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Paediatric Pulmonary Disorders

M3 India Newsdesk Jul 04, 2024

Explore the latest advancements in paediatric respiratory medicine, covering topics like cystic fibrosis, neonatal care, asthma, and emerging lung conditions. Discover the challenges and innovations shaping the future of paediatric lung health in this article.

Respiratory diseases in the paediatrics

Respiratory diseases in the paediatric age group are posing new challenges in the field of adult respiratory medicine. Respiratory doctors now face managing conditions like cystic fibrosis (CF) and the lasting effects of premature birth and congenital respiratory tract abnormalities, which were uncommon in adult clinics two decades ago. Additionally, childhood research is reshaping our understanding of respiratory conditions across the lifespan. Birth cohort studies have provided insights into the various wheezing patterns, their causes, and how they progress during childhood.

It is becoming increasingly clear that even for diseases, such as chronic obstructive pulmonary disease (COPD), antenatal and early life events are at least as important as smoking in adulthood.

Another notable shift is the increased survival of extremely premature infants, who are now growing into adults with compromised lung function and abnormal findings on CT scans. What lies ahead for the respiratory health of these individuals as they age?

Advances in understanding interstitial lung disease (ILD) are growing, with discoveries of new genetic factors like surfactant protein C mutations that are relevant to ILD in adults. The emergence of effective treatments, especially antibiotics and anti-inflammatory medications, has highlighted the necessity for improved monitoring methods. This includes the development of smaller bronchoscopes for evaluating even premature infants and innovative physiological testing techniques.

The newborn child

  1. Significant advancements have been achieved in neonatal care, including improved utilisation of well-known treatments like antenatal corticosteroids and exogenous surfactants, along with a better understanding of their lasting impacts.
  2. This lesson has also resulted in larger trials, such as the use of nitric oxide in preterm infants and noninvasive ventilation, in this population.
  3. There have been enhancements in defining physiological conditions like chronic lung disease of prematurity or bronchopulmonary dysplasia, and global research efforts are investigating the ideal oxygen levels for preterm babies.
  4. It is crucial to assess the extended respiratory effects to ascertain if prematurity increases the likelihood of developing COPD.
  5. There is growing acknowledgement that even mild lung issues in newborns can lead to substantial long-term implications.

Paediatric asthma

Childhood asthma continues to be a main focus of the speciality. While the occurrence of certain conditions may have stabilised in some advanced regions, it is increasing in other parts of the world. New approaches to the understanding of underlying mechanisms increasingly depend upon phenotype characterisation and cutting-edge biology techniques are needed to define phenotypes. Allergic sensitisation is frequently seen in asthma, but it is not always present. Having Immunoglobulin E sensitisation to particular allergens by itself is not adequate to cause allergic conditions.

Recent discoveries have focused on epithelial barrier function; recently shown to be important in sphingomyelin metabolism and the genes expressed in the epithelium, e.g. filaggrin are implicated in asthma.

While most children with asthma respond well to low doses of inhaled corticosteroids, there remains a subset of severe asthmatics who do not respond to standard treatments. The key task is to identify and categorise these individuals and determine the most suitable novel therapies, potentially targeting specific cytokines, for each patient.

Cystic fibrosis

  1. Historically, the emphasis has been on treating symptoms effectively; however, advancements in comprehending the cellular repercussions of cystic fibrosis transmembrane conductance regulator (CFTR) mutations have paved the way for interventions that address the underlying primary defect.
  2. Gene therapy, CFTR-specific pharmacotherapy and ion channel modulators are currently in clinical trials and advocated at many centres worldwide.
  3. In the end, a treatment approach combining various strategies to target distinct cellular dysfunctions, such as improving intracellular transport and enhancing channel opening, is anticipated to yield the most potent effectiveness.
  4. Newborn screening is available throughout Europe. An early diagnosis and implementation of CF care alone is not sufficient to avoid early lung damage.
  5. Although research is ongoing to discover more effective early intervention approaches, there are currently no established assessment criteria for infants and toddlers to determine the effectiveness of these interventions.
  6. Therefore, in parallel to developing new therapies, validation of new outcome measures is a high priority for the near future.

Primary ciliary dyskinesia and non-CF bronchiectasis

In primary ciliary dyskinesia (PCD), knowledge of the spectrum of gene mutations and their functional consequences has increased. In combination with noninvasive screening tests, such as measurement of nasal nitric oxide, this knowledge helps to clarify the incidence of this under-diagnosed disease.

Ciliopathy is a broadening range of conditions, with abnormalities in cilia function observed in cystic kidney and liver diseases, retinitis pigmentosa, heterotaxy, congenital heart disease, and intricate syndromes.

The diverse roles of primary, nodal, and motile cilia are still being comprehended, making them a significant focus of future research. Treatment approaches have mainly been borrowed from cystic fibrosis (CF). Ongoing longitudinal observational studies are essential in identifying critical factors influencing structural airway deterioration in both primary ciliary dyskinesia (PCD) and non-CF bronchiectasis.

Paediatric sleep medicine

Contemporary research is concentrated on determining the prevalence of obstructive sleep apnea (OSA) and exploring its correlation with subsequent cardiovascular conditions, neurological issues, and metabolic syndrome. Managing OSA in children poses challenges and lacks clear guidelines on treatment thresholds and the consequences of insufficient treatment.

The field of paediatric sleep medicine is just one of the aspects affected by the rise in childhood obesity. Future research will prioritise establishing standardised protocols for identifying at-risk children, along with defining specific indicators necessitating further evaluation or intervention.

Paediatric lung transplantation

Compared to adult lung transplantation, the field of paediatric lung transplantation is far smaller. Paediatric-focused research is often constrained, leading to many advancements being derived from research and practices in adult medicine. The improved outcomes reported in adults have also been reported in paediatric subjects, with international data now reporting a median post-transplantation survival of,5 years, and some centres reporting outcomes better than this.

Over the next ten years, the emphasis will be on working together with adult medical professionals to redefine bronchiolitis obliterans syndrome and chronic graft dysfunction. This partnership may be the beginning of a more thorough understanding of the underlying mechanisms and potential therapeutic approaches.

Monitoring tools

The respiratory physiology of children Children's respiratory physiology has advanced significantly during the last ten years, regardless of age. Infant lung function testing has become more standardised, with the majority of laboratories now employing commercial equipment and following internationally accepted protocols.

During this time, efforts have successfully connected infant lung function testing with lung function testing in school-age children. Collaborative efforts among international research groups to standardise and ensure quality control have resulted in the publication of guidelines by the European Respiratory Society and the American Thoracic Society.

The major feature of the last decade has been a resurgence of interest in alternative functional measurements. Significant progress has been achieved in techniques such as inert gas washout, oscillometry, interrupter resistance, plethysmographic airway resistance, and structured light plethysmography. While these advancements are notable, they often represent refinements and adaptations of existing methods rather than entirely novel techniques.

Paediatric airway endoscopy

  1. Modern ultrafine flexible instruments with a narrow working channel of 1.2 mm enable comprehensive examination of the entire airway, extending from the nasal passage to the bronchial tubes while minimising distortion of airway anatomy and dynamics.
  2. Paediatric flexible bronchoscopy (FB) is now widely accepted as a standard diagnostic procedure. In infants, the most frequent reason for performing FB is to assess airway obstruction, which may impact both the upper and lower airways.
  3. Combining bronchoalveolar lavage (BAL) with FB can enhance the diagnostic value of the procedure in various clinical scenarios. 
  4. BAL is an important tool in the diagnosis of lung infection in both immunocompromised and immuno-competent children because pathogens can easily be identified from small BAL samples.
  5. For children with chronic interstitial lung diseases (ILDs), bronchoalveolar lavage (BAL) can play a crucial role in ruling out or confirming a particular diagnosis, describing the inflammation in the alveoli, and monitoring the patient's progress during treatment and aftercare.
  6. Interventional bronchoscopy is growing in significance, focusing on actions like clearing or stabilising the airways and eliminating obstructions in the airways or alveoli that disrupt normal function.
  7. Procedures in interventional bronchoscopy encompass a range of treatments such as laser therapy, widening narrow airways, inserting stents, performing total lung lavage, and managing bronchopleural fistulas.
  8. In adult practice techniques such as endobronchial ultrasound are well established and as the equipment is miniaturised, children will be able to benefit from the techniques.

Intensive care

Significant advancements have been made in paediatric intensive care, particularly in safeguarding the lungs against damage from mechanical ventilation by lowering pressures and volumes. Modern ventilator technologies are aiding in achieving this goal, with the potential for patient-controlled ventilators such as neurally adjusted ventilatory assist on the horizon. Additionally, there is a rising awareness of the importance of addressing the ongoing care needs of children with neuromuscular disorders.

The road ahead

The population of children surviving premature birth, including those born before 25 weeks gestation with a birth weight below 500 g, is expected to grow. This group will pose a new high-risk population for chronic obstructive pulmonary disease (COPD) for adult healthcare providers. Virus-induced bronchitis and bronchiolitis will remain significant causes of respiratory issues in infants and young children.

There is currently no effective treatment for bronchiolitis, highlighting the need for advancements in this area. Developing vaccines to prevent early infections could have a substantial impact on reducing the risk of asthma developing later in childhood. Strategies are required to halt the progression from episodic wheezing triggered by viruses to multiple triggers of wheezing and ultimately asthma.

It is known that inhaled corticosteroids are not sufficient to prevent this transition, emphasising the necessity for new insights and approaches. Asthma currently ranks as the most prevalent chronic condition among school-aged children and teenagers.

For allergic rhinitis and asthma, personalised allergy vaccines may emerge as viable treatments to prevent asthma development or worsening. Innovative therapies and biomarkers will play a crucial role in identifying suitable candidates for treatment and monitoring treatment outcomes in children.  

In cystic fibrosis cases identified through newborn screening, there is a necessity for innovative therapeutic regimens to improve health outcomes. With the emergence of genotype-targeted treatments that are likely to be administered to young children at the early stages of the disease, there is a need for biomarkers to identify those at risk of accelerated decline and reliable measures to evaluate treatment response in very young individuals.

The resurgence of tuberculosis, particularly multidrug-resistant and extensively drug-resistant forms, poses a significant risk that demands collaborative efforts from diverse professionals and international attention. This also applies to other newly discovered infections, such as methicillin-resistant Staphylococcus aureus. New diagnostic tools, such as interferon-c assays, might provide more specific test results than the tuberculin skin test.

Advancements in medical care are enhancing our ability to save critically ill children. The use of chemotherapy for cancer treatment, as well as bone marrow and solid organ transplants such as lung transplants, will lead to improved outcomes for children with conditions like cancer and progressive lung diseases such as interstitial lung disease (ILD). Conversely, this will lead to increased pulmonary complications.

The use of technology, both noninvasive and invasive, to keep children with neurological and airway diseases alive and at home will become increasingly important. The advancement of medical techniques for increasingly smaller children highlights the growing importance of developing specialised machines and interfaces tailored to paediatric patients.

Orphan lung diseases, including pulmonary fibrosis with unknown causes, autoimmune conditions affecting the lungs, interstitial lung disease (ILD), and congenital respiratory tract malformations, will persist as significant challenges in healthcare, necessitating specialised care at advanced tertiary centres. Collaboration among international networks is progressively increasing to gather extensive patient data and insights to enhance understanding and management of these conditions.

It has been rightly said: A child is not a small adult.!


Disclaimer- The views and opinions expressed in this article are those of the author and do not necessarily reflect the official policy or position of M3 India.

About the author of this article: Dr Waseem Ud Din is a practising pulmonologist from Srinagar.

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