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To review demographics, effect of preoperative chemotherapy on tumour thrombus, imaging, operative strategy, and outcomes of 12 patients presenting with intracardiac extension of Wilms tumour thrombus. A retrospective audit was undertaken on patients with intracardiac extension of Wilms tumour. Patients were identified from the oncology database and information obtained on clinical presentation, stage, preoperative treatment, surgical procedures and complications, histology, and survival status. Ethics approval was obtained from the University of Cape Town Human Research Ethics Committee. From 1984 to 2016, 337 children with Wilms tumour were treated. Twelve (3.6%) had intracardiac extension of tumour thrombus, nine into the right atrium, and three into the right ventricle. Ultrasound, computerized tomography, magnetic resonance imaging, and echocardiograms were used to assess thrombus level. Patients were staged as stage III(8) and IV(4). All patients received preoperative chemotherapy. Thrombus retracted from the heart in two cases. One patient died preoperatively. Eleven underwent laparotomy, median sternotomy, and cardiopulmonary bypass (CPB). Four underwent cavectomy. Five required cavoatrial patches. Thrombus extending into the hepatic veins was extracted in five patients. There was one intraoperative complication and one perioperative death. Thrombus histology showed viable tumour in 9 of 11 patients. Three patients died of progressive disease. Seven patients are currently disease free. A combination of imaging is required to determine thrombus extent, and this facilitates surgical planning. Preoperative chemotherapy may cause thrombus regression, thus avoiding CPB. CPB offers appropriate conditions for safe tumour thrombus excision. Full management in centres with appropriately experienced staff and facilities for CPB is recommended.

Objective: To evaluate paediatric cardiac services in South Africa with respect to referral base, services provided and human resources. Study design: A descriptive study design was used. An audit of the referral base, personnel and activity of paediatric cardiac units throughout South Africa was conducted by means of a questionnaire. A specialist from each centre was asked to provide the relevant data. Where accurate data was not available, estimates were provided by practitioners within each centre. Results: All identified units participated in the audit. Three were private sector units while the other five were primarily public sector units. Twenty four paediatric cardiologists, equally distributed between public and private sector units, were practicing in the country as at end 2008, with a further eight paediatricians undergoing training in paediatric cardiology. This is significantly less than the 88 paediatric cardiologists required for the population of South Africa. Eight paediatric cardiac surgeons were operating predominantly on children in public hospitals and five in private institutions. An estimated 1370 operations for congenital heart disease were performed over a one year period, with 800 of these in the public sector. Extrapolating from accepted estimates of congenital heart disease incidence, this represents conservatively, less that 40% of operations required for the population. Additionally, only 26% of the estimated 114 simple transposition of great arteries born annually were operated on, indicating serious deficiencies in the ability to adequately detect and intervene in serious congenital heart disease presenting in the neonatal period. Conclusion: The infrastructure and resources to detect and manage heart disease in children in South Africa, particularly within the public sector, are grossly inadequate.

A 13-month-old infant was admitted to hospital approximately 3 weeks after ingestion of a button battery, which was lodged in the esophagus and had caused a tracheoesophageal fistula requiring mechanical ventilation. Since the battery had partially penetrated into the tracheal lumen just above the carina and also was in direct contact with the pulmonary artery, extensive considerations regarding airway and circulatory management were required preoperatively, which are presented and discussed in this case report.

Children with heart disease in Africa have little or no access to treatment of any kind, and cardiac surgical services are virtually absent outside a handful of centres in a few of the wealthier nations. There is little reliable data concerning the prevalence of congenital or acquired heart disease in African children, but there is sufficient information to indicate that the burden of cardiac disease is vast. This major non-communicable disease is largely hidden, overshadowed by the incidence of communicable diseases. There is as yet little evidence of the hoped-for epidemiological transition toward non-communicable diseases amongst children in Africa. The burden of congenital heart disease is only part of the problem, with rheumatic heart disease (RHD) remaining the commonest cardiac problem, related to poor socioeconomic conditions. RHD is the most preventable form of cardiac disease, yet there is little preventive work being done. The many obstacles to developing paediatric cardiac care are discussed, and some possible ways forward are proposed.

To explore the relationship between lactate:pyruvate ratio, hyperlactataemia, metabolic acidosis, and morbidity. Prospective observational study in the paediatric intensive care unit (PICU) of a university hospital. Ninety-seven children after open cardiac surgery. Most children (94%) fell into low-moderate operative risk categories; observed PICU mortality was 1%. Blood was sampled on admission for acid-base analysis, lactate, and pyruvate. Metabolic acidosis was defined as standard bicarbonate lower than 22 mmol/l, raised lactate as higher than 2 mmol/l, and raised lactate:pyruvate ratio as higher than 20. Median cardiopulmonary bypass and aortic cross-clamp times were 80 and 46 min. Metabolic acidosis occurred in 74%, hyperlactataemia in 42%, and raised lactate:pyruvate ratio in 45% of children. In multivariate analysis lactate:pyruvate ratio increased by 6.4 in children receiving epinephrine infusion and by 0.4 per 10 min of aortic cross-clamp. Duration of inotropic support increased by 0.29 days, ventilatory support by 0.27 days, and PICU stay by 0.42 days, for each 1 mmol/l increase in lactate. Neither standard bicarbonate nor lactate:pyruvate ratio were independently associated with prolongation of PICU support. Elevated lactate:pyruvate ratio was common in children with mild metabolic acidosis and low PICU mortality. Hyperlactataemia, but not elevated lactate:pyruvate ratio or metabolic acidosis, was associated with prolongation of PICU support. Routine measurement of lactate:pyruvate ratio is not warranted for children in low-moderate operative risk categories.

We retrospectively reviewed the surgical treatment of 12 patients (nine female, mean age 16.1+/-8.7 years) with sub-mitral aneurysms managed in our institution between 1991 and 2002. We identified three groups of patients in accordance with the degree of posterior mitral annular involvement by the aneurysm. A single aneurysm neck was found in seven patients, multiple necks in two and involvement of the entire posterior mitral annulus in three patients. Involvement of the entire posterior annulus by the aneurysmal process has not been previously described. The mean age of this latter group 29+/-5.1 years was significantly older than the former (P=0.001), suggesting a possible progressive nature of sub-mitral aneurysms. An intracardiac surgical approach was used in six patients and a combined intra and extracardiac approach in the remainder. There was no operative mortality. The mitral valve was initially repaired in eight patients. Failure of closure of the aneurysm necessitating reoperation occurred in four patients (33.3%). An understanding of the inter-relationship between the aneurysm and mitral valve is essential for successful surgical repair. Histology of the aneurysm tissue showed rheumatic heart disease in two patients and tuberculosis in two patients. Hence, although sub-valvar aneurysms are thought to be congenital, a third of our patients had evidence of co-existent rheumatic heart disease or tuberculosis.

Pediatric cardiac services are too expensive for most developing nations. Problems other than cardiac disease take priority when it comes to budget allocations. Poor health infrastructure and referral systems, malnutrition, and the HIV/AIDS pandemic aggravate the situation, and the increasing economic divide is threatening what services do exist. We highlight how the practice of pediatric cardiac surgery in South Africa compares with first-world standards and outline some of the problems faced by pediatric cardiac services in developing nations.

Objective: To assess the effectiveness, safety and cost implications of leflunomide treatment for rheumatoid arthritis. Design: Systematic review. Setting: Four trials retrieved from Medline, Embase, the Cochrane Library, Econlit, HMIC (Dhdata), HMIC (Helmis), HMIC (King’s Fund Database) and Best Evidence3. Main outcome measures: Efficacy measures (including tender joint counts, swollen joint counts, assessment of functioning, Health Assessment Questionnaire, Modified Health Assessment Questionnaire, pain (visual analogue scale), Erythrocyte Sedimentation Rate, C-reactive Protein), radiological progression and treatment adverse events. Results: Leflunomide therapy was demonstrated to be significantly superior to placebo in relation to the efficacy outcome measures and it slowed the radiological progression of patients’ disease in three studies. Treatment success and duration of sustained response were also significantly superior than on placebo, as were quality of life measures. Leflunomide treatment was comparable to sulphasalazine and methotrexate with respect to efficacy, radiological progression and quality of life measures. The most common adverse effects leading to withdrawal from leflunomide treatment were gastrointestinal symptoms (diarrhoea and nausea), allergic reactions (rash and pruritus), alopecia, dyspepsia, hypertension and elevated transaminase levels. Weight loss and dizziness have also been reported for leflunomide therapy. Leflunomide is more expensive than most DMARDs, costing about £400 a year more than sulphasalazine. Conclusion: Despite the small number of published articles relating to leflunomide treatment, the evidence suggests that leflunomide is similar in efficacy to both sulphasalazine and methotrexate, although with a differential pattern of side-effects. There is a need for further research to assess the long-term outcomes of leflunomide treatment.

The surgical management of a massive traumatic false aneurysm of the junction of the right subclavian artery with the innominate artery following a stab wound four months earlier is described. Surgical alternatives are discussed.

Aim The aim of this study was to review the management of children with Wilms' tumour who have intracardiac extension. Patients and Methods Data were collected from patient notes regarding presentation, operative details, and outcome. Results From 1984 through 2011, 264 children with Wilms' tumour were treated at our hospital. Nine (3.4%) had cavo-atrial extension of the tumour thrombus. The thrombus extended into the right ventricle in two children and involved the hepatic veins in both, and also two others. Pre-operative chemotherapy was administered in eight children with complete regression of the intra-cardiac tumour thrombus in two cases. One child died preoperatively of septicaemia and respiratory failure after two doses of chemotherapy. Six children with intra-cardiac tumour were operated on under cardiopulmonary bypass (CPB) with deep hypothermia and circulatory arrest (DHCA). The mean ischemic time was 30min. There was one peri-operative death in a child with hepatic vein involvement and Budd–Chiari syndrome. All others made a good postoperative recovery. All tumours were favourable histology. To date four children are still alive and disease free. Three children have died as a result of pulmonary metastases. Conclusion Intracardiac extension of Wilms' tumour is rare, and the management is technically challenging. Pre-operative chemotherapy is effective. CPB and DHCA for excision of the cavo-atrial tumour thrombus may be necessary. Distant metastatic disease is common and determines long term prognosis. Hepatic vein extension complicates surgery and remains challenging.

1. Introduction Ulla Grapard and Gillian Hewitson Part 1: The Robinsonades: The Development of the Unencumbered Rational Economic Man 2. Reading and Rewriting: The Production of an Economic Robinson Crusoe Michael V. White 3. Robinson Crusoe and the Secret of Primitive Accumulation Stephen Hymer 4. Robinson Crusoe and the Economists William S. Kern 5. Robinson Crusoe and the Subject of Economics Antonio Callari Part 2: The Quintessential Rational Economic Man: Feminist Interrogations 6. Robinson Crusoe: The Quintessential Economic Man? Ulla Grapard 7. Robinson Crusoe: The Paradigmatic 'Rational Economic Man' Gillian J. Hewitson 8. Family Troubles Brian Cooper 9. Economic Man Lost in Space Ulla Grapard and Gillian Hewitson 10. Robinson Crusoe and the Female Goddesses of Disorder Christine Owen Part 3: The Quintessential Rational Economic Man: Postcolonial Interrogations 11. Towards a Friday Model of International Trade Melanie Samson 12. Mercantilism and Criminal Transportation Anna Neill 13. What Would an African Student make of Robinson Crusoe? Eiman Zein-Elabdin 14. J.M. Coetzee's Foe: the 'Amazement of Reading' Nicole Bracker

Application of the University of Wisconsin cold storage solution has rapidly expanded to include medium-term to long-term preservation of virtually all intraabdominal organs. Its use in intrathoracic organ transplantation has also been suggested. We therefore examined the efficacy of the University of Wisconsin solution in a primate allotransplantation model for preservation of hearts, and as a simple single-solution system for static preservation of heart-lung blocks, for periods of ischemia ranging from 6 to 24 hours. For comparison, we employed the histidine-tryptophane-ketoglutarate cardioplegic solution of Bretschneider. University of Wisconsin solution provided superior results with regard to clinical outcome and hemodynamic recovery of hearts after ischemic periods of up to 16 hours. This was in contrast to Bretschneider's solution, which allowed storage of hearts for periods of only up to 10 hours. Heart-lung blocks were equally well preserved with either University of Wisconsin or Bretschneider's solution after 6 to 12 hours, although the University of Wisconsin solution group exhibited a more notable increase in pulmonary water content. This was in accordance with histological data, which suggested that, although hemodynamic recovery of hearts stored for periods longer than 10 hours was poor, preservation of pulmonary ultrastructure was far superior using Bretschneider's solution as compared with University of Wisconsin solution after an ischemic period of up to 16 hours.

The Budd-Chiari syndrome (BCS), characterized by hepatic venous outflow occlusion, is rare in children. We describe the cases of 3 patients with BCS who presented at different stages of liver dysfunction that required tailored management. A 10-year-old boy who presented with a weeklong history of abdominal discomfort received an early diagnosis. Treatment with thrombolytic therapy and adjunctive percutaneous interventional radiologic management led to a favorable outcome in this patient. A 2-year-old girl referred with a 6-week illness not amenable to thrombolytic therapy was managed with a pericardial patch atriocavoplasty to restore hepatic venous outflow tract patency. The third patient, a 5-year-old girl, presented with established cirrhosis and massive ascites. After we controlled the ascites with an extracorporeal shunt system and nutritional resuscitation, we conducted an orthotopic liver transplantation on this patient. The stage of disease at diagnosis influences the management strategy, and an early diagnosis offers patients the best possible chance of cure without major surgery. However, a later presentation can still be successfully managed by more radical surgical interventions. The aims of this case report were to emphasize the importance of a high index of suspicion in the diagnosis of pediatric BCS and to highlight the importance of early referral to a specialized pediatric liver unit with the necessary facilities to deal with the medical and surgical aspects of management.

About 1 in 1,000 children has Down syndrome. Extra chromosomal material results in a myriad of potential problems for the affected individual. About 40% of Down syndrome children will have cardiac abnormalities, ranging from the simple arterial duct to the complex atrioventricular septal defect. Virtually all these defects are amenable to surgical correction and extended survival is possible. In South Africa many of these children do not undergo cardiac surgery.

To compare children with Down syndrome and children without Down syndrome and investigate whether there is a significant difference in the burden that is placed on the health care system between these two groups only in respect of the repair of congenital heart disease at Red Cross War Memorial Children's Hospital, Cape Town, South Africa.This study is a retrospective case control review.Red Cross War Memorial Children's Hospital, Cape Town, South Africa.The sample group of 50 Down syndrome children who had received cardiac surgery between January 1998 and June 2003 was compared with a control group of 50 nonsyndromic children who had received cardiac surgery during the same period.Sex and diagnoses (cardiac and noncardiac), number of days spent in hospital and in ICU, complication rates, re-operation rates, early mortality rates, planned further cardiac surgery. Costs of these outcomes were not quantified in exact monetary terms.There was no significant difference between the two groups in terms of the burden that was placed on the health care system. Similar complication rates, re-operation rates and early mortality rates were recorded for both groups. The Down syndrome group appeared to benefit more from cardiac surgery than the non-Down syndrome group.Denying cardiac surgery to children with Down syndrome does not improve the efficiency of resource allocation. It is therefore not reasonable to suggest that the problem of scarce resources can be ameliorated by discriminating against children with Down syndrome.

Aims: To describe acid–base derangements in children following open cardiac surgery on cardiopulmonary bypass (CPB), using the Fencl–Stewart strong ion approach. Methods: Prospective observational study set in the paediatric intensive care unit (PICU) of a university children's hospital. Arterial blood gas parameters, serum electrolytes, strong ion difference, strong ion gap (SIG), and partitioned base excess (BE) were measured and calculated on admission to PICU. Results: A total of 97 children, median age 57 months (range 0.03–166), median weight 14 kg (range 2.1–50), were studied. Median CPB time was 80 minutes (range 17–232). Predicted mortality was 2% and there was a single non-survivor. These children showed mild metabolic acidosis (median standard bicarbonate 20.1 mmol/l, BE –5.1 mEq/l) characterised by hyperchloraemia (median corrected Cl 113 mmol/l), and hypoalbuminaemia (median albumin 30 g/l), but no significant excess unmeasured anions or cations (median SIG 0.7 mEq/l). The major determinants of the net BE were the chloride and albumin components (chloride effect –4.8 mEq/l, albumin effect +3.4 mEq/l). Metabolic acidosis occurred in 72 children (74%) but was not associated with increased morbidity. Hyperchloraemia was a causative factor in 53 children (74%) with metabolic acidosis. Three (4%) hyperchloraemic children required adrenaline for inotropic support, compared to eight children (28%) without hyperchloraemia. Hypoalbuminaemia was associated with longer duration of inotropic support and PICU stay. Conclusions: In these children with low mortality following open cardiac surgery, hypoalbuminaemia and hyperchloraemia were the predominant acid–base abnormalities. Hyperchloraemia was associated with reduced requirement for adrenaline therapy. It is suggested that hyperchloraemic metabolic acidosis is a benign phenomenon that should not prompt escalation of haemodynamic support. By contrast, hypoalbuminaemia, an alkalinising force, was associated with prolonged requirement for intensive care.

Background: Alternatives to allogeneic blood transfusion exist and are being used to varying extents in Australian hospitals. Evidence on effectiveness and cost-effectiveness is generally inconclusive and provides a suboptimal basis for policy development. Aim: To describe the influences on uptake of transfusion technologies as perceived by national and institutional stakeholders. Methods: Qualitative interview study. Interview transcripts were coded and analysed independently by at least two researchers. Participants had opportunity to comment on their transcript and the manuscript. Results: A total of 71 interviews were conducted with representatives of the media, specialist medical societies, consumer special interest groups, the Australian Red Cross Blood Service (ARCBS), government, private health insurers, technology manufacturers, prominent clinicians in the area and a sample of clinicians drawn from hospitals with variable use of blood-saving technologies. Technical advances and acceptance of lower transfusion triggers were identified as the main influences on the decrease in use of allogeneic blood transfusion in the past decade. Participants indicated that patients were most aware and supportive of autologous predonation. Participants noted that 'enthusiasts' were involved in educating about the need for alternatives, negotiating resourcing and maintaining the use of a technology. Funding mechanisms were seen as main barriers to use of alternatives. A discrepancy was noted in the rigour of evaluation and regulation of pharmaceuticals and devices/procedures. Conclusions: Uptake of blood transfusion technologies by institutions was dependent mostly on funding arrangements and the presence of an 'enthusiast'. Critical review of the evidence for effectiveness or cost-effectiveness of these technologies was rarely mentioned. Opportunities exist for evidence-based medicine principles to play a greater role in policy decisions in this area.

Objecti6e: Pulmonary artery banding remains a palliative option for patients with congenital heart disease and excessive pulmonary blood flow, if there is unfavourable anatomy or frail condition. In contrast to more developed countries, our patients at Red Cross Children’s Hospital, Cape Town, often present to medical services late and in poor nutritional condition. We retrospectively reviewed patients undergoing pulmonary artery banding to determine major variables that influenced long-term outcome. Methods: In a 10-year period ending June 1992, 135 consecutive patients underwent pulmonary artery banding; 89 with ventricular septal defect type non-mixing disorders, and 46 with mixing or complex disorders. The median age was 3.0 months and weight 3.5 kg with 74.8% of patients weighing less than the third percentile (NCHS adapted), and 39.3% had an additional serious medical illness. Results: Pulmonary banding mortality was 8.1%, and was higher in neonates (22.2%, P 0.04) but was not related to congenital disorder, associated medical illness, or associated coarctation or interrupted aortic arch. The pulmonary band was inadequate at follow-up in 28.9%, which occurred more commonly if banding was necessary before 3 months of age (41.5%, P0.003) but was not related to weight, congenital disorder or associated respiratory infection. Sixty patients (44.4%) have now proceeded to definitive repair with a mortality of 23.3%, which was increased if the pulmonary band was inadequate at the time of definitive repair (44.4%; P0.02), but was not related to the congenital disorder. Conclusions: An inadequate pulmonary artery band adversely affects outcome and demands further aggressive management prior to definitive repair. Copyright © 1997 Elsevier Science B.V.

Aims Prolonged jaundice in babies is common and usually harmless but a very small number of cases have serious pathology, such as biliary atresia, in which early detection is vital. Many “well” babies with prolonged jaundice undergo extensive investigations with a very low yield of important positive results. Our aim was to assess the safety, efficiency and cost effectiveness of a nurse-led prolonged jaundice clinic which has been in operation at our district general hospital for ten years, performing minimal investigations compared to standard practise. Methods We collected data retrospectively for all babies Results 91% (176) of 194 babies were managed in the nurse led prolonged jaundice clinic; 5.6% (10) had additional investigations which identified one case with significant pathology. Of the 9% (18) of babies seen elsewhere by doctors (A&E/outpatients/inpatients/daycare), 77% (14) had additional investigations. 3 babies had serious pathology including one case of biliary atresia which presented late (48 days old). Conclusion For the last ten years we have managed the vast majority of babies with prolonged jaundice in our nurse-led clinic, successfully avoiding unnecessary, time-consuming and expensive investigations for well babies whilst promptly identifying cases with serious pathology. Our district general hospital has recently merged with two local community health services to form an Integrated Care Organisation, providing a unique opportunity to move the nurse-led prolonged jaundice clinic into the community. Based on our well established model, such clinics could be conducted in local health centres by midwives or community paediatric nurses, supervised by a consultant paediatrician. This would allow further cost savings and be more convenient for families.

Twenty-eight patients were treated for acute blunt thoracic aortic rupture at Groote Schuur Hospital between January 1984 and March 1994. Aortic arch ruptures occurred in 2 patients and were successfully repaired by means of hypothermic circulatory arrest. Descending aortic ruptures were repaired more safely by insertion of an interposition graft as opposed to direct suture reapproximation, and with the aid of partial heparinless bypass as opposed to simple aortic cross-clamping.

A 2 year 8 month old girl with tetralogy of Fallot and a right modified Blalock-Taussig shunt was seen in January of 1998 with fever of a month’s duration. The shunt had been created 11 months previously following a life threatening hypercyanotic spell. She had the physical signs …

South Africa has failed to treat its children well, as demonstrated by our failure to achieve adequate progress towards the Millennium Development Goal of reducing the under-5 mortality rate, doing far worse than many poorer countries.

This ground-breaking new book will be essential reading for scholars of feminist economics and women’s studies.