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Published on: June 16, 2020
Interstitial Lung Disease in Children: "Specific Conditions of Undefined Etiology" Becoming Clearer
Santiago Presti1, Giuseppe Fabio Parisi1, Maria Papale1
1Pediatric Respiratory Unit, Department of Clinical and Experimental Medicine, University of Catania, Via Santa Sofia 78, 95123 Catania, Italy.
Insights
Neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis (PIG) are rare infant lung diseases. Current research suggests they may represent a spectrum of conditions due to shared pulmonary dysmaturity.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Neonatal Respiratory Medicine
Background:
- Children's interstitial lung disease (chILD) encompasses rare pediatric lung conditions affecting lung interstitium.
- Focus on specific infant chILD types: pulmonary interstitial glycogenosis (PIG) and neuroendocrine cell hyperplasia of infancy (NEHI).
Purpose of the Study:
- To review and synthesize current literature on NEHI and PIG.
- To explore the relationship between these two specific infant chILD conditions.
Main Methods:
- Conducted a narrative review of English-language articles.
- Searched PubMed using keywords: NEHI, PIG, chILD, and related terms.
Main Results:
- Growing interest and understanding of NEHI and PIG are evident in recent literature.
- Evidence suggests NEHI and PIG may exist on a disease continuum due to shared pulmonary dysmaturity.
Conclusions:
- NEHI and PIG are characterized by airway development dysmaturity and respiratory distress in infants.
- Further research into pathogenic mechanisms is crucial for developing targeted therapies to reduce mortality and morbidity.
Background:
Children's interstitial lung disease (chILD) is a rare group of pediatric lung diseases affecting the lung interstitium diffusely. In this work, we focused our attention on a specific infant group of chILD, also known as "specific conditions of undefined aetiology", including pulmonary interstitial glycogenosis (PIG) and neuroendocrine cell hyperplasia of infancy (NEHI).
Methods:
PubMed was searched to conduct this narrative review. We searched for articles in English using the following keywords: (1) neuroendocrine cell hyperplasia of infancy; (2) NEHI; (3) pulmonary interstitial glycogenosis; (4) PIG; (5) chILD.
Results:
An increasing interest and insight into these two conditions have been reported. The updated literature suggests that it is possible to look at these disorders as a continuum of diseases, rather than two different entities, since they share a pulmonary dysmaturity.
Conclusions:
NEHI and PIG are featured by dysmaturity of airway development and consequent respiratory distress. Understanding the underlying pathogenic mechanisms would lead to identifying new targeted therapies to ameliorate the mortality and morbidity of these rare conditions.
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