fiberoptic bronchoscopy on the differential diagnosis and treatment.Materials and MethodsThe demographic characteristics and flexible fiberoptic bronchoscopy results of 62 patients who were diagnosed as having difficult-to-treat asthma in our pediatric pulmonology department between January 2015 and June 2020 were evaluated retrospectively. The symptoms, history, medications, physical examination findings, pulmonary function tests, and radiologic findings of patients who underwent flexible fiberoptic bronchoscopy were evaluated.ResultsThe median age of the patients was 69 (interquartile range: 42-108 months). The most common reasons for the referral of these patients were chronic cough, recurrent pulmonary infections, and persistent wheezing. All patients had chest radiography and 37 (59.7%) had chest computed tomography at their first admission; 14 (37.8%) patients had abnormal findings on chest computed tomography. There was no significant difference in terms of age, physical examination findings, pulmonary function test results, and radiologic examinations between patients with and without pathologic bronchoscopy findings. None of the patients had complications during and after flexible fiberoptic bronchoscopy. The most common diagnoses of patients based on flexible fiberoptic bronchoscopy were persistent bacterial bronchitis in 19 (30.6%) patients, tracheomalacia and/or bronchomalacia in 12 (19.4%), and anatomic anomalies in 3 (4.8%) patients (separation of lingula into 3, separation of right upper lobe bronchus into 4, and tracheal dyskinesia). Mycobacterium tuberculosis growth was observed in the tuberculosis culture of 1 patient. According to the flexible fiberoptic bronchoscopy and bronchoalveolar lavage results, antituberculosis treatment was initiated in 1 patient and polypoid mass excision was performed in 1 patient. A proton pump inhibitor was started in 9 (15.5%) patients, physiotherapy in 5 (8.0%), antibiotics in 14 (22.5%), and ipratropium bromide in 7 (11.2%) patients. All patients were followed up with the diagnosis of asthma except for 2 patients.ConclusionTo date, there is no prospective study evaluating the importance of flexible fiberoptic bronchoscopy in difficult-to-treat asthma in childhood. In our small cohort, persistent bacterial bronchitis, airway tracheomalacia and/or bronchomalacia, gastroesophageal reflux, and other anatomic anomalies were successfully diagnosed using flexible fiberoptic bronchoscopy and treated without any complications, suggesting that flexible fiberoptic bronchoscopy is an important diagnostic tool with a low complication rate in children with difficult-to-treat asthma.KeywordsDifficult-to-treat asthma, flexible fiberoptic bronchoscopy, persistent bacterial bronchitis, gastroesophageal reflux

Cystic fibrosis newborn screening: Five-year experience from a tertiary care center.

Newborn screening (NBS) for cystic fibrosis (CF) was implemented in our country on January 1, 2015, based on immunoreactive trypsinogen tests (IRT/IRT). Here, we aimed to evaluate the diagnoses of patients and follow-up process within the first 5 years of NBS from a tertiary care center. This retrospective cohort study was conducted on patients who were admitted to our pediatric pulmonology department for sweat test (ST) via NBS. Patients with CF with negative NBS results and those with CF with positive NBS and joined our follow-up were also investigated. Clinical outcome measures were compared between patients with CF with positive and negative NBS. Six hundred sixty infants who were referred for ST via NBS were included. Across the entire study population (n = 683), 11.4% of patients had CF (14.1% of had negative NBS in this CF group). The sensitivity of NBS was found as 84.9% and the positive predictive value (PPV) was 9.4%. The median age at diagnosis was older (p < 0.001), reluctance for feeding and Pseudobartter syndrome (PBS) were significantly higher at presentation in the negative NBS group. There was no statistically significant difference between the groups regarding weight-for-age (p = 0.899) and height-for-age (p = 0.491) in the first 2 years' follow-ups. Our findings showed the low sensitivity and PPV of NBS; therefore, further studies based on all patients in our country are necessary for new cut-off values. PBS and reluctance for feeding should be alarm symptoms for CF even if the infants had negative NBS. Additionally, later diagnosis of patients who had negative NBS did not affect the nutritional outcomes; we need large-scale prospective studies to optimize nutritional benefits for all infants diagnosed via NBS.

Severe COVID‐19 evolving towards organizing pneumonia in a pediatric lung transplant recipient

Severe COVID‐19 evolving towards organizing pneumonia in a pediatric lung transplant recipient

Evaluation of age at diagnosis and clinical findings of children with primary ciliary dyskinesia.

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. 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. 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). 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.

VATS-резекція нижньої долі лівої легені як результат лікування віддаленого наслідку перенесеної аспірації стороннього тіла в дихальні шляхи дитини: клінічний випадок

Introduction. Foreign bodies in the airways are a very urgent problem that occurs at any age and quite often requires an urgent and sometimes urgent assessment of the situation, examination, and making the right decision. According to statistics, most often foreign body in airway are found in childhood. In about 95–98% of cases, this pathology is recorded in children aged 1.5 to 3 years. This is due to the behavior of children, their anatomical and physiological characteristics and underdevelopment of protective reflexes. Among all cases of foreign body in airway, foreign bodies of the larynx are found in 12%, trachea – in 18%, bronchus – in 70% of cases. In 80% of cases, CTs enter the right bronchus, as it is a broader and more direct continuation of the trachea. The correct diagnosis is established early after CT aspiration in 40–57% of patients. The mortality rate varies, according to different authors, from 2 to 15%. Clinical case. The boy V., born in 2008. was admitted to the Department of Pediatric Pulmonology on May 30, 2019, with complaints of frequent unproductive cough, mainly daytime, increasing with physical exertion, sometimes subfibril body temperature, weakness, lethargy, decrease appetite. These complaints have been observed for the third time in the last six months. Diagnosis: Foreign body B10 of the left lung. Conclusions. Aspiration of a foreign body into the respiratory tract most often occurs in early childhood (1–3 years). The clinical picture in the early stages is asymptomatic, and over time it leads to the development of inflammatory changes in the lungs, are treated conservatively. Given the complexity of diagnosis, aspiration of a foreign body can lead to frequent recurrent pneumonia, the formation of bronchiectasis in the lower parts of the lungs, which may require surgical treatment. The gold standard of diagnostics is fibrobronchoscopy and spiral computed tomography of the thoracic cavity (SCT OGK), with the help of which, early after aspiration, a foreign body can be detected and subsequently excluded from the respiratory tract. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of an participating institution.The informed consent of the child’s parents was obtained from the studies. No conflict of interest was declared by the authors. Key words: child, lungs, foreign body.

Correlation between the clinical severity of laryngomalacia and endoscopic findings.

Objectives:To correlate the clinical severity of laryngomalacia (LM) with endoscopic findings, swallowing evaluations and polysomnography in a cohort of patients .Methods:We conducted a retrospective analysis between 2017-2018 on a cohort of patients diagnosed with upper airway obstruction (UAO), stridor, noisy breathing or laryngomalacia. This study took place at the Pediatric Pulmonology Department, Riley Children’s Hospital, Indianapolis, United States of America.Results:There were 157 patients with laryngomalacia included in the study. Patients with severe LM were significantly younger than those with mild LM (p=0.0214) and moderate LM (p=0.0220). Subjects with type I of LM were significantly older than type III (p=0.0051).When associations were tested between polysomnogram (PSG) variables and clinical severity, there were significant associations with age at PSG. The overall apnea-hypopnea index (AHI) in mild (p=0.0103) and moderate (p=0.0242) were significantly lower than the severe group. The rapid eye movement (REM) AHI was significantly lower in moderate cases than severe (p=0.0134). The end-tidal carbon dioxide (EtCO2) peak was significantly lower in mild cases than severe (p=0.0141).The total sleep time (TST) peripheral capillary oxygen saturation (SpO2) 90% occurs in both mild (p=0.0197) and moderate (p=0.0498)) were significantly lower than the severe group.Conclusions:The severity of the clinical manifestations of LM did not correlate with the different endoscopic types in our study. The presence of cyanosis was associated with type III LM. Rapid eye movement AHI and EtCO2 in polysomnogram were remained significantly associated with clinical severity.

Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis.

Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood. Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with CF and investigate the association between the findings of the upper respiratory tract and markers of lung disease progression. Methods Retrospective study in patients with CF from the Pediatric Pulmonology Department who underwent otorhinolaryngological evaluation between 2015 and 2017. Nasal endoscopy and nasal swab collection were part of the evaluation. The severity markers used were: percentage of predicted forced expiratory volume in the first second (FEV1%), body mass index (BMI) and the Shwachman-Kulczycki (SK) clinical score. Results A total of 48 patients with CF were included. The mean of the predicted percentage of FEV1% was 83.36 ± 30.04. The average 14 and SK score 89.11 ± 10.50. The bacteriology of the nasal swab was positive in 27 (54.1%) patients. Staphylococcus aureus was positive in 18 patients, Pseudomonas aeruginosa in 5, Pseudomonas cepacea in 3 and Stenotrophomonas maltophila in 1 patient. Nasal polyps were found in nine participants. Nasal polyps were found in nine participants and were associated with lower SK score. Conclusion The pathogens found in the upper airway were, in order: S. aureus , P. aeruginosa , P. cepacea e S. maltophila . The presence of polyps in the nasal cavity showed statistical significance and appears to have association with the prognostic factor measured by the SK score.

From Diagnosis to Treatment of Pediatric Tuberculosis: Ten Years Experience in a Single Institution.

Aim and Objectives. To describe the patient characteristics, clinical-epidemiologic-microbiologic profile, and treatment outcomes, and to draw attention to tuberculosis (TB) in Turkey. Methods. A retrospective, descriptive study was undertaken of 93 children aged 0 to 18 years who were admitted to Hacettepe University Pediatric Pulmonology Department for treatment from January 2005 to December 2015. Review of hospital records was performed for all children diagnosed as having TB. Results. Pulmonary TB was detected in 51.6% of the patients, extrapulmonary involvement in 33.3%, and pulmonary TB with extrapulmonary involvement in 15.1%. The history of contact with an adult with TB was found in 29% of cases. The most common extrapulmonary TB was TB lymphadenitis. The most common symptom was cough followed by fever and night sweats. One fifth of the patients had normal physical examinations at the time of diagnosis. One fifth (20.4%) of the patients had culture, 17.2% had polymerase chain reaction, and 15% had acid-resistant bacillus positivity. In 21.4% of patients with culture growth, at least one anti-TB drug resistance was found. The mean duration of treatment of patients treated according to the national guideline was 8.6 months (range = 6-36 months). Recovery was seen in 91.4% of patients. Conclusion. The most important way to prevent childhood TB is to fight adult TB through early diagnosis and effective treatment. The presence of contact history must direct us to search for TB. While evaluating children with persisted nonspecific symptoms, TB must be kept in mind even if physical examinations are normal.

Pigeon breeder`s disease as a cause of hypersensitivity pneumonia in children.

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. 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. 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. 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.

7 Newborn screening programme with immunoreactive trypsinogen (IRT) in Turkey: evaluation of patients with high IRT scores in the newborn screening programme (NBS) referred for diagnostic testing to Hacettepe University Department of Pediatric Pulmonology

7 Newborn screening programme with immunoreactive trypsinogen (IRT) in Turkey: evaluation of patients with high IRT scores in the newborn screening programme (NBS) referred for diagnostic testing to Hacettepe University Department of Pediatric Pulmonology

Comparison of Bronchoalveolar Lavage and Sputum Microbiology in Patients with Primary Ciliary Dyskinesia

Chronic infections of primary ciliary dyskinesia (PCD) airways are strongly associated with morbidity and mortality; however, relatively limited data exist about their microbiology. Therefore, this study was aimed to determine whether there was a correlation between sputum and bronchoalveolar lavage (BAL) microbiology in PCD cases. This was accomplished by comparing the lower airway bacteriology in PCD with the results of sputum samples. We retrospectively analyzed the microbiology of BAL and sputum samples of 114 patients who were diagnosed as PCD and followed up at Hacettepe University Hospital Department of Pediatric Pulmonology. At the time of sampling, patients had no pulmonary exacerbation, and sputum samples were obtained within 3 months of BAL. The mean age of the patients was 13.5 ± 4.3 (range 3–21 years) years. Microbiological analysis revealed microorganisms in 47.4% of sputum samples and in 63.2% of BAL samples. In both BAL and sputum samples, Haemophilus influenzae was the most frequent patho...

The success of the different eradication therapy regimens for Pseudomonas aeruginosa in cystic fibrosis.

Antibiotic therapy aimed at eradicating Pseudomonas aeruginosa (Pa), and improved regimens to treat chronic Pa infection have played a major role in increasing the median survival of patients with cystic fibrosis (CF). However, different clinical centres use varying eradication regimens. The aim of this study was to evaluate the efficacy of multiple eradication treatments against initial Pa infection and to determine the factors affecting the treatment success. This study was conducted at the Hacettepe University Department of Pediatric Pulmonology. We examined the demographic, clinical and microbiological data of 146 CF patients with first Pa isolation in sputum culture from all 630 patients with CF studied. We aimed to identify the factors that affected the eradication of Pa infection and assessed the success rates of the different eradication protocols used. The mean age of the patients was 71·5 months (2 months-29 years) when Pa was first isolated; the mean duration from CF diagnosis to first Pa isolation was 40 months. The most common treatment choices consisted of 2 weeks of intravenous ceftazidim-amikacin for severe exacerbation or 3 months of inhaled gentamycin combined with 3 weeks of oral ciprofloxacin for mild exacerbation in asymptomatic patients. With these treatment regimens, eradication was observed in 47 patients (32%), intermittent colonization in 42 patients (28%) and chronic colonization in 57 patients (40%). Forced expiratory volume in 1 s decline was statistically significant in patients with chronic colonization (P = 0·006). Being older than 2 years of age or having symptoms at the first Pa isolation was negatively associated with the treatment success. Early antibiotic treatment for Pa can eradicate the bacteria, prevent or delay the development of chronic colonization and improve the general health status. The acquisition of Pa at an older age and having symptoms at first isolation negatively affected the success of eradication. The use of intravenous antibiotics may increase the efficacy of therapy. Inhaled tobramycin for Pa eradication was approved for reimbursement in Turkey from August 2014. The relatively low eradication rate may be explained by a lack of reimbursement for inhaled tobramycin and colistin in our country during the study period.

An 18-year-old man with recurrent pneumothorax since he was 10-year-old

An 18-year-old male patient was referred to the department of pediatric pulmonology with a history of recurrent pneumothorax. Initial pneumothorax occurred at the age of 10. Following diagnosis of congenital lobar emphysema, he had five episodes of pneumothorax and subsequently underwent right-lower lobe anterobasal segmentectomy. Based on thoracic computed tomography (CT) and clinical manifestation, Birt-Hogg-Dube (BHD) syndrome was suspected and confirmed following genetic testing. BHD syndrome is a rare tumor predisposition syndrome first described in 1977. The syndrome is characterized by skin fibrofolliculomas, lung cysts, recurrent spontaneous pneumothorax, and renal cell cancer. The underlying cause is a germline mutation in the folliculin (FLCN) gene located on chromosome 17p11.2. Clinical manifestation usually appears after the age of 20 years. In this case, we report a case of BHD with episodes of recurrent pneumothorax, the first of which occurred at the age of 10 years. Pulmonologists should be aware of this syndrome in patients with a personal and family history of pneumothoraces and CT findings of multiple pulmonary cysts as additional evaluation and testing may be warranted. Pediatr Pulmonol. 2016;51:E41-E43. © 2016 Wiley Periodicals, Inc.

169 Improved lung function and hepatosteatosis after oral choline substitution in CF patients

intestinal malabsorption of both fat-soluble vitamins and nutrients. In this cross-sectional study, we tried to evaluate the nutritional status and determine the prevelance of fat-soluble vitamin deficiency in patients with cystic fibrosis (CF) in Turkey. Methods: We retrospectively analysed the data of fat-soluble vitamin levels, microbiological status, lung function tests and nutritional status of patients with CF who attend to Hacettepe University Department of Pediatric Pulmonology and Gastroenterology within three months. Results: We evaluated 130 patients; the mean age of the patients was 11.9±6.7 years and the mean duration of following time was 8.7 years. According to WHO BMI Z score, 10.7% of patients had malnutrition. The mean FEV1 was 75±26% and the mean FVC was 75±22%. Staphyloccus aureus was the most common microorganism isolated from sputum culture. Pancreatic insufficiency was detected in 99% of patients. Vitamin A and D deficiencies were the most common findings with 26% and 32% respectively in our cohort. In addition to multivitamin complex, 20% of patients were receiving extra vitamin A and 22.3% of patients were receiving extra vitamin E supplement. Conclusion: Fat-soluble vitamin testing and assessment of nutritional status are essential to identify deficiency in pancreatic-insufficient children who may be noncompliant to supplementation or require a higher supplement dose and nutritional support.

The Effect of Vitamin D Levels on Pediatric Allergic Diseases: A Nationwide Population-Based Study

Hea-Kyoung Yang, Jaehee Choi, Woo Kyung Kim, So-Yeon Lee, MD, Yong Mean Park, Man-Yong Han, MD, Myung-Il Hahm, Yoomi Chae, Hye-young Kim, Kang Mo Ahn, MD, HoJang Kwon, MD, PhD, Jihyun Kim, MD; Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Environmental Health Center for Atopic Diseases, Samsung Medical Center, Seoul, Korea, Environmental Health Center for Atopic Diseases, Department of Pediatrics, Samyook Medical Center, Seoul, South Korea, Department of Pediatrics, Inje University College of Medicine, Seoul, Korea, Department of Pediatrics, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Department of Pediatrics, Hallym University Secred Heart Hospital, Anyang, South Korea, Kunkuk University Hospital, South Korea, CHA University Bundang Medical Center, Seongnam, South Korea, Department of Health Administration and Management, College of Medical Science, Soonchunhyang University, Asan, Korea, Department of Occupational and Environmental Medicine, College of Medicine, Dankook University, Department of Pediatrics, Pusan National University School of Medicine, Busan, Korea, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea, South Korea, Preventive Medicine, Dankook University College of Medicine, Cheonan, South Korea, Dankook University, Cheonan, Environmental Health Center for Atopic Diseases, Seoul, South Korea, Department of Pediatrics, SamsungMedical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea. RATIONALE: We aimed to investigate the association between concentration of serum vitamin D levels and recent symptoms of allergic diseases in elementary schoolchildren. METHODS: A nationwide cross-sectional survey was conducted in the first grade students from randomly selected 45 elementary schools. Recent symptoms of atopic dermatitis (AD) or asthma were defined as those who were medically treated for AD or asthma. Recent symptoms of allergic rhinitis (AR) were considered to be present if a child had the experience of treatment for AR during the last 12 months and showed a positive skin prick test responses to common 18 inhalant allergens. All the children were examined by a pediatrician to determine the presence of eczema in their neck and flexural areas of both arms. Serum 25-hydroxyvitaminD [25(OH) D] was measured by chemiluminescent microparticle immunoassay. RESULTS: The overall prevalence rates of vitamin D insufficiency (20 to 29 ng/mL) and deficiency (<20 ng/mL) were 64.1% and 18.4%. The adjusted odds ratio (aOR) for the presence of AR was 0.980 (95% CI: 0.962–0.998) for each increase of 1 ng/mL in 25(OH)D, while no association was found between 25(OH)D levels and the prevalence of AD or asthma. The aOR per increase of 1 ng/mL in 25(OH)D was 0.974 (95% CI 0.955-0.994) for the presence of eczema on the day of survey. CONCLUSIONS: A high prevalence of vitamin D deficiency and insufficiency was found in Korean elementary schoolchildren, and vitamin D level was associated with recent symptoms of AD and AR. 186 Clinical and Epidemiological Differences in Patients with Acute Urticaria and Chronic Urticaria Mehtap Haktanir Abul, MD, Fazil Orhan, Zekiye Ilke Kilic Topcu, Taner Karakas, Ali Baki; Karadeniz Technical University Faculty of Medicine Department of Pediatric Immunology and Allergy, Trabzon, Turkey, Karadeniz Technical University Faculty of Medicine Department of Pediatric Immunology and Allergy, Karadeniz Technical University Faculty of Medicine Department of Pediatric Pulmonology. RATIONALE: Urticaria is commonly seen and it is difficult to determine the etiologic factors in children. The aim of the study is to define different clinical patterns of urticaria in childhood. METHODS: This is a prospective study included the children who were referred to our clinic for evaluation of urticaria. Laboratory tests including serologic, autoimmune, and allergic analyses were conducted. RESULTS: One hundred and eighty two children (90 females and 92 males) were evaluated. The age range was 4 month17 years old. Of the cases, 58.2 % (n5106) were diagnosed with acute urticaria and 41.8 % (n576) were diagnosed with chronic urticaria. There wasn’t any significant difference in gender between acute and chronic urticaria group. The median agewas 5 years (range: 4month16 years old) and 6.5 years (range: 7 month17 years old) for acute and chronic urticaria groups respectively. CRP, IgE, eosinophil, C3 and C4 levels are higher in acute urticaria than chronic urticaria patients that were not statistically significant. Physical urticaria was commonly seen in children with chronic urticaria (48.7%). Spontaneous resolution is more frequently seen in acute urticaria patients than the chronic urticaria patients (p50.016). CONCLUSIONS: There are clinical and epidemiological differences in patients with acute urticaria and patients with chronic urticaria. Recurrence, spontaneous resolution seems to be the factors associated with the differences in both group of patients. Physical urticaria is commonly seen and should be questioned in childrenwith chronic urticaria.

Long term azithromycin therapy in patients with cystic fibrosis.

Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF. Twenty four patients with CF aged 4-23 years followed at Hacettepe University Department of Pediatric Pulmonology between May 2007- December 2014 enrolled in the study from 630 CF patients. They received 10 mg/kg/day of AZM three times a week. Pulmonary function parameters, sputum cultures, body mass index (BMI) Z scores and number of pulmonary exacerbations were analyzed at different time intervals (at the visits at months 6, 9 and 12). Median age of the patients was 14.7 (range 4-23 years) years and median treatment duration was 14 months (range 6-60 months). Initially, median FEV1% was found 68% (range 30%-100%), BMI was found 17.05 (range 13.3-26.5) and oxygen saturation was found 95% (range 84%-99%). At the end of the 6th, 9th and 12th months of the AZM therapy; no significant differences in FEV1% and oxygen saturation parameters were found compared to the initial time, however BMI increased significantly (p=0.03), also the number of pulmonary exacerbations (p < 0.001) and severe exacerbations (p < 0.001) needing intravenous antibiotic treatment were significantly reduced at the 6th and 12th month. At the end of the 12th month of AZM; Methicillin sensitive S. aureus (MSSA) colonization was significantly increased (p=0.005) and increased macrolide resistance was detected (p=0.008). Although, this study could not be designed as a placebo controlled study, the results showed that at least 6 months of AZM treatment led to a significiant reduction in the number of pulmonary exacerbations requiring antibiotics and improvement on nutritional status. Despite increased P. aeruginosa antibiotic resistance and MSSA colonization rates, the lower incidence of acute exacerbations in patients receiving AZM is an important and clinically relevant measure of beneficial effect. Therefore, long term use of AZM may be considered to slow pulmonary deterioration in CF patients with P. aeruginosa colonization.