Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa
Background. Biliary atresia (BA) is an obstructive inflammatory disease of the bile ducts. Without intervention, the disease rapidly progresses to liver cirrhosis and fibrosis, with end-stage liver failure and death occurring within the first 3 years of life. It is the most common indication fo...
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South African Medical Association
2023-11-01
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| Online Access: | https://samajournals.co.za/index.php/samj/article/view/845 |
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| author | E Brits S M le Grange |
| author_facet | E Brits S M le Grange |
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Background. Biliary atresia (BA) is an obstructive inflammatory disease of the bile ducts. Without intervention, the disease rapidly progresses to liver cirrhosis and fibrosis, with end-stage liver failure and death occurring within the first 3 years of life. It is the most common indication for liver transplantation (LT) in the paediatric population. The management of BA in South Africa (SA) faces multiple challenges, such as late referrals and socioeconomic burdens, with suboptimal outcomes.
Objectives. To determine risk factors and shortcomings that are detrimental to the outcome of the paediatric patient population by reviewing the profile, management and outcome of patients with BA treated at Universitas Academic Hospital Complex (UAHC), Bloemfontein, SA.
Methods. This was a retrospective analytical record review of all patients diagnosed with BA and treated at UAHC from 1 January 2009 to 31 December 2019.
Results. In total, 67 patients were included; 74.6% were female, and 86.6% were black Africans. Most (62.7%) had isolated BA. A Kasai portoenterostomy (KPE) was performed in 32 patients (47.8%). Of 5 patients referred for LT evaluation, 2 received a transplant. Of 55 patients with known outcomes, 5.5% (n=3) survived and 94.5% (n=52) died after receiving a KPE or palliative treatment. Of the 3 patients who were alive at the end of the study period, 1 had a KPE and 2 had LTs.
Conclusion. Late presentation, cholangitis and cessation of bile flow after an initial successful KPE, and socioeconomic challenges are issues of concern and had a detrimental influence on the outcome of BA in our study population. Implementing screening measures and education programmes at the primary healthcare level is essential to diagnose and refer BA patients timeously. Establishing support systems to assist socioeconomically disadvantaged patients will enable them to qualify for LT.
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| format | Article |
| id | doaj-art-dda4b44df68a4b9c973a9579a17d5382 |
| institution | Kabale University |
| issn | 0256-9574 2078-5135 |
| language | English |
| publishDate | 2023-11-01 |
| publisher | South African Medical Association |
| record_format | Article |
| series | South African Medical Journal |
| spelling | doaj-art-dda4b44df68a4b9c973a9579a17d53822025-02-10T12:26:41ZengSouth African Medical AssociationSouth African Medical Journal0256-95742078-51352023-11-011131110.7196/SAMJ.2023.v113i11.845Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South AfricaE Brits0S M le Grange1https://orcid.org/0000-0002-5868-181XDepartment of Surgery, Faculty of Health Sciences, University of the Free State, Bloemfontein, South AfricaDepartment of Surgery, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa Background. Biliary atresia (BA) is an obstructive inflammatory disease of the bile ducts. Without intervention, the disease rapidly progresses to liver cirrhosis and fibrosis, with end-stage liver failure and death occurring within the first 3 years of life. It is the most common indication for liver transplantation (LT) in the paediatric population. The management of BA in South Africa (SA) faces multiple challenges, such as late referrals and socioeconomic burdens, with suboptimal outcomes. Objectives. To determine risk factors and shortcomings that are detrimental to the outcome of the paediatric patient population by reviewing the profile, management and outcome of patients with BA treated at Universitas Academic Hospital Complex (UAHC), Bloemfontein, SA. Methods. This was a retrospective analytical record review of all patients diagnosed with BA and treated at UAHC from 1 January 2009 to 31 December 2019. Results. In total, 67 patients were included; 74.6% were female, and 86.6% were black Africans. Most (62.7%) had isolated BA. A Kasai portoenterostomy (KPE) was performed in 32 patients (47.8%). Of 5 patients referred for LT evaluation, 2 received a transplant. Of 55 patients with known outcomes, 5.5% (n=3) survived and 94.5% (n=52) died after receiving a KPE or palliative treatment. Of the 3 patients who were alive at the end of the study period, 1 had a KPE and 2 had LTs. Conclusion. Late presentation, cholangitis and cessation of bile flow after an initial successful KPE, and socioeconomic challenges are issues of concern and had a detrimental influence on the outcome of BA in our study population. Implementing screening measures and education programmes at the primary healthcare level is essential to diagnose and refer BA patients timeously. Establishing support systems to assist socioeconomically disadvantaged patients will enable them to qualify for LT. https://samajournals.co.za/index.php/samj/article/view/845biliary atresiadeveloping countryLesothoSouth Africa |
| spellingShingle | E Brits S M le Grange Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa South African Medical Journal biliary atresia developing country Lesotho South Africa |
| title | Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa |
| title_full | Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa |
| title_fullStr | Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa |
| title_full_unstemmed | Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa |
| title_short | Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa |
| title_sort | biliary atresia the profile management and outcome of patients treated at a tertiary hospital in central south africa |
| topic | biliary atresia developing country Lesotho South Africa |
| url | https://samajournals.co.za/index.php/samj/article/view/845 |
| work_keys_str_mv | AT ebrits biliaryatresiatheprofilemanagementandoutcomeofpatientstreatedatatertiaryhospitalincentralsouthafrica AT smlegrange biliaryatresiatheprofilemanagementandoutcomeofpatientstreatedatatertiaryhospitalincentralsouthafrica |