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Updated: Sep 3, 2026
Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus
Published on: March 6, 2019
The Value of Lung Biopsy in Infants up to 3 Months With Diffuse Lung Disease in a Resource-Limited Setting
Pierre Goussard1, Pawel Schubert2, Lizelle van Wyk1
1Department of Paediatrics and Child Health, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa, sun.ac.za.
Introduction:
Neonatal lung biopsy guides management of unusually severe, diffuse lung disease with an uncertain diagnosis. Childhood interstitial lung disease (chILD) constitutes a diverse group of uncommon respiratory diseases which are associated with major morbidity and mortality. The incidence, outcome and mortality of chILD and other severe respiratory diseases in resource-limited settings (RLS) are unclear.
Objective:
This retrospective, descriptive study examined lung biopsy in an RLS on infants up to 3 months of age to diagnose and facilitate management. This study included neonates with severe respiratory distress not responding to surfactant replacement.
Results:
Lung biopsy was diagnostic in 94% (29/31) of patients without procedure-related mortality. The mean gestational age at birth was 35.8 weeks (SD ± 4.6). The mean presentation age was 20 days of life (IQR 1-28). Of 31 participants, 16% (n = 5) had histological findings in keeping with pulmonary interstitial glycogenosis (PIG) only. Sixteen percent (n = 5) of biopsies showed findings in keeping with adenosine triphosphate (ATP)-binding cassette subfamily A member 3 (ABCA3) deficiency in combination with features of PIG on electron microscopy. Surfactant protein (SFTP)-B deficiency and undefined surfactant deficiency were equally prevalent (6% (n = 2). Pulmonary hypertension (PHT) featured in 61% (n = 17) of infants. Genetic testing was performed on 29% (n = 9) of infants, with 56% (n = 5) showing normal results. The cohort mortality was 42% (n = 13).
Conclusions:
ChILD should be considered in neonates with severe respiratory distress and PHT not responding to surfactant replacement. Lung biopsy is safe and diagnostic and may prevent long and futile treatment. Histopathological diagnosis frequently assists treatment decisions. Future multicentre studies should include children from an RLS to collect information on diagnoses, genetics and treatment responses.
