Interstitial lung disease in infants under 3 months in a developing country
Date
2022-04
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Stellenbosch : Stellenbosch University
Abstract
ENGLISH SUMMARY: Introduction: Interstitial Lung Disease is uncommon and constitutes a diverse group of
respiratory disorders. It is accompanied by major morbidity and mortality which makes it
a formidable spectrum of disorders. Childhood interstitial lung diseases are increasingly
recognised and contain many lung developmental and genetic disorders not yet identified
in adult pneumology. Negative sequalae of ILD extend into adult life if the affected
individual, in the rare occurrence, does survive into adulthood. Unfortunately, this group
of diseases are seemingly still very underdiagnosed and even when diagnosed early has
proven to be a challenge for clinicians to manage satisfactorily. Lung biopsies have been
shown to be valuable in aiding the diagnosis.
Objective: The aim of this study was to describe the spectrum of pathologies of lung
biopsies performed in children who presented with respiratory concerns before 3 months
old with interstitial lung pattern on CXR in a developing country.
Design: A retrospective (2004-2021)
Results: The biopsy was diagnostic in 94 % (n=29) of patients with no mortality related
to the biopsy. The mean gestational age at birth was 35.8 weeks (SD ±4.6). Nineteen
percent (n=6) of the participants weighed 1500g or less at birth. The mean age at
presentation was 20 days of life (IQR 1-28). Out of the 31 participants 19%(n=6) had
features in keeping with PIG only. Sixteen percent (n=5) participants had findings in
keeping with ABCA3 mutation with features of PIG. This was the most common
combination of abnormalities seen on biopsy results. Six percent (n= 2) patients had
features of ABCA3 mutation as a single modality. Thirteen percent (n=4) had a lung
biopsy showing CPI. SFPT-B deficiency and Surfactant deficiency undefined were equally
shown on 6 % (n=2) of lung biopsy findings. Six percent (n=2) patients were found to
have pneumonic changes/ abscesses on biopsy. One of these pneumonias were
secondary to CMV and PJP infections. There was one case of congenital syphilis. Fiftyfive
percent(n=12) participants had features of PHT, 63% (n=19) of participants had
chronic lung disease. The mortality among the cohort was 42%(n=13).
Conclusion Neonatal ILD cases are present in South Africa and should be included in
the differential diagnosis of neonates especially in term infants with severe Hyaline
Membrane Disease and pulmonary hypertension not responding to surfactant
replacement. Lung biopsy can be diagnostic and can prevent long and futile treatment
and ventilation in the developing world.
"Geen opsomming beskikbaar"
"Geen opsomming beskikbaar"
Description
Thesis (MMed)--Stellenbosch University, 2022.
Keywords
Interstitial lung diseases in children, Pediatric respiratory diseases, Neonatology, Lungs -- Biopsy, UCTD