Pediatric Allergy Immunology and Pulmonology最新文献

Background: Increased lactate concentrations are directly related to the severity of shock and mortality rates. There are limited data regarding the prognostic value of lactate among lower respiratory tract infections. We aimed to investigate the impact of lactate levels on admission on the clinical outcomes of children with lower respiratory tract infections. Methods: We performed a retrospective study of hospitalized patients aged 1-12 months. We recorded data on patient demographics, clinical, laboratory, treatment, and outcomes. The primary outcome measure was the length of hospital stay, and the secondary outcome was transfer to the pediatric intensive care unit (PICU) and/or mortality rates. Results: A total of 304 infants were included in the study. There were 198 infants with lactate levels of >2 mmol/L. Lactic acidosis was present in 6 infants, with a mean hospital stay of 8 ± 3 days. Only 1 (0.3%) patient required intubation, and 5 (1.6%) were transferred to the PICU. The overall mortality rate was 0%. Lactate levels (≤2 and >2 mmol/L) were not related to the length of hospital stay, transfer to PICU/discharge, and the need for intubation (P = 0.16, 0.8, and 0.46, respectively). The length of hospital stay was not correlated with lactate levels on admission (r = 0.01, P = 0.84), pCO₂ (r = 0.03, P = 0.52), pH (r = 0.07, P = 0.19), C-reactive protein (r = 0.06, P = 0.28), and oxygen saturation (r = -0.02, P = 0.72). Conclusions: Lactate levels on admission did not predict the length of hospital stay in children with lower respiratory infections and were not related to the need for transfer to the intensive care unit. We suggest using lactate levels in combination with clinical, laboratory, and physical examination findings as predictors of disease severity.

Background: Tobacco-smoking in children is one of the most crucial public health concerns, which could be highly prevalent in underprivileged populations. Methods: A cross sectional, random sampling survey was conducted to determine the prevalence of current tobacco-smoking and related risk factors among adolescents living in a low-income area of Santiago de Chile. Results: Of the 2,747 adolescents participating in the study, 24.0% [95% confidence interval (CI) 22.3-25.5] were current smokers, with no significant difference between girls and boys. Factors associated with current smoking were as follows: positive attitude to smoking cigarettes offered by peers [odds ratio (OR) 8.0; 95% CI 5.7-11.3, P < 0.001], having smoking best friends (OR 4.0; 95% CI 2.6-6.0, P < 0.001), and passive smoking in the house (OR 1.9; 95% CI 1.2-3.1, P = 0.008). A total of 16.8% (95% CI 11.4-18.2) of children had smoked an entire cigarette at the age of ≤12, and 62.3% (95% CI, 60.5-64.1) were passive smokers at home. Regarding nonsmoking children, 52.4% (95% CI 49.74-55.06) were exposed to tobacco smoke at home. Conclusion: The prevalence of current tobacco-smoking in adolescents is high and increasing compared to previous studies undertaken in the selected low-resourced area. Two-thirds of children who started smoking before the age of 12 years were current smokers at the time of the survey, highlighting the prevalence of the powerful addiction generated and the need for better preventive strategies against tobacco-smoking for children living in low-resource communities.

Introduction: Hyperimmunoglobulin E syndromes (HIESs) are characterized by a high serum immunoglobulin E (IgE) level, eczematoid rashes, recurrent staphylococcal skin abscesses, and recurrent pneumonia and pneumatocele formation. Autosomal dominant HIES is the most common form of HIES and mainly occurs due to loss-of-function mutations in the Signal Transducer and Activator of Transcription 3 (STAT3) gene (STAT3 LOF). Case Presentation: We report the case of an 11-year-old Peruvian girl diagnosed with STAT3 LOF caused by p.R382W mutation. She presented with recurrent staphylococcal pneumonia and empyema caused by the rarely reported Achromobacter xylosoxidans, which led to severe destruction of the lung parenchyma, multiple lung surgeries, and the development of bronchopleural fistulas. A laparotomy was also performed, which showed evidence of sigmoid colon perforation. The patient received immunoglobulin replacement therapy (IRT) and antibiotic prophylaxis, and the frequency of her infections has decreased over the past 3 years. Conclusion: This is the first case of STAT3 LOF diagnosed by genomic sequencing in Peru. Patients with this mutation have recurrent pulmonary infections, and require multiple surgical procedures with frequent complications. A. xylosoxidans infection could be related to the prolonged stay in intensive care leading to high mortality; therefore, additional care must be taken when treating patients with this infection. In addition, colonic perforation is a rare complication in STAT3 LOF patients. IRT and antibiotic prophylaxis appear to decrease the frequency of infections and hospitalizations.

Background: To determine the lung function of children admitted to the intensive care unit (ICU) for a severe asthma exacerbation in the medium- to long-term following hospital discharge. Methods: We performed a retrospective chart review of children ≥6 years of age admitted to the ICU for a severe asthma exacerbation at a tertiary care center from January 1, 2000, to December 31, 2013. Lung function was ascertained during outpatient follow-up visits at 3-12 months and 12-24 months postdischarge. A total of 72 subjects met the inclusion criteria. Results: Subjects were predominantly boys (56.9%) and had a mean (standard deviation [SD]) age at admission of 10.3 years (3.4 years). The median (interquartile range) length of stay in the ICU was 1 day (1-3 days). Thirty-eight and 28 subjects performed pulmonary function tests with acceptable technique at the 3-12 months and 12-24 months postdischarge visits, respectively. At 3-12 months, the mean (SD) predicted forced expiratory volume in 1 s (FEV₁) and forced expiratory flow between 25% and 75% of vital capacity (FEF_25-75) percent were 95.9 (16.7) and 76.7 (25.8), respectively, and 97.4 (17.6) and 70.5 (24.9), respectively, at 12-24 months. FEV₁/forced vital capacity (FEV₁/FVC) was 81.7 (8.3) at 3-12 months and 79.3 (7.7) at 12-24 months. A paired t-test on 20 subjects who performed acceptable spirometry at both visits showed a significant intraindividual decrease in FEV₁ (P = 0.008), FEF_25-75 (P = 0.02), and FEV₁/FVC (P = 0.01) between the 2 time points. Conclusion: Although prospective studies are required to confirm our findings, our study suggests that children admitted to the ICU for severe asthma exacerbations may be at risk for declining pulmonary function in the medium- to long-term postdischarge.

Background: Interstitial lung disease (ILD) has been recently reported in a few patients with pathogenic variants in the Filamin A (FLNA) gene with variable presentation and prognosis. This study evaluated the respiratory manifestations and clinical features in children with FLNA disease. Methods: We conducted a retrospective review of pediatric patients with variants in FLNA in a tertiary children's hospital. The clinical features, genotype, management, and outcomes were analyzed. Results: We identified 9 patients with variants in FLNA aged 15 months to 24 years, 4 females and 5 males. Six patients had abnormal chest imaging ranging from mild interstitial prominence to atelectasis, interstitial densities, and hyperinflation. Three patients with ILD presented during the neonatal period or early infancy with respiratory distress or respiratory failure requiring supplemental oxygen or assisted ventilation via tracheostomy. We report male twins with the same FLNA variant and lung disease, but different ages and clinical features at presentation eventually culminating in respiratory failure requiring assisted ventilation. All patients had FLNA variants identified by FLNA sequencing, had abnormal echocardiograms, and none of the patients underwent lung biopsy or lung transplantation. The outcomes were variable and could be as severe as chronic respiratory failure. Conclusion: The wide spectrum of respiratory manifestations and abnormal chest imaging in our study highlights the importance of evaluation for lung disease in patients with variants in FLNA. FLNA sequencing in suspected cases with ILD may obviate the need for a lung biopsy, prompt surveillance for progressive lung disease, and evaluation for associated clinical features.

Background: Although the impulse oscillometry system (IOS) is a noninvasive, easily accessible, well-tolerated, and alternative test, routine use of IOS in cystic fibrosis (CF) patients is not widespread. In our unit, IOS is routinely used for the evaluation and follow-up of patients with CF. We aimed to show that IOS may be utilized as a complement for measuring pulmonary function in CF patients. Materials and Methods: Retrospective data collection and analysis of pulmonary function tests on CF patients followed at our center between January 1, 2018 and February 1, 2019. IOS and spirometry data were compared as correlated with patients' clinical exacerbation, treatment response, bronchodilator response, and trends during follow-up intervals. Results: There was a significant correlation between spirometry and IOS parameters in 70 patients. In exacerbation, Z5, R5-R10, AX, Fres, and delta R5-R20 were significantly increased and X5-X20 was significantly decreased compared with baseline in 25 patients. After treatment, IOS parameters were observed to return to baseline values. In the evaluation of bronchodilator response in 33 patients, significant changes in IOS (decrease in Z5, R5-R10, AX, Fres, and delta R5-R20, and increase in X5-X10) and in spirometry [increase in forced expiratory volume in 1 s (FEV1) and forced expiratory flow during the middle half of forced vital capacity (FEF_25-75)] were found after bronchodilator. While there was no significant difference between spirometry values in follow-up visits in 31 patients, there was a significant increase in Z5% and R5%-R20%. Unlike other studies, there was a significant correlation between clinical scores and IOS. Conclusions: These results show that although IOS is not the gold standard method such as spirometry, it is an alternative method that can be used successfully in the evaluation and follow-up of CF patients. Clinical Trials.gov ID: 99166796-050.06.04.

Introduction: In 2019, an alarming number of cases coined as e-cigarette, or vaping, product use-associated lung injury (EVALI) were described in adolescents ranging from mild respiratory distress to fulminant respiratory failure. Limited data have been published on outcomes at short-term follow-up. We aimed to describe pulmonary manifestations, function, and radiologic findings after corticosteroid therapy in a cohort of adolescent patients. Methods: A retrospective chart analysis of all patients presenting to our institution between July 2019 and December 2019 with EVALI was conducted. Patients who had pulmonary follow-up were included. Spirometry was performed before discharge from the hospital and during outpatient follow-up. A paired t-test was used to compare serial spirometry data between visits. Results: Eight patients (6 males) were included. Two patients required intubation with mechanical ventilation, 2 required bilevel positive airway pressure, and 3 required oxygen supplementation. All patients received glucocorticoids (3 receiving pulse dosing). Initial spirometry revealed decreased forced expiratory volume in one second and forced vital capacity with clinically and statistically significant improvement at follow-up (mean follow-up was 46.5 days). Radiographic manifestations also improved after vaping was discontinued. Conclusion: In our cohort of patients with EVALI, at short-term follow-up, all normalized their spirometry parameters and showed clinical resolution of symptoms.

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.