Early onset children’s interstitial lung diseases: Discrete entities or manifestations of pulmonary dysmaturity?

Bush, Andrew; Griese, Matthias; Seidl, Elias; Kerem, Eitan; Reu, Simone; Nicholson, Andrew G.

doi:10.1016/j.prrv.2018.09.004

Cited by 26 publications

(51 citation statements)

References 83 publications

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“…16 Others postulate a developmental delay as increased NEC can be found in the airways of the developing lung. 17 Only case reports and small case series describing the clinical presentation and clinical course exist. 7,16,[18][19][20] To our knowledge, no death or lung transplantation has been described up to date.…”

Section: Introductionmentioning

confidence: 99%

Persistent tachypnea of infancy: Follow up at school age

Seidl

Carlens

Schwerk

et al. 2020

Pediatric Pulmonology

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Background: Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical courses beyond infancy have rarely been assessed. Methods: Patients included in the Kids Lung Register diagnosed with PTI as infants and now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry and clinical outcome were analyzed. Results: Thirty-five children older than 5 years with PTI diagnosed as infants were analyzed. At the age of 5 years, 74% of the patients were reported as asymptomatic and did not develope new symptoms during the observational period at school-age (mean, 3.9 years; range, 0.3-6.3). At the age of about 10 years, none of the symptomatic children had abnormal oxygen saturation during sleep or exercise anymore.

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Section: Introductionmentioning

confidence: 99%

Persistent tachypnea of infancy: Follow up at school age

Seidl

Carlens

Schwerk

et al. 2020

Pediatric Pulmonology

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“…Furthermore, PNEC degranulation and bombesin like peptide secretion are known to be induced by hypoxia, which is also associated with increased reactive oxygen species in postnatal lung diseases 2,3 . Number of PNECs continues to decline in the first year of life 6‐8 …”

Section: Introductionmentioning

confidence: 99%

Variation in the bombesin staining of pulmonary neuroendocrine cells in pediatric pulmonary disorders—A useful marker for airway maturity

Emiralioğlu

Orhan

Cinel

et al. 2020

Pediatric Pulmonology

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Objectives Pulmonary neuroendocrine cells (NEC) increase with age due to pulmonary maturity. The aim of this study was to determine whether open lung biopsies from patients with interstitial lung diseases have increased pulmonary NEC compared with neuroendocrine cell hyperplasia of infancy (NEHI). Our second aim was to assess pulmonary NECs in the lung autopsy of children without lung disease who died from different causes. Methods Lung tissue of 5 infants with NEHI; 21 patients with pediatric interstitial lung disease (chILD); 17 lung autopsies of infants at varying age without lung disease were included. The percentage of the airways containing neuroendocrine cells, the average percentage of neuroendocrine cells (NECs) per airway, and the number of neuroendocrine bodies (NEBs) in each case were analyzed. Results The mean percentage of the airways containing neuroendocrine cells were 95% in the NEHI group, 30% in the chILD group, 89% under Intrauterine 37 weeks, 70% between intrauterine 37 to 40 weeks, 52% at postnatal 4 days to 6 months of autopsy ages. In the NEHI group, diffuse NE cell distribution and large NEBs were noticed in the lung biopsy. In the chILD group, neuroendocrine cells were dispersed, did not form clusters and NE cells showed solitary distribution. In the lung autopsy group, linear NE cells were detected at younger aged fetuses and solitary distribution of NE cells was detected with the older increasing age. Conclusions Our findings confirm that NECs are seen in many other childhood interstitial lung diseases; NE cell hyperplasia may be a marker of decreased pulmonary development and NE cells decrease with the increasing age of the fetus during Intrauterine life.

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“…Symptoms are not detectable at birth but usually present before 12 months (mean: 4 months, interquartile range = 2-6 months) 41 . Remarkable in their absence are prematurity, evidence of pulmonary dysmatura-tion, underlying causes of diffuse lung disease, congenital heart disease, or genetic characteristics 3,39,40,42,43 . However, a recent review on 117 NEHI children demonstrated that 17% of them had evidence of immune system abnormalities including low immunoglobulin (Ig)G and IgA levels, low complement 3 concentrations, and cyclic neutropenia of infancy 41 .…”

Section: Nehimentioning

confidence: 99%

“…Hence, despite the positive correlation between the load of pulmonary NEC and severity of obstruction, a discrepancy remains between abundant pulmonary NEC and paucity of inflammation and histological abnormalities, as compared to BPD and other conditions with increased pulmonary NEC. An alternative explanation for NEHI proposed by recent publications, suggests that pulmonary neuroendocrine cells are a marker of airway underdevelopment and immaturity 18,19,43 and persistence of bombesin is shared in postnatal life by a variety of infantile pathologies. 18 Due to low prevalence and lack of animal models, the etiology and pathophysiology of NEHI remain elusive.…”

Section: Nehimentioning

confidence: 99%

“…In this context, that in post-viral obstructive disease the severity of the response might be determined by an underlying genetic abnormality. Alternatively, if one accepts the notion that the presence of bombesin is a marker of dysmaturity 18,19,43 the fact that the presentation of NEHI is delayed beyond the neonatal period, points to the need for a second hit for a clinical presentation to emerge. It is tempting to expand the hypothesis to include post-infectious bronchiolitis obliterans (PIBO), a disorder where viral infection leads to severe structural airway damage 58 .…”

Section: Nehimentioning

confidence: 99%

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Upregulation of neuropeptides and infant obstructive airway disorder in post-RSV wheezing and NEHI

Wang

Cárdenas

Bedoya

et al. 2020

Preprint

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Obstructive airway disorders are common in infancy and early childhood. The leading example of such disorder is post-viral wheezing, predominantly the well characterized disorder that follows respiratory syncytial virus (RSV) infection and leads to intermittent, long-term wheezing. The underlying mechanisms of the airway reactivity related to RSV infection have been extensively studies and are associated with dysregulation of the nonadrenergic-noncholinergic (NANC) system, via upregulation of neurotransmitters, typically Substance P. Neuroendocrine hyperplasia of infancy (NEHI), while a less common entity, is a disorder of infancy characterized by more severe and long-term obstructive airway disease. NEHI is pathophysiologically characterized by abundance of neuroendocrine cells in the airways containing the neuroimmune mediator bombesin, the release of which is presumed to be the driver of the persistent small airway obstruction and functional air-trapping. Here we review the NANC and NEC neurotransmitter systems and their studied roles in pulmonary diseases with a focus on their role in lung development, and subsequent various pediatric lung diseases. We focus on the juxtaposition of the separate neuroimmune mechanisms underlying the pathogenesis of post-RSV recurrent wheezing and NEHI persistent small airway obstruction. We finally raise the question whether substance P is indeed specific to post-RSV infection and bombesin to NEHI and then propose a unifying concept of post-viral spectrum of respiratory disorders that may encompass these two entities and possibly others.

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Early onset children’s interstitial lung diseases: Discrete entities or manifestations of pulmonary dysmaturity?

Cited by 26 publications

References 83 publications

Persistent tachypnea of infancy: Follow up at school age

Persistent tachypnea of infancy: Follow up at school age

Variation in the bombesin staining of pulmonary neuroendocrine cells in pediatric pulmonary disorders—A useful marker for airway maturity

Upregulation of neuropeptides and infant obstructive airway disorder in post-RSV wheezing and NEHI

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