Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia

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dc.contributor.advisorGoddard, Elizabethen_ZA
dc.contributor.advisorDe Lacy, Ronaldaen_ZA
dc.contributor.advisorPillay, Komalaen_ZA
dc.contributor.authorLevin, Lindsey Nicolaen_ZA
dc.date.accessioned2017-01-19T12:24:26Z
dc.date.available2017-01-19T12:24:26Z
dc.date.issued2016en_ZA
dc.description.abstractBackground: Biliary atresia (BA) is a progressive obstructive cholangiopathy of unknown aetiology, occurring during the perinatal period. If left untreated it rapidly progresses to hepatic fibrosis and cirrhosis, with death occurring within 2 years. It is the leading cause of end-stage liver disease in the paediatric population and remains the most common indication for paediatric liver transplantation in South Africa. Objectives: Despite a wealth of information from developed countries, very little information is available in Africa and other developing nations. This study aimed to describe the age of presentation, clinical course and outcome of infants presenting to Red Cross War Memorial Children's Hospital (RCWMCH) with BA. Methods: A retrospective folder review was conducted on all patients with BA presenting to RCWMCH between January 2003 and December 2013. The main outcomes assessed were median time to presentation to tertiary services, clearance of jaundice post Kasai procedure (bilirubin <20μmol/L) and 2- and 5-year overall survival (OS) and survival with native liver (SNL). Results: The median age at presentation in the 80 cases reviewed was 70 days. Kasai procedure (KP) was performed in 62 (77.5%) patients at a median age of 68 days. 18 patients who presented late did not undergo KP. Clearance of jaundice was achieved in 39% of KPs. 13 patients underwent KP beyond 90 days with a success rate of 38%. 2- and 5-year SNL rates were 41% and 37.5% respectively with OS of 59% at 2-years and 56% at 5-years. Liver transplant was only performed in 12 of the 54 patients who showed progression to require transplantation. Conclusions: Jaundice clearance post KP and SNL compared favourably with international figures, however, lower overall survival rates reflected lack of access to transplantation. Age at KP was not a predictor of poor outcome.en_ZA
dc.identifier.apacitationLevin, L. N. (2016). <i>Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia</i>. (Thesis). University of Cape Town ,Faculty of Health Sciences ,Department of Paediatrics and Child Health. Retrieved from http://hdl.handle.net/11427/22822en_ZA
dc.identifier.chicagocitationLevin, Lindsey Nicola. <i>"Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia."</i> Thesis., University of Cape Town ,Faculty of Health Sciences ,Department of Paediatrics and Child Health, 2016. http://hdl.handle.net/11427/22822en_ZA
dc.identifier.citationLevin, L. 2016. Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia. University of Cape Town.en_ZA
dc.identifier.ris TY - Thesis / Dissertation AU - Levin, Lindsey Nicola AB - Background: Biliary atresia (BA) is a progressive obstructive cholangiopathy of unknown aetiology, occurring during the perinatal period. If left untreated it rapidly progresses to hepatic fibrosis and cirrhosis, with death occurring within 2 years. It is the leading cause of end-stage liver disease in the paediatric population and remains the most common indication for paediatric liver transplantation in South Africa. Objectives: Despite a wealth of information from developed countries, very little information is available in Africa and other developing nations. This study aimed to describe the age of presentation, clinical course and outcome of infants presenting to Red Cross War Memorial Children's Hospital (RCWMCH) with BA. Methods: A retrospective folder review was conducted on all patients with BA presenting to RCWMCH between January 2003 and December 2013. The main outcomes assessed were median time to presentation to tertiary services, clearance of jaundice post Kasai procedure (bilirubin <20μmol/L) and 2- and 5-year overall survival (OS) and survival with native liver (SNL). Results: The median age at presentation in the 80 cases reviewed was 70 days. Kasai procedure (KP) was performed in 62 (77.5%) patients at a median age of 68 days. 18 patients who presented late did not undergo KP. Clearance of jaundice was achieved in 39% of KPs. 13 patients underwent KP beyond 90 days with a success rate of 38%. 2- and 5-year SNL rates were 41% and 37.5% respectively with OS of 59% at 2-years and 56% at 5-years. Liver transplant was only performed in 12 of the 54 patients who showed progression to require transplantation. Conclusions: Jaundice clearance post KP and SNL compared favourably with international figures, however, lower overall survival rates reflected lack of access to transplantation. Age at KP was not a predictor of poor outcome. DA - 2016 DB - OpenUCT DP - University of Cape Town LK - https://open.uct.ac.za PB - University of Cape Town PY - 2016 T1 - Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia TI - Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia UR - http://hdl.handle.net/11427/22822 ER - en_ZA
dc.identifier.urihttp://hdl.handle.net/11427/22822
dc.identifier.vancouvercitationLevin LN. Biliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia. [Thesis]. University of Cape Town ,Faculty of Health Sciences ,Department of Paediatrics and Child Health, 2016 [cited yyyy month dd]. Available from: http://hdl.handle.net/11427/22822en_ZA
dc.language.isoengen_ZA
dc.publisher.departmentDepartment of Paediatrics and Child Healthen_ZA
dc.publisher.facultyFaculty of Health Sciencesen_ZA
dc.publisher.institutionUniversity of Cape Town
dc.subject.otherPaediatricsen_ZA
dc.titleBiliary atresia at Red Cross War Memorial Children's Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresiaen_ZA
dc.typeMaster Thesis
dc.type.qualificationlevelMasters
dc.type.qualificationnameMMeden_ZA
uct.type.filetypeText
uct.type.filetypeImage
uct.type.publicationResearchen_ZA
uct.type.resourceThesisen_ZA
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