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

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dc.contributor.advisorGoussard, Pierreen_ZA
dc.contributor.authorBooysen, Lynnen_ZA
dc.contributor.otherStellenbosch University. Faculty of Medicine and Health Sciences. Dept. of Paediatrics and Child Health.en_ZA
dc.date.accessioned2022-03-05T11:35:25Zen_ZA
dc.date.accessioned2022-04-29T09:22:13Zen_ZA
dc.date.available2022-03-05T11:35:25Zen_ZA
dc.date.available2022-04-29T09:22:13Zen_ZA
dc.date.issued2022-04en_ZA
dc.descriptionThesis (MMed)--Stellenbosch University, 2022.en_ZA
dc.description.abstractENGLISH 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.en_ZA
dc.description.abstract"Geen opsomming beskikbaar"af_ZA
dc.description.versionMastersen_ZA
dc.format.extent62 pages : illustrationsen_ZA
dc.identifier.urihttp://hdl.handle.net/10019.1/124610en_ZA
dc.language.isoen_ZAen_ZA
dc.publisherStellenbosch : Stellenbosch Universityen_ZA
dc.rights.holderStellenbosch Universityen_ZA
dc.subjectInterstitial lung diseases in childrenen_ZA
dc.subjectPediatric respiratory diseasesen_ZA
dc.subjectNeonatologyen_ZA
dc.subjectLungs -- Biopsyen_ZA
dc.subjectUCTDen_ZA
dc.titleInterstitial lung disease in infants under 3 months in a developing countryen_ZA
dc.typeThesisen_ZA
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