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Interstitial lung disease in infants under 3 months in a developing country

Thesis (MMed)--Stellenbosch University, 2022.

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Main Author: Booysen, Lynn
Other Authors: Goussard, Pierre
Format: Thesis
Language:en_ZA
Published: Stellenbosch : Stellenbosch University 2022
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access_status_str Open Access
author Booysen, Lynn
author2 Goussard, Pierre
author_browse Booysen, Lynn
Goussard, Pierre
author_facet Goussard, Pierre
Booysen, Lynn
author_sort Booysen, Lynn
collection Thesis
dc_rights_str_mv Stellenbosch University
description Thesis (MMed)--Stellenbosch University, 2022.
format Thesis
id oai:scholar.sun.ac.za:10019.1/124610
institution Stellenbosch University (South Africa)
language en_ZA
last_indexed 2026-06-10T12:40:50.669Z
license_str Other — see source repository
provenance_str_mv Harvested via OAI-PMH from SUNScholar — Stellenbosch University Repository
publishDate 2022
publishDateRange 2022
publishDateSort 2022
publisher Stellenbosch : Stellenbosch University
publisherStr Stellenbosch : Stellenbosch University
record_format dspace
source_str SUNScholar — Stellenbosch University Repository
spelling oai:scholar.sun.ac.za:10019.1/124610 Interstitial lung disease in infants under 3 months in a developing country Booysen, Lynn Goussard, Pierre Stellenbosch University. Faculty of Medicine and Health Sciences. Dept. of Paediatrics and Child Health. Interstitial lung diseases in children Pediatric respiratory diseases Neonatology Lungs -- Biopsy UCTD Thesis (MMed)--Stellenbosch University, 2022. 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" Masters 2022-03-05T11:35:25Z 2022-04-29T09:22:13Z 2022-03-05T11:35:25Z 2022-04-29T09:22:13Z 2022-04 Thesis http://hdl.handle.net/10019.1/124610 en_ZA Stellenbosch University 62 pages : illustrations application/pdf Stellenbosch : Stellenbosch University
spellingShingle Interstitial lung diseases in children
Pediatric respiratory diseases
Neonatology
Lungs -- Biopsy
UCTD
Booysen, Lynn
Interstitial lung disease in infants under 3 months in a developing country
title Interstitial lung disease in infants under 3 months in a developing country
title_full Interstitial lung disease in infants under 3 months in a developing country
title_fullStr Interstitial lung disease in infants under 3 months in a developing country
title_full_unstemmed Interstitial lung disease in infants under 3 months in a developing country
title_short Interstitial lung disease in infants under 3 months in a developing country
title_sort interstitial lung disease in infants under 3 months in a developing country
topic Interstitial lung diseases in children
Pediatric respiratory diseases
Neonatology
Lungs -- Biopsy
UCTD
url http://hdl.handle.net/10019.1/124610
work_keys_str_mv AT booysenlynn interstitiallungdiseaseininfantsunder3monthsinadevelopingcountry