The paediatric liver transplant experience in Johannesburg, South Africa: A broad overview and update.

BACKGROUND: The Wits Transplant Unit performed its first paediatric liver transplant in 2005. Initial experiences from the unit were published in 2012 and 2014. Since then, significant progress has been made in capacity-building the unit, improving outcomes and enhancing service delivery. This paper presents a broad overview and update of the unit's 17-year experience.   Methods: We conducted a retrospective review of all paediatric liver transplants performed in Johannesburg from 1 January 2005 to 31 December 2021 with a minimum one-year follow-up. Data were accessed from the Wits Donald Gordon Medical Centre Paediatric Liver Transplant Research Database (University of the Witwatersrand Human Research Ethics approval: M190749). The following data were collected: donor and recipient sociodemographic and clinical characteristics, details of transplant procedures, donor grafts and recipient outcomes (post-operative complications, graft and recipient survival).   Results: A total of 270 transplants were performed during the review period. Two thirds of recipients (n=180, 67%) were younger than 5 years at time of transplant and half (n=135, 50%) received a living donor graft. The most common indication for liver transplant was biliary atresia, followed by acute liver failure. Unadjusted recipient survival was 80% (95% CI: 75-85%) at one year, and 68% (95% CI: 59-75%) at five years. Waiting list mortality decreased from 27.3% in 2017 to 5.9% in 2021. One hundred and fifty-four (57.0%) recipients experienced at least one type of intervention requiring surgical complication - the most common being biliary in nature (n = 91; 33.7%).   Conclusion: Over last seventeen years, a sustainable paediatric liver transplantation service has been established in Johannesburg. Living donor, split and ABO incompatible liver transplants have been incorporated in response to the severe organ shortage in South Africa. However, our outcomes can be improved. Additionally, a national transplant initiative to coordinate timeous referrals and expand access to liver transplantation for children with severe acute and chronic liver failure is advised.

The prevalence of coeliac disease-associated human leukocyte antigens in South African transplant donors and recipients.

BACKGROUND: Coeliac disease (CD) is an autoimmune condition occurring in genetically predisposed individuals exposed to an environmental trigger. The human leukocyte antigen (HLA) haplotypes HLA-DQ2.5 and HLA-DQ8 have the strongest association with CD, and 90 - 95% of CD patients bear these haplotypes. The susceptibility of the South African (SA) population to CD has not been studied previously. OBJECTIVES: To describe the genetic propensity of the SA population to CD. METHODS: The South African National Blood Service database was used to analyse the prevalence of HLA-DQ2.5 and HLA-DQ8 in potential donors and recipients of organ transplants. Self-reported ethnic group was used to estimate the prevalence among different population groups. RESULTS: The overall prevalence of HLA-DQ2.5 and HLA-DQ8 was 19.8%. The prevalence was lower in black participants (15.9%) than in whites (28.6%). Coloured (22.0%) and Indian (17.4%) participants had an intermediate prevalence. There was no significant difference between potential transplant donors and recipients. CONCLUSIONS: The prevalence of HLA-DQ2.5 and HLA-DQ8 differed among SA study participants of different ethnicities. However, the notion that CD does not occur in black South Africans owing to lack of a genetic predisposition is incorrect.

Outcomes of Kasai hepatoportoenterostomy in children with biliary atresia in Johannesburg, South Africa.

BACKGROUND: Without timely surgical intervention, most children with biliary atresia (BA) are not expected to live beyond 2 years of age. The initial intervention, the Kasai hepatoportoenterostomy (KPE), aims to achieve biliary drainage. Liver transplantation (LT) is performed if jaundice fails to clear or when biliary cirrhosis occurs. In under-resourced South African (SA) academic state hospitals, KPE procedures are the standard of care for the majority of children with BA, but LT is becoming more routinely available. OBJECTIVES: To describe the outcomes of children with BA undergoing KPE, and to identify presenting clinical, laboratory and histological features that were associated with a more favourable outcome. METHODS: All children with BA who underwent KPE between January 2009 and June 2012 at the Johannesburg academic-hospital complex were included. Clinical and laboratory parameters, including paediatric end-stage liver disease (PELD) score at the time of KPE, liver histology fibrosis score, clearance of jaundice at 6 months and 24-month survival were determined. RESULTS: Of 70 children with BA diagnosed during the study period, 43 (61.4%) underwent KPE, but only 12 (27.9%) achieved early resolution of jaundice. By 24 months, 14 (32.6%) of 43 children undergoing KPE were alive with their native liver, and 2 (4.7%) other children underwent LT. PELD score <15 and early resolution of jaundice, but not age at surgery or histological fibrosis score, predicted a favourable outcome. CONCLUSION: Children with BA undergoing KPE in SA state hospitals have a poor prognosis. The PELD score at the time of KPE best predicts 24-month survival.