Pediatric pulmonology
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Pediatric pulmonology · Feb 2010
ReviewCystic fibrosis lung disease starts in the small airways: can we treat it more effectively?
The aims of this article are to summarize existing knowledge regarding the pathophysiology of small airways disease in cystic fibrosis (CF), to speculate about additional mechanisms that might play a role, and to consider the available or potential options to treat it. In the first section, we review the evidence provided by pathologic, physiologic, and imaging studies suggesting that obstruction of small airways begins early in life and is progressive. In the second section we discuss how the relationships between CF transmembrane conductance regulator (CFTR), ion transport, the volume of the periciliary liquid layer and airway mucus might lead to defective mucociliary clearance in small airways. ⋯ In the final section, we discuss how established therapies impact small airways disease and new directions that may lead to improvement in the treatment of small airways disease. We conclude that there are many reasons to believe that small airways play an important role in the pathophysiology of (early) CF lung disease. Therapy should be aimed to target the small airways more efficiently, especially with drugs that can correct the basic defect at an early stage of disease.
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Pediatric pulmonology · Jan 2010
ReviewPediatric respiratory medicine--an international perspective.
Although Pediatric Respiratory Medicine as a subspecialty has a long tradition and is well established in some countries, there is a wide variation across different regions of the world with regard to e.g. recognition of the discipline, training requirements, training facilities and clinical needs. This review summarizes the situation in North America (US and Canada), South America, Asia, Australia, Israel and Europe with the aim to highlight commonalities and differences and, ultimately, to further support continuous development of paediatric Respiratory Medicine Worldwide.
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Pediatric pulmonology · Jul 2009
ReviewHigh flow nasal cannula therapy as respiratory support in the preterm infant.
We reviewed the literature on the effects of high flow nasal cannula (HFNC) and heated, humidified, high-flow, nasal cannula (HHHFNC) treatment in preterm infants. We found nine studies, but only two were randomized controlled trials. These studies show that: HFNC application is associated to the delivery of continuous distending pressure (CDP) in patients with closed mouth, whose value is proportional to the delivered flow only in smaller infants; the CDP delivered by HFNC is unpredictable and present large inter-patient and intra-patient variability; the use of recently available HHHFNC devices is effective in minimizing nasal mucosa injuries compared to traditional HFNC; the effectiveness of HHHFNC versus NCPAP for the treatment of apnoea of prematurity, respiratory distress syndrome, and the prevention of extubation failure, has been poor investigated and firm conclusions cannot be drawn on this matter. In conclusion, on the basis of published data, the routinary application of HFNC should be limited to patients requiring oxygen-therapy, HHHFNC devices should be preferred to HFNC, but their employment as an alternative to NCPAP should wait for the conclusion of randomized controlled trials.
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Pediatric pulmonology · Jun 2009
ReviewCongenital central hypoventilation syndrome from past to future: model for translational and transitional autonomic medicine.
The modern story of CCHS began in 1970 with the first description by Mellins et al., came most visibly to the public eye with the ATS Statement in 1999, and continues with increasingly fast paced advances in genetics. Affected individuals have diffuse autonomic nervous system dysregulation (ANSD). The paired-like homeobox gene PHOX2B is the disease-defining gene for CCHS; a mutation in the PHOX2B gene is requisite to the diagnosis of CCHS. ⋯ A growing number of cases of CCHS are identified after the newborn period, with presentation from infancy into adulthood. An improved understanding of the molecular basis of the PHOX2B mutations and of the PHOX2B genotype/CCHS phenotype relationship will allow physicians to anticipate the clinical phenotype for each affected individual. To best convey the remarkable history of CCHS, and to describe the value of recognizing CCHS as a model for translational and transitional autonomic medicine, we present this review article in the format of a chronological story, from 1970 to the present day.
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Pediatric pulmonology · Jun 2008
ReviewChronic wet cough: Protracted bronchitis, chronic suppurative lung disease and bronchiectasis.
The role of persistent and recurrent bacterial infection of the conducting airways (endobronchial infection) in the causation of chronic respiratory symptoms, particularly chronic wet cough, has received very little attention over recent decades other than in the context of cystic fibrosis (CF). This is probably related (at least in part) to the (a) reduction in non-CF bronchiectasis in affluent countries and, (b) intense focus on asthma. In addition failure to characterize endobronchial infections has led to under-recognition and lack of research. ⋯ Also misdiagnosis of asthma is common and the diagnostic process is further complicated by the fact that the co-existence of asthma is not uncommon. The principles of managing PBB, CSLD and bronchiectasis are the same. Further work is required to improve recognition, diagnosis and management of these causes of chronic wet cough in children.