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Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy/Neuroendocrine Cell
Honorata Marczak1, Katarzyna Krenke1, Matthias Griese2
1Department of Pediatric Pneumology and Allergy, Medical University of Warsaw, Warsaw, Poland.
Insights
Persistent tachypnea of infancy (PTI), or neuroendocrine cell hyperplasia of infancy (NEHI), is a common childhood lung disease. This study found variations in diagnostic approaches across European countries, highlighting the need for standardized guidelines.
Area of Science:
- Pediatric Pulmonology
- Childhood Interstitial Lung Disease (chILD)
Background:
- Persistent tachypnea of infancy (PTI), also known as neuroendocrine cell hyperplasia of infancy (NEHI), is a frequent form of chILD in young children.
- Currently, there is no standardized diagnostic protocol for PTI/NEHI.
Purpose of the Study:
- To investigate variations in clinical presentation and diagnostic strategies for PTI/NEHI across European countries.
- To identify potential areas for standardization in the diagnosis of this condition.
Main Methods:
- A retrospective, observational, multicenter study involving 378 children diagnosed with PTI/NEHI from 17 European countries.
- Analysis of clinical characteristics, diagnostic investigations, and treatment approaches.
Main Results:
- The primary symptoms included tachypnea, chest retractions, crackles, hypoxemia, and failure to thrive, with diagnosis typically occurring at 9 months.
- High-resolution CT (HRCT) was universally used; lung biopsies were performed in 23.5% of cases, showing neuroendocrine cell hyperplasia in 52.8%.
- Significant country-specific variations were observed in the utilization of diagnostic tests, including lung biopsies and genetic testing, with a trend towards decreased biopsy use over time.
Conclusions:
- Diagnosis of PTI/NEHI primarily relies on clinical presentation and HRCT findings.
- Substantial heterogeneity exists in diagnostic practices across European nations.
- Standardized diagnostic guidelines are essential to ensure consistent and timely diagnosis of PTI/NEHI.
Background:
Persistent tachypnea of infancy (PTI) or neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease (chILD) that predominantly affects young children. Although it is one of the most common chILDs, no unified diagnostic approach specific to this condition exists.
Research Question:
Are the clinical presentation and the diagnostic approach different in patients with PTI/NEHI among European countries?
Study Design And Methods:
This was a European multicenter, retrospective, observational study. Data on clinical characteristics and diagnostic strategies in patients with PTI/NEHI were analyzed and compared across participating countries.
Results:
The study included 378 children with PTI/NEHI from 17 countries (63.5% male, 97.4% White) who received a diagnosis at a median age of 9 months (interquartile range, 6-13 months). The most common baseline symptoms were tachypnea, chest retractions, crackles on auscultation, hypoxemia, and failure to thrive. High-resolution CT (HRCT) imaging was performed in all patients, with most undergoing chest radiography, echocardiography, and immunology tests. Lung biopsy was carried out in 23.5% of patients, with a decreasing trend over time and variation by country; its use was associated with longer diagnostic delay. Histopathologic examination showed a hyperplasia of pulmonary neuroendocrine cells in 52.8% of patients. Genetic testing was rare, and its application varied significantly among countries. Additional investigations that do not have an established role, such as assessment for gastroesophageal reflux disease and OSA, infant pulmonary function tests, and lung ultrasound, were limited to single countries.
Interpretation:
Diagnosis of PTI/NEHI relies on clinical symptoms and HRCT imaging results, with lung biopsies less commonly performed. Differences exist among countries regarding the number and type of investigations. A need exists for guidelines that will standardize the diagnostic approach.
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