Combined paediatric liver-kidney transplantation: Analysis of our experience and literature review

Background. Renal insufficiency is increasingly common in end-stage liver disease and allocation of livers to this category of patient has escalated. The frequency of combined liver-kidney transplantation (CLKT) has consequently increased. Indications for CLKT in children differ from those for adults and typically include rare congenital conditions; subsequently limited numbers of this procedure have been performed in paediatric patients worldwide. Scant literature exists on the subject. Methods. Subsequent to institutional approval, a retrospective chart analysis of all paediatric CLKTs performed at the Transplant Unit, Wits Donald Gordon Medical Centre, University of the Witwatersrand, Johannesburg, South Africa between January 2005 and July 2013 was conducted. Results. Defining children as younger than 18 years of age, 43 patients had received a liver transplant since 2005, of whom 8 received a CLKT. Indications included autosomal recessive polycystic kidney disease (n=3), primary hyperoxaluria type 1 (n=4) and heterozygous factor H deficiency with atypical haemolytic uraemic syndrome (n=1). Graft combinations included whole liver and one kidney (n=5), whole liver and two kidneys (n=1) and left lateral liver segment and one kidney (n=2), all from deceased donors. Patient age ranged from 4 to 17 years (median 9) and included 4 females and 4 males. Weight ranged from 13 to 42 kg (median 22.5). We describe one in-hospital mortality. The remaining 7 patients were long-term survivors with a survival range from 6 to 65 months. Conclusions. Although rarely indicated in children, CLKT is an effective treatment option, appropriately utilising a scarce resource and significantly improving quality of life in the recipient.

Diagnosis, monitoring and treatment of tuberous sclerosis complex: A South African consensus response to international guidelines

Tuberous sclerosis complex (TSC) is a genetic disorder with multi-system manifestations and a high burden of disease. In 2013, an international panel of TSC experts revised the guidelines for the diagnosis, surveillance and treatment of the disorder. In South Africa (SA), a local multidisciplinary group of healthcare professionals and TSC researchers reviewed the international guidelines to generate an SA consensus clinical update on the identification, diagnosis, treatment and lifelong monitoring of individuals who live with TSC. We strongly endorse dissemination and use of the international guidelines for the assessment, monitoring and treatment of TSC. In addition, we strongly support access to genetic testing and to mTOR (mammalian target of rapamycin) inhibitors to treat subependymal giant cell astrocytomas not amenable to surgery and renal angiomyolipomas larger than 3 cm, and as adjunctive treatment for refractory focal seizures. We await with interest results from mTOR inhibitor trials of skin and TSC-associated neuropsychiatric disorders (TAND). With regard to training, we recommend the inclusion of TSC in undergraduate and postgraduate medical and health sciences curricula, and the promotion of other continuing professional development events to raise awareness about TSC. We also support the development of a TSC user/carer/parent organisation to provide an informal support network for families across SA. We acknowledge that some progress has been made in recent years in SA, but much remains to be done. We hope that this SA onsensus clinical update based on the international guidelines will make a positive contribution to increase knowledge and improve clinical care for all patients who live with TSC in SA, and their families

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

Diagnosis, monitoring and treatment of tuberous sclerosis complex: A South African consensus response to international guidelines

Tuberous sclerosis complex (TSC) is a genetic disorder with multi-system manifestations and a high burden of disease. In 2013, an international panel of TSC experts revised the guidelines for the diagnosis, surveillance and treatment of the disorder. In South Africa (SA), a local multidisciplinary group of healthcare professionals and TSC researchers reviewed the international guidelines to generate an SA consensus clinical update on the identification, diagnosis, treatment and lifelong monitoring of individuals who live with TSC. We strongly endorse dissemination and use of the international guidelines for the assessment, monitoring and treatment of TSC. In addition, we strongly support access to genetic testing and to mTOR (mammalian target of rapamycin) inhibitors to treat subependymal giant cell astrocytomas not amenable to surgery and renal angiomyolipomas larger than 3 cm, and as adjunctive treatment for refractory focal seizures. We await with interest results from mTOR inhibitor trials of skin and TSC-associated neuropsychiatric disorders (TAND). With regard to training, we recommend the inclusion of TSC in undergraduate and postgraduate medical and health sciences curricula, and the promotion of other continuing professional development events to raise awareness about TSC. We also support the development of a TSC user/carer/parent organisation to provide an informal support network for families across SA. We acknowledge that some progress has been made in recent years in SA, but much remains to be done. We hope that this SA onsensus clinical update based on the international guidelines will make a positive contribution to increase knowledge and improve clinical care for all patients who live with TSC in SA, and their families

The South African guidelines on enuresis-2017

Introduction: Enuresis (or Nocturnal Enuresis) is defined as discreet episodes of urinary incontinence during sleep in children over 5 years of age in the absence of congenital or acquired neurological disorders. Recommendations: Suggestions and recommendations are made on the various therapeutic options available within a South African context. These therapeutic options include; behavioural modification, pharmaceutical therapy [Desmospressin (DDAVP), Anticholinergic (ACh) Agents, Mirabegron (beta 3-adrenoreceptor agonists), and Tricyclic Antidepressants (TCA)], alternative treatments, complementary therapies, urotherapy, alarm therapy, psychological therapy and biofeedback. The role of the Bladder Diary, additional investigations and Mobile Phone Applications (Apps) in enuresis is also explored. Standardised definitions are also outlined within this document. Conclusion: An independent, unbiased, national evaluation and treatment guideline based on the pathophysiological subcategory is proposed using an updated, evidence based approach. This Guideline has received endorsement from the South African Urological Association, Enuresis Academy of South Africa and further input from international experts within the field

An effective approach to chronic kidney disease in South Africa

CITATION: Moosa, M. R., et al. 2016. An effective approach to chronic kidney disease in South Africa. South African Medical Journal, 106(2):156-159, doi:10.7196/SAMJ.2016.v106i2.9928.The original publication is available at http://www.samj.org.zaENGLISH ABSTRACT: Very few patients with end-stage kidney disease in South Africa receive renal replacement treatment (RRT), despite the rapidly growing demand, because of resource constraints. Nephrologists who agonise daily about who to treat and who not to, and have been doing so since the inception of dialysis in this country, welcomed the opportunity to interact with the National Department of Health at a recent summit of stakeholders. The major challenges were identified and recommendations for short- to long-term solutions were made. While the renal community can still improve efficiencies, it is clear that much of the responsibility for improving access to RRT and reducing inequities must be borne by the national government. The summit marks the first step in a process that we hope will ultimately culminate in universal access to RRT for all South Africans.AFRIKAANSE OPSOMMING: Geen opsomming beskikbaarhttp://www.samj.org.za/index.php/samj/article/view/9928Publisher's versio