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The immunopathology of respiratory syncytial virus (RSV) infection, the most common cause of lower respiratory tract infections (LRTI) in the pediatric population, with severe disease being the exception. The variability of the clinical... more
The immunopathology of respiratory syncytial virus (RSV) infection, the most common cause of lower respiratory tract infections (LRTI) in the pediatric population, with severe disease being the exception. The variability of the clinical presentation is incompletely explained by host, viral and environmental factors but, in infants and young children, disease severity is certainly linked to the physiological immune immaturity. There is evidence that the maturation of the host immune response is, at least in part, promoted by the composition of the nasopharyngeal microbiome that, modulating excessive inflammation, can counteract the predisposition to develop viral respiratory infections and lower the risk of disease severity. However, interaction between the nasopharyngeal microbiota and respiratory viruses can be bidirectional. Microbial dysbiosis can drive disease pathogenesis but may also represents a reflection of the disease-induced alterations of the local milieu. Moreover, viru...
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An 11-month-old healthy infant girl was noted to have left lower lobe (LLL) opacities on chest X-ray (CXR) after developing desaturations during an elective cochlear implant surgery. Repeat CXR 10 days later revealed hyperexpansion of the... more
An 11-month-old healthy infant girl was noted to have left lower lobe (LLL) opacities on chest X-ray (CXR) after developing desaturations during an elective cochlear implant surgery. Repeat CXR 10 days later revealed hyperexpansion of the left lung and persistent LLL opacity. Chest computerized tomography revealed enlarged mediastinal lymph nodes, left mainstem bronchial obstruction, and nodular opacities. Bronchoscopic biopsy of the endobronchial tissue revealed multiple necrotizing granulomas and grew,, and. This case illustrates the potential for significant mediastinal lymphadenopathy, and endobronchial and parenchymal lesions caused by nontuberculous mycobacteria, which can present insidiously and without respiratory symptoms in otherwise healthy infants, despite advanced pulmonary disease.
Increased incidence and prevalence of asthma have been documented for perinatally HIV-infected youth 10 to 21 years of age compared with HIV-exposed uninfected (HEU) youth. We sought to perform objective pulmonary function tests (PFTs) in... more
Increased incidence and prevalence of asthma have been documented for perinatally HIV-infected youth 10 to 21 years of age compared with HIV-exposed uninfected (HEU) youth. We sought to perform objective pulmonary function tests (PFTs) in HIV-infected and HEU youth with and without diagnosed asthma. Asthma was determined in 370 participants (218 HIV-infected and 152 HEU participants) by means of chart review and self-report at 13 sites. Interpretable PFTs (188 HIV-infected and 132 HEU participants) were classified as obstructive, restrictive, or normal, and reversibility was determined after bronchodilator inhalation. Values for HIV-1 RNA, CD4 and CD8 T cells, eosinophils, total IgE, allergen-specific IgE, and urinary cotinine were measured. Adjusted prevalence ratios (PRs) of asthma and PFT outcomes were determined for HIV-infected participants relative to HEU participants, controlling for age, race/ethnicity, and sex. Current asthma was identified in 75 (34%) of 218 HIV-infected p...
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Rossi GA, Colin AA(.) Respiratory syncytial virus - host interaction in the pathogenesis of bronchiolitis and its impact on respiratory morbidity in later life. Pediatr Allergy Immunol. Respiratory syncytial virus (RSV) is the most common... more
Rossi GA, Colin AA(.) Respiratory syncytial virus - host interaction in the pathogenesis of bronchiolitis and its impact on respiratory morbidity in later life. Pediatr Allergy Immunol. Respiratory syncytial virus (RSV) is the most common agent of severe airway disease in infants and young children. Large epidemiologic studies have demonstrated a clear relationship between RSV infection and subsequent recurrent wheezing and asthma into childhood, thought to be predominantly related to long-term changes in neuroimmune control of airway tone rather than to allergic sensitization. These changes appear to be governed by the severity of the first RSV infection in infancy which in term depends on viral characteristics and load, but perhaps as importantly, on the genetic susceptibility and on the constitutional characteristic of the host. A variety of viral and host factors and their interplay modify the efficiency of the response to infection, including viral replication and the magnitude...
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Nitric oxide (NO) that is produced within the airways can be measured in the exhaled air. Concentrations of exhaled NO (FENO) are decreased in cystic fibrosis (CF) and, in cross sectional studies, have been shown to be even lower in... more
Nitric oxide (NO) that is produced within the airways can be measured in the exhaled air. Concentrations of exhaled NO (FENO) are decreased in cystic fibrosis (CF) and, in cross sectional studies, have been shown to be even lower in patients with more advanced pulmonary disease. This may result from retention and metabolisation of NO within viscous airway secretions. Treatment with recombinant human DNase I (dornase alpha) modifies the rheological properties of airway secretions and thereby improves pulmonary function even in young and apparently healthy patients with CF. We studied FENO and pulmonary function in children with CF with little clinical evidence of lung morbidity in a two-year randomized double-blind placebo-controlled study with nebulized dornase alpha. Mean age at enrollment was 8 years (range 6 to 11 years), mean forced vital capacity (FVC) was 112% (range 86 to 133%), and mean forced expiratory volume in one second (FEV1)was 109% (range 88 to 128%) of predicted values. In five of six (83%) of the dornase alpha treated patients, FENO changed in parallel to changes in pulmonary function tests while no such correlation was observed in any of the eight patients receiving placebo. This difference between treatment groups was statistically significant for both FVC (P = 0.026, Wilcoxon-test) and FEV1 (P = 0.042). These data suggest that FENO may serve as a surrogate measure for evaluating the effectiveness of interventions that affect airway clearance in CF.
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The prevalence of exercise-induced asthma was studied in 313 Haifa schoolchildren, 160 living in an unpolluted urban area close to the sea and 153 living in a rural kibbutz (communal agricultural settlement) in the proximity of polluting... more
The prevalence of exercise-induced asthma was studied in 313 Haifa schoolchildren, 160 living in an unpolluted urban area close to the sea and 153 living in a rural kibbutz (communal agricultural settlement) in the proximity of polluting industries. The overall prevalence was 8.6%; it was higher in the kibbutz group, but the difference was not significant. The prevalence tended to fall with increasing age and did not change with sex.
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MicroRNAs (miRNAs) are small single-stranded RNA molecules involved in the regulation of gene expression at the post-transcriptional level. In the airways, miRNAs are implicated in the modulation of antiviral defense, through modulation... more
MicroRNAs (miRNAs) are small single-stranded RNA molecules involved in the regulation of gene expression at the post-transcriptional level. In the airways, miRNAs are implicated in the modulation of antiviral defense, through modulation of both innate and adaptive immune response in inflammatory and immune effector cells but also in parenchymal cells. The first target of respiratory viruses are airway epithelial cells. Following infection, an altered expression of distinct miRNAs occurs in airway cells aimed at inhibiting viral replication and preserving the airway epithelial barrier, while at the same time viruses induce or repress the expression of other miRNAs that favor viral replication. Understanding the changes in miRNA expression profile, identification of miRNAs target genes and their contribution to the pathogenesis of the disease may help the intricate mechanisms of virus-host interaction. Further understanding of these molecular mechanisms could lead to development of ne...
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High doses of ibuprofen have been shown to delay the progression of lung disease without serious adverse effects in patients with cystic fibrosis. To be effective, peak ibuprofen concentration of 50 to 100 mg/L has to be achieved. We... more
High doses of ibuprofen have been shown to delay the progression of lung disease without serious adverse effects in patients with cystic fibrosis. To be effective, peak ibuprofen concentration of 50 to 100 mg/L has to be achieved. We developed an HPLC assay to rapidly determine plasma ibuprofen concentration. We used this assay to determine the pharmacokinetics of ibuprofen in patients with cystic fibrosis. The assay possessed linearity up to 500 mg/L, sensitivity to 1 mg/L, average recovery of 98%, and run-to-run precision (n = 23) of 3%. Furthermore, the assay proved to be free of interference from 51 medications. Observed time to peak concentration varied significantly between those receiving ibuprofen tablets (mean + SD, 94 +/- 29 min, n = 16) and syrup (30 +/- 0 min, n = 4) (P < 0.0001). We conclude that the method described here is ideal for therapeutic monitoring of ibuprofen.
Research Interests: Cystic Fibrosis, Treatment, Pharmacokinetics, Adolescent, Clinical Chemistry, and 15 moreHumans, Child, Medical Biotechnology, Female, Male, Infant, Clinical Sciences, Ibuprofen, Adult, Sensitivity and Specificity, Drug Stability, Child preschool, Adverse effect, Medical biochemistry and metabolomics, and HPLC Chromatography
The underlying cause of cystic fibrosis (CF) is the loss of epithelial chloride and bicarbonate transport due to mutations in the CF transmembrane conductance regulator (CFTR) gene encoding the CFTR protein. Ivacaftor is a gene-specific... more
The underlying cause of cystic fibrosis (CF) is the loss of epithelial chloride and bicarbonate transport due to mutations in the CF transmembrane conductance regulator (CFTR) gene encoding the CFTR protein. Ivacaftor is a gene-specific CFTR potentiator that augments in vivo chloride transport in CFTR mutations affecting channel gating. Originally approved for the G511D CFTR mutation, ivacaftor is now approved for eight additional alleles exhibiting gating defects and has also been tested in R117H, a CFTR mutation with residual function that exhibits abnormal gating. P67L is a class 4 conductance (nongating) mutation exhibiting residual CFTR function. We report marked clinical improvement, normalization of spirometry, and dramatic reduction in radiographic structural airway changes after > 1 year of treatment with ivacaftor in a young adult with the compound heterozygous genotype P67L/F508del CFTR. The case suggests that ivacaftor may have a potential benefit for patients with CF...
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We compare a chest radiographic scoring system developed by our group to spirometry in quantifying the longitudinal progression of lung disease among cystic fibrosis (CF) patients, and we evaluate the use of this radiographic scoring... more
We compare a chest radiographic scoring system developed by our group to spirometry in quantifying the longitudinal progression of lung disease among cystic fibrosis (CF) patients, and we evaluate the use of this radiographic scoring system in identifying the treatment effect of an inhaled antibiotic. Results suggest that longitudinally acquired chest radiographs, when scored using our scoring system, are at least as sensitive as lung function in detecting the progression of lung disease in CF patients.
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We used respiratory inductance plethysmography to record tidal respiration in 27 healthy unsedated infants and children 1 mo to 8 yr of age during sleep. Rib cage and abdominal outputs were present at approximately equal gains and summed... more
We used respiratory inductance plethysmography to record tidal respiration in 27 healthy unsedated infants and children 1 mo to 8 yr of age during sleep. Rib cage and abdominal outputs were present at approximately equal gains and summed to obtain an estimate of volume. Flow-volume curves were generated from the uncalibrated volume signal and its flow derivative. Expiratory time constants (tau) were obtained by visually drawing a line through the linear portion of the expiratory flow-volume relationship. tau increased significantly during the first 10 mo of life. After 10 mo, the estimated rate of increase of tau for older children was less than 5% of the estimated initial rate and not significantly different from zero. Prolongation of tau was paralleled by an increase in expiratory time (Te), and no changes in Te/tau were observed in the first 2 yr of life. These changes in tau likely reflect the increase in lung compliance induced by rapid alveolar growth during infancy. After the...
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As the shape, compliance, and deformability of the rib cage (RC) change during infancy, RC participation in quiet breathing may increase. We used respiratory inductive plethysmography (RIP) to determine the relative contributions of the... more
As the shape, compliance, and deformability of the rib cage (RC) change during infancy, RC participation in quiet breathing may increase. We used respiratory inductive plethysmography (RIP) to determine the relative contributions of the RC and abdomen (AB) to tidal volume (VT) in 20 studies in 14 healthy infants 1 to 26 months of age during quiet natural sleep. RIP was calibrated with simultaneous flow measurements (anesthesia mask and pneumotachograph) by the least squares method of statistical analysis. We analyzed segments of breathing with and without flow measurement for RIP-derived VT, change in RC volume (Vrc) and AB volume (Vab) with each breath and the RC contribution to tidal breathing (%RC = Vrc/Vrc + Vab). The %RC increased with age: %RC = 1.4 age (months) + 33 (r = 0.69, p less than 0.01). After 9 months of age, %RC resembled that found in quietly sleeping adolescents. Mask placement increased VT in all but one subject (mean increase, 29 +/- 23% of baseline +/- SD; p le...
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Nontuberculous mycobacteria (NTM) have been increasingly recognized in recent years as contributors to clinically significant lung infection in cystic fibrosis (CF) patients. Reports of prevalence vary from 6 to 22% around the world.... more
Nontuberculous mycobacteria (NTM) have been increasingly recognized in recent years as contributors to clinically significant lung infection in cystic fibrosis (CF) patients. Reports of prevalence vary from 6 to 22% around the world. Prevalence estimates in childhood CF is challenging since sputum producers are rare, bronchoalveolar lavage is an invasive procedure and may not be feasible, and mounting evidence deeming throat cultures to be unsuccessful in detecting NTM. We report a case of an overall healthy 13 year old young adolescent female with CF, who presented with Mycobacterium avium complex (MAC) as her first documented lung infection, and while only presenting with minor cough proved to have severe purulent bronchial infection. Contrary to common paradigms, NTM can be the first infection in a non-previously infected airway, and, this first infection can be of a serious nature unlike the more subtle anticipated pattern. The causes of the high and probably increasing overall incidence of NTM and specifically in CF elude explanation and mandate further study. Continuing efforts should be invested into the study of all aspects of this ominous infection. Pediatr Pulmonol. © 2014 Wiley Periodicals, Inc.
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There is evidence that respiratory viruses play a key role in the development and exacerbation of obstructive respiratory diseases in children. This review attempts to juxtapose the separate profiles and prototypes of pathogenenetic... more
There is evidence that respiratory viruses play a key role in the development and exacerbation of obstructive respiratory diseases in children. This review attempts to juxtapose the separate profiles and prototypes of pathogenenetic mechanisms represented by the two most common amongst such viruses: respiratory syncytial virus (RSV) and human rhinovirus (HRV). RSV represents the most common agent of severe airway disease in infants and young children, and is predominant in winter months. Large epidemiological studies have revealed an unequivocal relationship between RSV infection and subsequent wheezing into childhood, thought to be related to long-term changes in neuroimmune control of the airways rather than allergic sensitisation. HRV is a highly diverse group of viruses that affect subjects of all ages, is ubiquitous and occurs year-round. In contrast to RSV, infections with HRV cause minimal cytotoxicity but induce a rapid production of cytokines and chemokines with amplificati...
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Unstimulated saliva, citric acid stimulated saliva, and serum were collected from 31 asthmatic children taking theophylline. Salivary theophylline concentrations, and total and unbound serum theophylline concentrations were measured. The... more
Unstimulated saliva, citric acid stimulated saliva, and serum were collected from 31 asthmatic children taking theophylline. Salivary theophylline concentrations, and total and unbound serum theophylline concentrations were measured. The correlation coefficients between both types of saliva, and total and unbound serum theophylline were all statistically significant (p less than 0.001). The highest correlation coefficients were obtained with stimulated saliva and total serum concentrations (r = 0.98), and stimulated saliva and unbound serum (r = 0.96). The coefficients when unstimulated saliva was compared to either total or unbound serum concentrations were 0.90 and 0.89, respectively. Serum binding of theophylline averaged 58.1%. Unstimulated saliva had a higher mean theophylline concentration than stimulated saliva. The results suggest that salivary monitoring using stimulated saliva may be used to predict serum concentrations with a high degree of confidence when the saliva levels are substantially lower than, higher than, or in the middle of the therapeutic range, but there is a considerable degree of uncertainty when the salivary values are near the lower or upper end of the therapeutic range.
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To report the performance of fetal bronchoscopy in a case of pulmonary sequestration. A 24 year-old female, Gravida 2, Para 1, was referred at 27.5 weeks with a large fetal left lung mass with marked right mediastinal shift and no visible... more
To report the performance of fetal bronchoscopy in a case of pulmonary sequestration. A 24 year-old female, Gravida 2, Para 1, was referred at 27.5 weeks with a large fetal left lung mass with marked right mediastinal shift and no visible normal left lung. Differential diagnosis included possible bronchial atresia. The patient underwent fetal laryngoscopy and fetal bronchoscopy at 31.5 weeks. The right lung and a portion of the left lung expanded during surgery as a result of bronchial lavage. Bronchial atresia or bronchogenic cyst were not found. Pregnancy continued uneventfully, with continuous growth of the right lung and a small amount of left lung. The patient delivered vaginally at term. The baby underwent thoracoscopic resection of a pulmonary sequestration at 10.5 months of age and did well. Fetal bronchoscopy is feasible. The procedure may prove useful in the differential diagnosis and in the potential treatment of different fetal lung lesions, as well as aid in the understanding of the role of bronchial obstruction as a common pathophysiologic mechanism for different fetal lung masses. Risks and benefits of fetal bronchoscopy warrant further experience.
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To assess the rate of decline in pulmonary function in Duchenne muscular dystrophy (DMD) before and after posterior spinal fusion for scoliosis. To compare the rate of respiratory decline using percent normal forced vital capacity (%FVC)... more
To assess the rate of decline in pulmonary function in Duchenne muscular dystrophy (DMD) before and after posterior spinal fusion for scoliosis. To compare the rate of respiratory decline using percent normal forced vital capacity (%FVC) measurements before and after posterior spinal fusion. Posterior spinal fusion for scoliosis is used widely in DMD, although the long-term pulmonary effects have not been well established. Fifty-six patients were assessed. Percent forced vital capacity was the outcome parameter with data analysis using a mixed-model repeated-measures ANOVA and paired t tests. Group 1: Inclusion criteria were a diagnosis of DMD, 2 or more pulmonary function tests presurgery, and 2 or more postsurgery. Group 2: The rates of respiratory decline before and after spinal fusion for the whole study population were determined by within-subjects mixed-model regression analysis to account for the varying number of FVC studies between patients and unequal spacing between tests. Group 1: 20 patients. Mean length of time of respiratory value determination was 2.5 +/- 1.0 years presurgery and 5.6 +/- 2.8 years postsurgery. Mean rate of decline presurgery was 8.0% +/- 4.1% per year, which decreased to 3.9% +/- 1.9% per year postsurgery (paired t test = 4.58, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). Group 2: 56 patients. The respiratory value determinations ranged from 4 years presurgery to 8 years postsurgery. The rates of respiratory decline based on the whole study population were 4% per year presurgery, which decreased to 1.75% per year postsurgery (F-test comparison of slopes = 19.71, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001). Posterior spinal fusion for scoliosis in DMD is associated with a significant decrease in the rate of respiratory decline postsurgery compared with presurgery rates.
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Late preterm (LP) infants are defined as those born at 34-0/7 to 36-6/7 weeks&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestational age. LP infants were previously... more
Late preterm (LP) infants are defined as those born at 34-0/7 to 36-6/7 weeks&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; gestational age. LP infants were previously referred to as near term infants. The change in terminology resulted from the understanding that these infants are not fully mature and that the last 6 weeks of gestation represent a critical period of growth and development of the fetal brain and lungs, and of other systems. There is accumulating evidence of higher risks for health complications in these infants, including serious morbidity and a threefold higher infant mortality rate compared with term infants. This information is of critical importance because of its scientific merits and practical implications. However, it warrants a critical and balanced review, given the apparent overall uncomplicated outcome for the majority of LP infants. Others reviewed the characteristics of LP infants that predispose them to a higher risk of morbidity at the neonatal period. This review focuses on the long-term neurodevelopmental and respiratory outcomes, with the main aim to suggest putative prenatal, neonatal, developmental, and environmental causes for these increased morbidities. It demonstrates parallelism in the trajectories of pulmonary and neurologic development and evolution as a model for fetal and neonatal maturation. These may suggest the critical developmental time period as the common pathway that leads to the outcomes. Disruption in this pathway with potential long-term consequences in both systems may occur if the intrauterine milieu is disturbed. Finally, the review addresses the practical implications on perinatal and neonatal care during infancy and childhood.
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To identify the causes of mortality in children with vertically transmitted human immunodeficiency virus (HIV) infection and to study age-related mortality trends. In the multicenter P(2)C(2) HIV Study, 816 children born to HIV-infected... more
To identify the causes of mortality in children with vertically transmitted human immunodeficiency virus (HIV) infection and to study age-related mortality trends. In the multicenter P(2)C(2) HIV Study, 816 children born to HIV-infected mothers were followed for a median of 3.6 years. Two hundred five study participants with HIV infection were enrolled at a median age of 23 months; 611 were enrolled either prenatally or in the neonatal period before their HIV infection status was known. There were 121 deaths in study patients. The cause of death for all patients, its relationship to HIV infection, and pulmonary or cardiac involvement were determined. Age trends in disease-specific mortality were summarized for the HIV-related deaths. Ninety-three children died of HIV-related conditions. Infection was the most prevalent cause of death for children under 6 years of age with 32.3% caused by pulmonary infection and another 16.9% caused by nonpulmonary infection. The frequency of pulmonary disease as the underlying cause of death decreased significantly with increasing age: 5/9 (55.6%) by age 1, 1/12 (8.3%) after age 10 years. The frequency of chronic cardiac disease as the underlying cause increased with age-0% by age 1 year, 3/12 (25.0%) after age 10 years, as did the frequency of wasting syndrome with disseminated Mycobacterium avium complex-0% by age 1 year, 6/12 (50.0%) after age 10 years. Children with HIV who survive longer are less likely to die of pulmonary disease or infection and more likely to die of cardiac causes or with wasting syndrome.pediatric acquired immunodeficiency syndrome, mortality, human immunodeficiency virus.
Research Interests: Pediatrics, Mortality, Humans, Child, Female, and 15 moreMale, Lung Diseases, Infant, Newborn Infant, Human immunodeficiency virus, Longitudinal Studies, Age Factors, AIDS Dementia Complex, Fetal death, Heart Diseases, Child preschool, Psychology and Cognitive Sciences, Cause of death, Medical and Health Sciences, and HIV infections
Research Interests: Pediatrics, Lung Function, Pregnancy, Humans, Female, and 15 moreMale, Lung Diseases, Incidence, Newborn Infant, Lung Development, Forced Vital Capacity, Infant Mortality, Prognosis, Airway Obstruction, Bronchopulmonary Dysplasia, Gestational Age, Functional residual capacity, Psychology and Cognitive Sciences, Cause of death, and Medical and Health Sciences
Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss... more
Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.
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This paper assesses the effectiveness of aerosolized tobramycin (TOBI) on cystic fibrosis (CF) lung disease, using a radiologic tool. The published tool, the age-based severity curve (ABS), is derived from Brasfield scoring of chest... more
This paper assesses the effectiveness of aerosolized tobramycin (TOBI) on cystic fibrosis (CF) lung disease, using a radiologic tool. The published tool, the age-based severity curve (ABS), is derived from Brasfield scoring of chest X-rays (CXR). This study evaluates both the usefulness of the ABS as an assessment tool and the effectiveness of TOBI. Thirty-eight patients were treated with TOBI. Twenty-four treated with dornase alfa were excluded. Fourteen patients, aged 2 months to 22 years (mean, 17 months of TOBI treatment), comprised the study group. Radiographs were obtained over a mean of 7.8 years (SD = 6.5 years; range, 9 months-18 years). Two hundred and eighty-two CXR of TOBI patients were analyzed following the ABS protocol. Rate of decline in radiologic status of the TOBI group and ABS were compared. Also, TOBI was assessed by comparing rate of decline before and after initiation of treatment. The TOBI group&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s radiologic assessment was compared to its rate of decline in pulmonary function studies and published population data. Rate of decline in ABS was 0.175 Brasfield points/year vs. 0.150 points/year in the TOBI group (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). Before treatment, the TOBI group&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s rate of decline was 0.169 Brasfield points/year; after treatment, it was 0.150 points/year (P = 0.02). Forced vital capacity revealed a statistically significant slowing in rate of decline on TOBI. Although not statistically significant, rate of decline in forced expiratory volume at 1 sec showed a similar trend. The degree of slowing in decline is similar to that previously reported for pulmonary function studies.
Research Interests: Pediatrics, Cystic Fibrosis, Adolescent, Aerosols, Humans, and 15 moreChild, Female, Male, Exploration, Infant, Pediatric, Pediatric Pulmonology, Radiography, Observer Variation, Adult, Retrospective Studies, Database, Child preschool, Respiratory function tests, and Paediatrics and reproductive medicine
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Our objective was to describe the respiratory complications, clinical findings, and chest radiographic changes in the first year of life in infected and uninfected children born to HIV-1-infected women. We prospectively followed a cohort... more
Our objective was to describe the respiratory complications, clinical findings, and chest radiographic changes in the first year of life in infected and uninfected children born to HIV-1-infected women. We prospectively followed a cohort of 600 infants born to HIV-1-infected women from birth to 12 months in a multicenter study. Of these, 93 infants (15.5%) were HIV-1-infected, 463 were uninfected, and 44 were of unknown status prior to death or loss to follow-up. The cumulative incidence ( +/- SE) of an initial pneumonia episode at 12 months was 24.1 +/- 4.7% in HIV-1-infected children compared to 1.4 +/- 0.6% in HIV-1-uninfected children (P &lt; 0.001). The rate of Pneumocystis carinii pneumonia (PCP) was 9.5 per 100 child-years. The HIV-1 RNA load was not higher in the group that developed pneumonia in the first year vs. those who did not. Children who developed lower respiratory tract infections or PCP had increased rates of decline of CD4 cell counts during the first 6 months of life. Lower maternal CD4 cell counts were associated with higher rates of pneumonia, and upper and lower respiratory tract infections. The rates of upper respiratory tract infection and bronchiolitis/reactive airway disease in infected children were not significantly different than in uninfected children. At 12 months, significantly more HIV-1-infected than uninfected children had tachypnea and chest radiographs with nodular and reticular densities. There was no relationship between cytomegalovirus infection in the first year of life and radiographic changes or occurrences of pneumonia. In conclusion, despite a low incidence of PCP, rates of pneumonia remain high in HIV-infected children in the first year of life. The incidence of pneumonia in uninfected infants born to HIV-1-infected mothers is low. Chest X-ray abnormalities and tachypnea suggest that subacute disease is present in infected infants. Further follow-up is warranted to determine its nature.
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The purpose of this study was to investigate how adolescents and adults with cystic fibrosis (CF) view preventive counseling and their transition to adult-centered care within a... more
The purpose of this study was to investigate how adolescents and adults with cystic fibrosis (CF) view preventive counseling and their transition to adult-centered care within a children&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s hospital. Thirty-two patients &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;/=16 years old diagnosed with CF were recruited from a pediatric tertiary care setting. During face-to-face interviews, patients were asked 27 structured questions and completed a 30-item self-administered questionnaire on preventive counseling by healthcare providers and on transition issues. The median age of patients was 25.5 years (range, 16-43 years); 69% of patients identified a pulmonologist as their &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;main doctor,&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot; even though 78% had a primary care provider. Participants felt that 13-16 years of age was the best time for them to begin spending time alone with their main doctor. Less than half of the participants recalled receiving preventive counseling during the previous 12 months, and more patients wanted to discuss issues than actually did. Qualitative data emphasized the importance of independence in making decisions in healthcare and establishing relationships with providers, and many patients did not desire to transfer care to an adult hospital. Participants identified adult-focused services such as inpatient rooms, discussion groups, work options, and social service support that would enhance care. In conclusion, the majority of adolescent/young adult patients with CF receiving care in a pediatric institution reported satisfaction with their healthcare. However, patients identified preventive issues that they desired to be more regularly addressed, starting in early adolescence, and changes in the delivery of services to enhance transition to adult-oriented care. This study underscored the understanding of the integration of transition planning into the facilitation of healthcare decision-making by the adolescent in issues of self-care, sexuality, education, and finances. Future initiatives to enhance the care of patients with CF should provide training of pulmonologists in preventive care and increased attention to helping patients utilize appropriate primary-care services during the adult years. In addition, prospective studies are needed to compare outcomes of CF patients who have transitioned and transferred to adult hospitals and those transitioning to adult-oriented services in a pediatric institution.
Research Interests: Aging, Cystic Fibrosis, Preventive medicine, Adolescent, Counseling, and 15 moreProspective studies, Humans, Needs Assessment, Female, Male, Massachusetts, Pediatric, Pediatric Pulmonology, Boston, Day Care, Adult, Pulmonary Medicine, Continuity of Patient Care, Health Care Surveys, and Paediatrics and reproductive medicine
Suppurative complications of pneumonia such as empyema, lung abscess, pyopneumothorax, and necrotizing pneumonia (NP) are uncommon in children. Over the last decade an increasing incidence of NP has been reported. Streptococcus pneumoniae... more
Suppurative complications of pneumonia such as empyema, lung abscess, pyopneumothorax, and necrotizing pneumonia (NP) are uncommon in children. Over the last decade an increasing incidence of NP has been reported. Streptococcus pneumoniae continues to be the predominant causative organism of NP, and while sporadic cases were reported prior to routine administration of heptavalent pneumococcal vaccine, a marked increase in NP appears to relate to replacement pneumococcal strains. Pleural involvement is almost universal in NP, and the course of pleural disease often determines its duration and outcome, particularly as it relates to complication of bronchopleural fistula. Cavities are formed in NP within the lung parenchyma and in the pleural space as the fibrosing pleural process organizes. The similarity of the radiologic appearance of parenchymal and pleural space cavities often makes the differentiation of pneumatocele versus residua of loculated pneumothorax challenging. The prevailing perception from most reports on childhood NP is of a favorable outcome with conservative approach. We report two pediatric cases with pneumonia who presented with prolonged fever despite antibiotic treatment, eventually diagnosed with NP. After stabilization on prolonged IV antibiotics, and weeks after discharge, they presented with unexpected acute respiratory failure due to a life-threatening tension air collection. In this article we discuss the nature of NP, its typical presentation, benign course and outcome, albeit its potential to cause serious late complications in the light of our recent experiences. Increasing awareness of such complications will result in more careful follow-up and in providing appropriate recommendations to parents of patients recovering from NP.
Research Interests:
Research Interests: Cystic Fibrosis, Adolescent, Humans, Female, Thorax, and 13 moreMale, Pediatric, Pediatric Pulmonology, Adult, Vital Capacity, Time Factors, Sensitivity and Specificity, Reference Values, Analog to Digital Conversion, Abdomen, Case Control Studies, Respiratory function tests, and Paediatrics and reproductive medicine
Three adults with cystic fibrosis (one after lung transplantation) presented with fever, chest pain, and acute radiographic changes. The changes included a cavitary lesion of the lung, acute dense infiltrates, and lobar collapse. After... more
Three adults with cystic fibrosis (one after lung transplantation) presented with fever, chest pain, and acute radiographic changes. The changes included a cavitary lesion of the lung, acute dense infiltrates, and lobar collapse. After failing conventional antibiotic therapy, the patients underwent flexible bronchoscopy. All had bronchial obstruction by a membrane that had completely occluded the bronchial orifice at the bifurcation of the bronchi. Therapeutic interventions ranged from continuing intravenous antibiotics, bronchoscopy-assisted perforation of the membrane by sharp instrumentation, and transthoracic needle-guided perforation of the membrane with subsequent stenting of the orifice. The patients recovered, but the posttransplant patient had recurrent membranous obstructions with multiple interventions. The cause and triggers of the process are unknown. Based on repeated observations of the evolution of the membranes, and histologic material from bronchoscopies, we propose a putative paradigm of the natural history of the process. We suggest that local stimuli generate a richly vascularized granulation polyp, which progresses in a &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;shutter-like&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;quot; motion to form partial or completely obstructive membranes. The subsequent course depends on the vascular supply to the membrane. We also propose that similar processes may be the underlying pathologic events in some cases of lung abscess and necrotizing pneumonia.