Russell Britz

Heterozygote to homozygote related living donor liver transplantation in maple syrup urine disease: A case report

Liver transplantation is an accepted treatment modality in the management of MSUD. To our knowledge, ours is only the second successful case to date of a patient with MSUD receiving an allograft from an RLD who is a heterozygous carrier for the disease. In view of the worldwide shortage of available organs for transplantation, heterozygote to homozygote transplantation in the setting of MSUD may provide a viable alternative for those awaiting transplantation. We report on the case of a two‐yr‐old infant with MSUD, who received a left lateral segment (segments II and III) liver transplant from his mother, a heterozygote carrier of one of the three abnormal genes implicated in MSUD. Post‐operative BCAA levels normalized in our patient and remained so on an unrestricted protein diet and during times of physiological stress. To date, this is only the second case of a successful RLD liver transplant in a child with MSUD. Preliminary results indicate that RLD liver transplants are at least equivalent to deceased donor liver transplants in the treatment of MSUD, although longer term follow‐up is required. Heterozygote to homozygote RLD transplant in patients with MSUD presents a new pool of potential liver donors.

Paediatric liver transplantation in Johannesburg: Initial 29 cases and prospects for the future

BACKGROUND The Wits Donald Gordon Medical Centre paediatric liver transplant programme is the second such unit in sub-Saharan Africa. Initiated in November 2005, it forms part of the centres solid organ transplant unit, comprising kidney, liver and simultaneous kidney-pancreas arms. Initially established in the private sector, we recently received government approval to expand our programme into the provincial sector and have performed transplants on several provincial-sector patients. Current challenges relate to the lack of appropriately trained paediatric sub-specialists, specifically critical care practitioners and hepatologists. METHODS Subsequent to institutional approval, a retrospective chart analysis of all paediatric liver transplants performed at our facility to date was conducted. RESULTS Defining children as those under 18 years of age, 29 patients have received a cadaveric liver transplant since 2005, using 16 whole livers, 10 reduced-size grafts, and 3 split segments; 13 were transplanted with biliary atresia, 3 hyperoxalurea, 3 autosomal recessive polycystic disease, 2 alpha-1 antitrypsin deficiency, and 2 idiopathic, with the remainder for a wide spectrum of other pathologies. Seven patients received combined liver-kidney transplants. There were 3 in-hospital mortalities. The remaining 26 patients are all long-term survivors. We describe 7 acute surgical morbidities in 6 patients, and 8 long-term surgical morbidities. One patient was subsequently re-transplanted in Cape Town. CONCLUSIONS Despite a shortage of organs, we have overcome a steep learning curve, with results comparable with other early series. The current threat to the continued viability of our unit is the lack of appropriately trained paediatric hepatologists and intensivists.

Favourable outcomes for the first 10 years of kidney and pancreas transplantation at Wits Donald Gordon Medical Centre, Johannesburg, South Africa

BACKGROUND It is important for centres participating in transplantation in South Africa (SA) to audit their outcomes. Wits Donald Gordon Medical Centre (WDGMC), Johannesburg, SA, opened a transplant unit in 2004. The first 10 years of kidney and pancreas transplantation were reviewed to determine outcomes in respect of recipient and graft survival. METHODS A retrospective review was conducted of all kidney-alone and simultaneous kidney-pancreas (SKP) transplants performed at WDGMC from 1 January 2004 to 31 December 2013, with follow-up to 31 December 2014 to ensure at least 1 year of survival data. Information was accessed using the transplant registers and clinical records in the transplant clinic at WDGMC. The Kaplan-Meier method was used to estimate 1-, 5- and 10-year recipient and graft survival rates for primary (first graft) kidney-alone and SKP transplants. RESULTS The overall 10-year recipient and graft survival rates were 80.4% and 66.8%, respectively, for kidney-alone transplantation. In the kidney-alone group, children tended towards better recipient and graft survival compared with adults, but this was not statistically significant. In adults, recipient survival was significantly better for living than deceased donor type. Recipient and graft survival were significantly lower in black Africans than in the white (largest proportion in the sample) reference group. For SKP transplants, the 10-year recipient survival rate was 84.7%, while kidney and pancreas graft survival rates were 73.1% and 43.2%, respectively. CONCLUSION Outcomes of the first 10 years of kidney and pancreas transplantation at WDGMC compare favourably with local and international survival data.

A Ten Year Retrospective Review of Infections in the First 12 Months Post Paediatric Liver and Liver-Kidney Transplantation at a Single Centre in Johannesburg, South Africa

Introduction Two paediatric liver transplant centres exist in South Africa, one in Johannesburg and the other in Cape Town. The programme in Johannesburg at Wits Donald Gordon Medical Centre (WDGMC) began in 2005 and is now the largest volume paediatric liver transplant unit in the country. WDGMC also hosts the only live donor paediatric liver transplant programme in sub-Saharan Africa. This research determined the incidence and profile of infectious events in the first year after transplantation. Materials and Methods This retrospective record review included paediatric liver and liver/kidney transplants from 2005-2015. The paediatric population was defined as any recipient <18 years of age on the day of transplant. Only laboratory confirmed infections were included. Ethics approval was obtained from the University’s Institutional Review Board. Information was sourced from clinical records and pathology reports. Statistical analysis was carried out using SAS version 9.4 with a 5% significance level. Results and Discussion There were 69 transplants in 65 patients.Most were female (42/69;61%), median age was 49 months (IQR 27-152) and biliary atresia was the most common indication for transplant (42%).A total of 253 infections occurred, mainly in the first three months after transplant. The most common site was bloodstream, followed by the abdomen and urinary tract. Bacterial infections occurred most commonly (193/253;76.3%). Of these, gram negative organisms predominated (120/193; 62.2%) and klebsiella species (50/120; 41.7%) was most frequently identified. In the gram positive group (72/193; 37.3%), enterococcus faecium predominated (25/72; 34.7%). Drug resistance was higher in the gram negative group (75.0%; 36/48) when compared to the gram positives (22.9% (11/48). Prevalence of viral infection was 16.6% (42/253) and fungal was 7.1% (18/253). Candida parapsilosis was the most commonly identified fungus. TB prophylaxis is not routinely administered and there was only one case of mycobacterial infection. This is the first study of its kind from sub-Saharan Africa. Gram negative infections were the most common, mirroring findings from the USA and Israel. What differed was that candida parapsilosis, not albicans, was the most frequently identified fungus and the most common primary site of infection was not the abdomen, but rather the bloodstream. Risk factors that may predispose to infection are severe malnutrition at the time of transplant and delayed referrals of young children with advanced disease. Conclusion Our findings suggest that standard TB prophylaxis may not be warranted, even in a country with high TB infection rates. This research has resulted in an urgent policy review in the transplant unit which has prioritised the nutritional status of children pre-transplant and implemented stringent infection control measures to prevent bloodstream infections. Study data were collected and managed using REDCap1 electronic data capture tools hosted at University of the Witwatersrand. This research was supported by Wits Donald Gordon Medical Centre, Johannesburg, South Africa. Data capture performed by the medical student group, Chanceplant from the University of the Witwatersrand. Figure. No caption available. Figure. No caption available.

Rationing healthcare in South Africa: Renal replacement therapy - a case in point

The South African Dialysis and Transplant Registry issued its last report on renal replacement therapy (RRT) in South Africa (SA) in 1994, followed by an unfortunate hiatus for 20 years. The recent publication of the long-awaited South African Renal Registry Annual Report 2012 should be highly commended. The private sector deserves to be acknowledged for its financial support of this initiative. Since 1994, the SA population has increased from 40.436 million to 52.275 million and the treatment rate for end-stage renal disease (ESRD) per million population (pmp) has improved from 70 pmp in 1994 to 164 pmp in 2012. The treatment rate in 2012 for the public sector is essentially unchanged at 73 pmp, compared with 620 pmp (of insured persons) in the private sector. In contrast, the low national kidney transplant rate of 4.7 pmp highlights the dire need for organs.