Background
Hypersensitivity pneumonitis (HP) is a rare interstitial lung disease in children, and very little data are available on the frequency, diagnosis, and outcomes of HP. In a pediatric cohort ...with HP, the characteristics of the CD4/CD8 lymphocyte ratio are often described as nonspecific.
Methods
We used the National French Database (RespiRare) to collect data from the last decade on HP. The diagnosis of HP was defined by the presence of a relevant exposure, clinical symptoms, and compatible lung imaging radiology and was usually defined by positive precipitins antibodies.
Results
A total of 16 children with a mean age of 10 years (4‐13) presented with HP. All children presented with dyspnea on exertion. Diffuse ground‐glass opacity was present in all computed tomography (CT) scans. Research guided by a questionnaire and precipitins antibodies against the corresponding antigens showed that patients were positive for contact with birds with or without fungi. Bronchoalveolar lavage (BAL) was performed in 12 children. The total cell counts were elevated in BAL fluid, with a mean value of 36% lymphocytes. The CD4/CD8 lymphocyte ratio was below one for all children.
Conclusion
BAL in our pediatric cohort with HP had the same characteristics as that of adults with HP. An HP diagnosis must be considered when dyspnea on exertion and diffuse ground‐glass opacity are observed. Carrying out BAL and serological tests can help diagnose and avoid lung biopsy.
Background
Beta‐lactam hypersensitivity (HS) is suspected in 5–12% of the children, but proven in only 10–15% of those children, based on skin and challenge tests results. In contrast, 30–60% of ...patients with cystic fibrosis (CF) are diagnosed allergic to beta‐lactams, based mainly on the clinical history of the patients.
Objectives
To confirm or rule out a suspected beta‐lactam HS in CF children and to determine the prevalences of suspected and confirmed beta‐lactam HS in those children.
Patients and methods
Children with CF and suspected beta‐lactam HS were explored by means of skin and challenge tests with the suspected and alternate beta‐lactams. The results in CF children were compared with those reported in the literature in non‐ CF children.
Results
Eight of the 701 CF children followed in our center between 1990 and 2011 (1.14%), and 11 other children from other centers were explored for suspected beta‐lactam HS. Beta‐lactam HS was diagnosed in nine of these children (47.3%). Based on the results in the children followed in our center, the prevalence of beta‐lactam HS was 0.71% (5/701) in CF children vs. a mean estimated prevalence of 1–1.5% in the general pediatric population.
Conclusion
Our results contrast with those of most previous studies. Although half of the CF children with suspected beta‐lactam HS were truly allergic to beta‐lactams, the general prevalence of beta‐lactam HS in CF children was very low. This may result from tolerance induced by frequent and/or prolonged treatments with beta‐lactams.
Background and objectives
Lysinuric protein intolerance (LPI) is a rare multisystemic metabolic disease. The objective of the study was to describe presentation and course of lung involvement in a ...cohort of ten children.
Patients and methods
Retrospective review of patients followed at Necker-Enfants Malades University Hospital between 1980 and 2012 for a LPI. In patients with lung involvement, clinical data, chest radiographs, pulmonary function tests, bronchoalveolar lavages, and lung biopsies were analyzed. The first and last high-resolution computed tomography (HRCT) were also reviewed.
Results
Lung involvement was observed in ten of 14 patients (71 %). Five patients had an acute onset of respiratory symptoms, three had a progressive onset and two were free of symptoms. During the period studied, six patients (60 %) died, all in a context of respiratory failure. Clinical presentation and course were highly variable, even in the same family. HRCT were performed in seven cases, showing in all cases an interstitial pattern and fibrosis in four. All ten patients had pulmonary alveolar proteinosis (PAP) confirmed by histopathological analysis. Five patients had pulmonary fibrosis (at biopsy and/or HRCT scan). Two patients underwent whole lung lavages, without efficiency.
Conclusion
PAP is a constant feature in children with LPI and lung involvement. Pulmonary fibrosis is frequent and these two pathologies may develop independently. This study shows the heterogeneity of presentation and outcome. Lung injury could be secondary to impaired phagocytic function and abnormal inflammatory and immune responses intrinsic to the
SLC7A7
mutant phenotype. HRCT is recommended to detect lung involvement.
Hymenoptera venom allergy is the second cause of anaphylaxis in European children. Systemic reactions occur in up to 0.8% of schoolchildren. Venom immunotherapy (VIT) is recommended in sensitized ...children having systemic skin reactions exceeding generalized skin symptoms. VIT carries an 8–20% risk of systemic adverse events. Short protocols achieve the maintenance dose faster than conventional protocols but are more frequently associated with anaphylactic reactions. We report the cases of teenagers who experienced severe anaphylactic reactions (SAR) during the rush phase of VIT (Table 1). After an informed consent, a second rush VIT was performed after a pretreatment with omalizumab and was tolerated. No injection of omalizumab was required during the maintenance phase.
Background
It is unclear whether sensitization patterns differentiate children with severe recurrent wheeze (SRW)/severe asthma (SA) from those with non‐severe recurrent wheeze (NSRW)/non‐severe ...asthma (NSA). Our objective was to determine whether sensitization patterns can discriminate between children from the French COBRAPed cohort with NSRW/NSA and those with SRW/SA.
Methods
IgE to 112 components (c‐sIgE) (ImmunoCAP® ISAC) were analyzed in 125 preschools (3–6 years) and 170 school‐age children (7–12 years). Supervised analyses and clustering methods were applied to identify patterns of sensitization among children with positive c‐sIgE.
Results
We observed c‐sIgE sensitization in 51% of preschool and 75% of school‐age children. Sensitization to house dust mite (HDM) components was more frequent among NSRW than SRW (53% vs. 24%, p < .01). Sensitization to non‐specific lipid transfer protein (nsLTP) components was more frequent among SA than NSA (16% vs. 4%, p < .01) and associated with an FEV1/FVC < −1.64 z‐score. Among sensitized children, seven clusters with varying patterns were identified. The two broader clusters identified in each age group were characterized by “few sensitizations, mainly to HDM.” One cluster (n = 4) with “multiple sensitizations, mainly to grass pollen, HDM, PR‐10, and nsLTP” was associated with SA in school‐age children.
Conclusions
Although children with wheeze/asthma display frequent occurrences and high levels of sensitization, sensitization patterns did not provide strong signals to discriminate children with severe disease from those with milder disease. These results suggest that the severity of wheeze/asthma may depend on both IgE‐ and non‐IgE‐mediated mechanisms.
IgE sensitization patterns in severe recurrent wheeze/school‐age asthma.
This article is the summary of a workshop, which took place in November 2013, on the roles of microorganisms in chronic respiratory diseases. Until recently, it was assumed that lower airways were ...sterile in healthy individuals. However, it has long been acknowledged that microorganisms could be identified in distal airway secretions from patients with various respiratory diseases, including cystic fibrosis (CF) and non-CF bronchiectasis, chronic obstructive pulmonary disease, asthma and other chronic airway diseases (e.g. post-transplantation bronchiolitis obliterans). These microorganisms were sometimes considered as infectious agents that triggered host immune responses and contributed to disease onset and/or progression; alternatively, microorganisms were often considered as colonisers, which were considered unlikely to play roles in disease pathophysiology. These concepts were developed at a time when the identification of microorganisms relied on culture-based methods. Importantly, the majority of microorganisms cannot be cultured using conventional methods, and the use of novel culture-independent methods that rely on the identification of microorganism genomes has revealed that healthy distal airways display a complex flora called the airway microbiota. The present article reviews some aspects of current literature on host-microbe (mostly bacteria and viruses) interactions in healthy and diseased airways, with a special focus on distal airways.
The European charter for idiopathic pulmonary fibrosis is a welcome document 1, highlighting unmet needs in patients which will inform policy makers across Europe. However, it is sad that an ...important opportunity to involve children with interstitial lung diseases (ILDs) has been missed. There is a current pan-European paediatric ILD consortium 2 which could have been used to access these children and families. Our own work also highlighted similar themes 3: diagnostic delay, the need for improved holistic care and written information, and also interestingly highlighted a previously undescribed issue, the high prevalence of feeding disorders in these children. So the European Charter, excellent as it is, sadly represents a lost opportunity to represent the needs of children with rare lung diseases to health commissioners.
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