Scielo RSS <![CDATA[SAMJ: South African Medical Journal]]> http://www.scielo.org.za/rss.php?pid=0256-957420140011&lang=es vol. 104 num. 11 lang. es <![CDATA[SciELO Logo]]> http://www.scielo.org.za/img/en/fbpelogp.gif http://www.scielo.org.za <![CDATA[<b>Mediation - an alternative to litigation in medical malpractice</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100001&lng=es&nrm=iso&tlng=es <![CDATA[<b>CME: Immunology</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100002&lng=es&nrm=iso&tlng=es <![CDATA[<b>Bureaucracy and clinical performance</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100003&lng=es&nrm=iso&tlng=es <![CDATA[<b>A puzzling case of cryptococcal meningitis</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100004&lng=es&nrm=iso&tlng=es <![CDATA[<b>Dr Mary Gordon</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100005&lng=es&nrm=iso&tlng=es <![CDATA[<b>Ebola global response: 'not in my back yard'</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100006&lng=es&nrm=iso&tlng=es <![CDATA[<b>New editor for SA Journal of Obstetrics and Gynaecology</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100007&lng=es&nrm=iso&tlng=es <![CDATA[<b>Emergency care doctors - 'off-task and in the wrong places'</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100008&lng=es&nrm=iso&tlng=es <![CDATA[<b>The smell of coffee, blood and disinfectant...</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100009&lng=es&nrm=iso&tlng=es <![CDATA[<b>Discard the placenta at your peril, pathologist warns doctors</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100010&lng=es&nrm=iso&tlng=es <![CDATA[<b>New editor for SA Journal of Child Health</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100011&lng=es&nrm=iso&tlng=es <![CDATA[<b>Principles of Medicine in Africa</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100012&lng=es&nrm=iso&tlng=es <![CDATA[<b>Creative Arts in Humane Medicine</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100013&lng=es&nrm=iso&tlng=es <![CDATA[<b>Blood-borne infections in healthcare workers in South Africa</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100014&lng=es&nrm=iso&tlng=es The risks associated with infection of healthcare workers and students with blood-borne pathogens, specifically HIV, hepatitis B virus and hepatitis C virus, are often neglected. South Africa (SA) currently has no official policies or guidelines in place for the prevention and management of these infections. This article reviews the available data and international guidelines with regard to infected healthcare practitioners and makes minimum recommendations for the SA setting. <![CDATA[<b>Impressions of defensive medical practice and medical litigation among South African neurosurgeons</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100015&lng=es&nrm=iso&tlng=es From a litigation perspective, neurosurgery is considered a 'super high-risk' field, and this has been associated with rapidly increasing malpractice cover costs. In 2013 the annual Medical Protection Society fee for cover was R250 900. We wished to determine whether high malpractice cover was influencing how neurosurgeons managed patients. A 40-question online survey asking questions on defensive medicine was distributed to determine perceptions around liability risk and whether these influenced how patients were managed. Eighty-four per cent of respondents agreed that a medicolegal crisis existed, and over half (53.8%) had been sued for malpractice during their career. Altering practice behaviour to minimise the risk of a lawsuit is common. The increasing number of legal claims against respondents in this survey has resulted in most neurosurgeons practising defensive medicine. Arguably this will result in increased healthcare costs, inferior patient care and decreased access to skilled surgeons. <![CDATA[<b>The impact of the Consumer Protection Act on pharmacists</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100016&lng=es&nrm=iso&tlng=es The Consumer Protection Act of 2008 has had far-reaching consequences for suppliers of goods and services in South Africa. The implementation of the Act has important implications for all suppliers who enter into 'consumer transactions'. This article aims to stimulate awareness of the legal consequences of the Act arising from day-to-day situations occurring in the pharmacy, and to highlight the compliance obligations that the Act creates for pharmacists. <![CDATA[<b>Interference with the clinical independence of doctors in hospitals faced with a shortage of resources: What should doctors do?</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100017&lng=es&nrm=iso&tlng=es In the face of interference with their clinical independence in hospitals with a shortage of resources, what should doctors do? The question can be answered by considering: (i) the constitutional right to healthcare and emergency treatment; (ii) the common-law position regarding unlawful homicide and the doctrine of 'superior orders'; (iii) the ethical rules of the Health Professions Council of South Africa; and (iv) whether there is any protection for doctors who refuse to carry out unprofessional, unethical or unlawful directives from their superiors. While this article focuses on the public sector, some of the legal principles, where relevant, apply equally to doctors in the private sector. <![CDATA[<b>Recommendations for the management of sickle cell disease in South Africa</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100018&lng=es&nrm=iso&tlng=es The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (i) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subjects with sickle cell trait are largely asymptomatic. SCD is characterised by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges. <![CDATA[<b>Public somnambulism: a general lack of awareness of the consequences of increasing medical negligence litigation</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100019&lng=es&nrm=iso&tlng=es The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (i) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subjects with sickle cell trait are largely asymptomatic. SCD is characterised by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges. <![CDATA[<b>Ebola virus disease in West Africa - South African perspectives</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100020&lng=es&nrm=iso&tlng=es The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (i) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subjects with sickle cell trait are largely asymptomatic. SCD is characterised by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges. <![CDATA[<b>Compensation for research injuries: Thoughts from a human research ethics committee chair</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100021&lng=es&nrm=iso&tlng=es The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (i) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subjects with sickle cell trait are largely asymptomatic. SCD is characterised by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges. <![CDATA[<b>Who will guard the guards? Medical leadership and conflict of interest in South African healthcare</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100022&lng=es&nrm=iso&tlng=es The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (i) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical disease of varying severity that is influenced by biological and environmental factors, whereas subjects with sickle cell trait are largely asymptomatic. SCD is characterised by vaso-occlusive episodes that result in tissue ischaemia and pain in the affected region. Repeated infarctive episodes cause organ damage and may eventually lead to organ failure. For effective management, regular follow-up with support from a multidisciplinary healthcare team is necessary. The chronic nature of the disease, the steady increase in patient numbers, and relapsing acute episodes have cost implications that are likely to impact on provincial and national health budgets. Limited resources mandate local management protocols for the purposes of consistency and standardisation, which could also facilitate sharing of resources between centres for maximal utility. These recommendations have been developed for the South African setting, and it is intended to update them regularly to meet new demands and challenges. <![CDATA[<b>Compensation for research-related harm: the implications of <i>Venter v Roche products (Pty) Limited and Others</i> for research ethics committees</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100023&lng=es&nrm=iso&tlng=es BACKGROUND: The issue of what type of compensation a research participant would be entitled to in a clinical trial when they have signed an informed consent document excluding certain forms of compensation recently came before our courts in the matter of Venter v Roche Products (Pty) Limited and Others (Case No. 12285/08). In this case, the court had to consider whether the plaintiff, Mr Venter, was entitled to claim for non-medical costs such as pain and suffering, loss of income and general damages, even though the informed-consent document expressly excluded such claims. OBJECTIVES: To set out the facts, issues and judgment in the case, concluding with a discussion of the implications of the judgment for research ethics committees (RECs). METHODS: Critical review of a judgment of the Western Cape High Court. RESULTS: The court concluded that Mr Venter's application for damages should be dismissed because he had voluntarily agreed to the limited compensation as set out in the informed consent form that had been approved by both the local RECs and the Medicines Control Council. CONCLUSIONS: The Venter case has shown that delictual claims for research-related injuries will not be successful if plaintiffs have agreed to limit their own rights through signing an informed-consent form that limits compensation. This places an important obligation on RECs to ensure that they carefully review compensation clauses in informed-consent documents and that these are made clear to potential research participants. <![CDATA[<b>Appropriateness of computed tomography and magnetic resonance imaging scans in the Eden and Central Karoo districts of the Western Cape Province, South Africa</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100024&lng=es&nrm=iso&tlng=es INTRODUCTION: Computed tomography (CT) and magnetic resonance imaging (MRI) are an essential part of modern healthcare. Marked increases in clinical demand for these imaging modalities are straining healthcare expenditure and threatening health system sustainability. The number of CT and MRI scans requested in the Eden and Central Karoo districts of the Western Cape Province, South Africa (SA), almost doubled from 2011 to 2013 OBJECTIVE: To determine the appropriateness of CT and MRI scans and relate this to the requesting department and clinician METHODS: This was a retrospective analytical cohort study. All scans during October 2012 were analysed as a sample. Appropriateness of scans was determined using the American College of Radiologists (ACR) Appropriateness Criteria and the Royal College of Radiology Guidelines. Appropriateness was also correlated back to the requesting department and clinician RESULTS: Of a total of 219 scans, 53.0% were abnormal. Overall 6.4% of scans were considered inappropriate. Interns and registrars requested no inappropriate scans. The orthopaedics department scored the highest rate of appropriate scans (80.0%) and the oncology department the highest rate of inappropriate scans (20.8%) CONCLUSION: The limited resources available for healthcare in a developing country like SA should be a motivation to implement control mechanisms aimed at appropriate utilisation of imaging examinations. The Eden and Central Karoo districts have a low rate of inappropriate scans (6.4%). We recommend that the current preauthorisation system by consultants and other senior clinicians continues, but with increased clinician awareness of the ACR Appropriateness Criteria and the Royal College guidelines <![CDATA[<b>A laboratory-based study to identify and speciate non-tuberculous mycobacteria isolated from specimens submitted to a central tuberculosis laboratory from throughout KwaZulu-Natal Province, South Africa</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100025&lng=es&nrm=iso&tlng=es BACKGROUND: Non-tuberculous mycobacteria (NTM) are important environmental pathogens capable of causing a spectrum of infection. The different species exhibit varied geographical prevalence worldwide. Identification of the infecting organism may be helpful in determining the clinical significance of the isolate OBJECTIVE: To describe the spectrum of NTM isolated from clinical specimens received at the National Health Laboratory Service central tuberculosis laboratory in KwaZulu-Natal Province, South Africa METHOD: In a laboratory-based prospective study, 200 suspected NTM were randomly selected over a period of 1 year and identified to species level using a commercially available DNA strip assay (GenoType Mycobacterium, CM/AS; Hain Lifescience, Germany RESULTS: Of the 200 suspected NTM, 133 (66.5%) were confirmed to be NTM by the molecular test. The most frequently isolated NTM species were Mycobacterium intracellulare (45.9%), M. avium subspecies (11.3%), M. gordonae (6.0%) and M. kansasii (4.5% CONCLUSION: It is important for laboratories to document the local spectrum of NTM because of the geographical variation in the different NTM species isolated. Although molecular tests for identifying NTM are relatively expensive, they have the advantage of providing rapid and accurate identification of the various NTM species <![CDATA[<b>Determining need for hospitalisation: Evaluation of the utility of the CRB-65 score in patients with community-acquired pneumonia presenting to an emergency department</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100026&lng=es&nrm=iso&tlng=es BACKGROUND: The CRB-65 severity of illness score, used for assessing patients with community-acquired pneumonia (CAP), may be of particular benefit in resource-constrained areas, since it relies purely on clinical parameters OBJECTIVE: To assess the potential accuracy of the CRB-65 score when used in deciding whether to hospitalise patients with CAP presenting to an emergency department (ED METHODS: Prospective, observational study in an academic hospital in Johannesburg, South Africa. Data from adult patients with radiologically confirmed CAP were analysed RESULTS: Overall, 152 patients were enrolled (79 females, 73 males; median age 36.5 years). Several diverse criteria had been used by the ED doctors in admission decisions, while the CRB-65 score had been used in only 3/152 patients (1.6%). Overall, 68/152 patients (44.7%) had been managed as inpatients and 84/152 (55.3%) as outpatients. If the CRB-65 had been used as the sole criterion for site-of-care decisions, 107/152 patients (70.4%) would potentially have been managed as outpatients and 45/152 (29.6%) as inpatients. Achieving a stable clinical condition took longer (p=0.037) and mortality was higher (p<0.001) in patients with higher than lower CRB-65 scores. All five patients who died were inpatients. Of these, three (60.0%) would have been classified by the CRB-65 as having an intermediate mortality risk and two (40.0%) as having a high mortality risk CONCLUSIONS: This study demonstrates the utility of the CRB-65 score in accurately determining the need for admission of patients with CAP presenting to an ED in a resource-constrained environment <![CDATA[<b>The association of <i>khat (Catha edulis)</i> chewing and orodental health: A systematic review and meta-analysis</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100027&lng=es&nrm=iso&tlng=es BACKGROUND: It has been claimed that chewing khat (Catha edulis), a plant common in parts of eastern and southern Africa and the Arabian Peninsula, is associated with a range of orodental problems OBJECTIVE: To provide a synthesis of the evidence on the association between khat chewing and orodental health METHOD: A systematic review and meta-analysis of studies that reported on the association of khat chewing and outcomes related to orodental health identified through a systematic search using web-based electronic search engines RESULTS: Nineteen studies were found suitable for this review. Of these, between two and five (based on the type of outcome measured) were suitable for meta-analysis. The rest were used only for qualitative synthesis. A meta-analysis of the association of khat chewing with mucosal white lesions, gum recession, periodontal pocketing and gum bleeding showed that chewing increased the odds of the respective oral problems. However, qualitative synthesis of the findings on the effect of khat chewing on oral micro-organisms showed no evidence that the practice favours the presence of pathogenic micro-organisms in the oral cavity - instead, it seems to favour the proliferation of micro-organisms compatible with orodental health CONCLUSION: Khat chewing is associated with adverse orodental health outcomes. While literature on the topic is scarce and there is a need for generation of more evidence from different countries, on the basis of the evidence accumulated to date, public health officials and health practitioners should consider khat a threat to orodental health and take appropriate action <![CDATA[<b>Duchenne muscular dystrophy: High-resolution melting curve analysis as an affordable diagnostic mutation scanning tool in a South African cohort</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100028&lng=es&nrm=iso&tlng=es BACKGROUND: Duchenne/Becker muscular dystrophy (D/BMD) is an X-linked recessive muscle disorder affecting 1/3 500 live male births worldwide. Up to 70% of all D/BMD cases are caused by exonic deletions or duplications routinely identified in diagnostic laboratories worldwide. The remaining patients harbour other sequence alterations for which testing availability is limited owing to the expense of interrogating the large DMD gene. Genetic screening for D/BMD in South Africa currently includes multiple ligase-dependent probe amplification (MLPA) for exonic deletions and duplications and linkage analysis. No genetic testing for small mutations in the DMD gene is offered, leaving a third of D/BMD families without genetic closure. The advent of potential mutation-specific therapies for DMD necessitates comprehensive testing protocols OBJECTIVE: To investigate the effectiveness and affordability of high-resolution melting curve analysis (hrMCA) for detection of small/point mutations in the DMD gene, for possible inclusion into the local public health-funded diagnostic service METHODS: DNA from 24 patients who had previously tested deletion-negative with multiplex polymerase chain reaction (mPCR) was analysed by MLPA and hrMCA RESULTS: MLPA revealed eight previously undetected exonic rearrangements: five deletions and three duplications. HrMCA of the remaining samples revealed three nonsense, four frameshifts, one splice-site, one missense and one single-base substitution in the Dp427promoter/exon1 of the DMD gene. In addition, 41 polymorphisms and three changes of uncertain significance were detected CONCLUSION: These findings identify hrMCA as an affordable and effective mutation scanning tool for incorporation into the local diagnostic setting, allowing for better genetic counselling of more DMD families and selection of potential candidates for future therapies <![CDATA[<b>Immunology as a medical discipline in South Africa: Why, how and what form?</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100029&lng=es&nrm=iso&tlng=es BACKGROUND: Duchenne/Becker muscular dystrophy (D/BMD) is an X-linked recessive muscle disorder affecting 1/3 500 live male births worldwide. Up to 70% of all D/BMD cases are caused by exonic deletions or duplications routinely identified in diagnostic laboratories worldwide. The remaining patients harbour other sequence alterations for which testing availability is limited owing to the expense of interrogating the large DMD gene. Genetic screening for D/BMD in South Africa currently includes multiple ligase-dependent probe amplification (MLPA) for exonic deletions and duplications and linkage analysis. No genetic testing for small mutations in the DMD gene is offered, leaving a third of D/BMD families without genetic closure. The advent of potential mutation-specific therapies for DMD necessitates comprehensive testing protocols OBJECTIVE: To investigate the effectiveness and affordability of high-resolution melting curve analysis (hrMCA) for detection of small/point mutations in the DMD gene, for possible inclusion into the local public health-funded diagnostic service METHODS: DNA from 24 patients who had previously tested deletion-negative with multiplex polymerase chain reaction (mPCR) was analysed by MLPA and hrMCA RESULTS: MLPA revealed eight previously undetected exonic rearrangements: five deletions and three duplications. HrMCA of the remaining samples revealed three nonsense, four frameshifts, one splice-site, one missense and one single-base substitution in the Dp427promoter/exon1 of the DMD gene. In addition, 41 polymorphisms and three changes of uncertain significance were detected CONCLUSION: These findings identify hrMCA as an affordable and effective mutation scanning tool for incorporation into the local diagnostic setting, allowing for better genetic counselling of more DMD families and selection of potential candidates for future therapies <![CDATA[<b>Superheroes in autoimmune warfare: Biologic therapies in current South African practice</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100030&lng=es&nrm=iso&tlng=es Biologic drugs targeting immune cells or cytokines underlying systemic inflammation have dramatically improved outcomes in patients with rheumatological and autoimmune diseases. Nine biologic drugs are currently available in South Africa (SA) - all showing good efficacy and safety profiles. Their high cost and potential adverse events preclude them from being used as first-line agents. They are therefore indicated for severe disease refractory to standard therapies, and their use must be initiated by a specialist. The most important adverse effect of this class of drugs is infection and, in SA, tuberculosis is of particular concern. As new targets in the immune system are identified, new biologics will be developed. The current challenges are to optimise standard care for all patients with autoimmune diseases, and to offer the appropriate biologic to patients with refractory disease. <![CDATA[<b>Investigation of adult immunodeficiency and indications for immunoglobulin replacement therapy</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100031&lng=es&nrm=iso&tlng=es Biologic drugs targeting immune cells or cytokines underlying systemic inflammation have dramatically improved outcomes in patients with rheumatological and autoimmune diseases. Nine biologic drugs are currently available in South Africa (SA) - all showing good efficacy and safety profiles. Their high cost and potential adverse events preclude them from being used as first-line agents. They are therefore indicated for severe disease refractory to standard therapies, and their use must be initiated by a specialist. The most important adverse effect of this class of drugs is infection and, in SA, tuberculosis is of particular concern. As new targets in the immune system are identified, new biologics will be developed. The current challenges are to optimise standard care for all patients with autoimmune diseases, and to offer the appropriate biologic to patients with refractory disease. <![CDATA[<b>Investigation and management of primary immunodeficiency in South African children</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100032&lng=es&nrm=iso&tlng=es Biologic drugs targeting immune cells or cytokines underlying systemic inflammation have dramatically improved outcomes in patients with rheumatological and autoimmune diseases. Nine biologic drugs are currently available in South Africa (SA) - all showing good efficacy and safety profiles. Their high cost and potential adverse events preclude them from being used as first-line agents. They are therefore indicated for severe disease refractory to standard therapies, and their use must be initiated by a specialist. The most important adverse effect of this class of drugs is infection and, in SA, tuberculosis is of particular concern. As new targets in the immune system are identified, new biologics will be developed. The current challenges are to optimise standard care for all patients with autoimmune diseases, and to offer the appropriate biologic to patients with refractory disease. <![CDATA[<b>Advances in the diagnosis and management of allergic disease: applications to South African practice</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100033&lng=es&nrm=iso&tlng=es There have been a number of advances in the diagnosis and management of allergic diseases that are relevant to South African (SA) circumstances. These are all published or about to be published in new guidelines that provide practical advice to guide SA doctors who treat patients with these conditions. The guidelines include those for atopic dermatitis, allergic rhinitis and food allergy. This article reflects the most pertinent aspects of the guidelines. It also provides a short summary of a new allergy diagnostic test available in SA, the multiplex microarray chip, known as the immuno-solid-phase allergen chip (ISAC) test. It provides component-resolved allergy testing for special circumstances and complex allergic problems and is certainly not required as a screening allergy test. Finally, this article gives an update on allergen immunotherapy - some patients with allergic conditions may benefit from immunotherapy. In SA, some forms of immunotherapy for allergic rhinitis and mild asthma may currently include sublingual immunotherapy. <![CDATA[<b>Immune tolerance and immunosuppression in solid organ transplantation</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100034&lng=es&nrm=iso&tlng=es Organ transplantation is the treatment of choice for patients with end-stage organ failure. Most of them will require lifelong immunosuppression to prevent both acute and chronic rejection. T-cell recognition of the allograft major histocompatibility complex antigens is the central event initiating cellular rejection of the allograft, and subsequent full T-cell activation requires three signals. Immunosuppressive regimens currently used in clinical practice are nonspecific and target T-cell activation, clonal expansion or differentiation into effector T cells. While these therapeutic regimens have advanced considerably and one-year graft survival figures for most solid organ transplants (SOTs) are >90%, the long-term graft survival remains fair owing to graft loss from chronic rejection. The 'holy grail' of SOT is therefore the development of a permanent specific immune tolerance against donor allogeneic antigens without the long-term use of immunosuppression. <![CDATA[<b>Recommendations for the use of immunoglobulin therapy for immunomodulation and antibody replacement</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100035&lng=es&nrm=iso&tlng=es Polyvalent immunoglobin, derived from pooled human plasma, can be administered via the intravenous, subcutaneous or intramuscular route. Therapy is standard of care in the treatment of a number of immune-mediated pathologies across disciplines. By volume, the majority is used in neurology (~40%). In primary immunodeficiencies, therapy reconstitutes humoral immunity at replacement doses (0.4 - 0.6 g/kg/month), decreasing infections, and is usually lifelong. However, high doses, usually 2 g/kg total dose over five days, are required for immunomodulation in autoimmune and inflammatory indications. A high-quality evidence base supports use in primary antibody failure, Guillain-BarrĂ© syndrome, chronic inflammatory demyelinating polyneuropathy, acute idiopathic thrombocytopenia, Kawasaki disease and immunobullous diseases. Low-quality evidence shows benefit in many other uncommon autoimmune and immunodeficient conditions. In South Africa, use of immunoglobulin therapy is restricted and, given the cost involved, will likely remain so. Therefore, the incremental benefit over other forms of immunosuppression, particularly corticosteroids, must be assessed carefully on a case-by-case basis. In most cases, therapy will be second-line or 'rescue' and motivation will be required. This short review aims to provide clinicians with the necessary understanding of the therapy, general considerations for use, and evidence base and quality thereof for well-established indications. <![CDATA[<b>Liver disease in children: From neonatal jaundice to living donor liver transplantation</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100036&lng=es&nrm=iso&tlng=es Polyvalent immunoglobin, derived from pooled human plasma, can be administered via the intravenous, subcutaneous or intramuscular route. Therapy is standard of care in the treatment of a number of immune-mediated pathologies across disciplines. By volume, the majority is used in neurology (~40%). In primary immunodeficiencies, therapy reconstitutes humoral immunity at replacement doses (0.4 - 0.6 g/kg/month), decreasing infections, and is usually lifelong. However, high doses, usually 2 g/kg total dose over five days, are required for immunomodulation in autoimmune and inflammatory indications. A high-quality evidence base supports use in primary antibody failure, Guillain-BarrĂ© syndrome, chronic inflammatory demyelinating polyneuropathy, acute idiopathic thrombocytopenia, Kawasaki disease and immunobullous diseases. Low-quality evidence shows benefit in many other uncommon autoimmune and immunodeficient conditions. In South Africa, use of immunoglobulin therapy is restricted and, given the cost involved, will likely remain so. Therefore, the incremental benefit over other forms of immunosuppression, particularly corticosteroids, must be assessed carefully on a case-by-case basis. In most cases, therapy will be second-line or 'rescue' and motivation will be required. This short review aims to provide clinicians with the necessary understanding of the therapy, general considerations for use, and evidence base and quality thereof for well-established indications. <![CDATA[<b>Paediatric liver transplantation in Johannesburg revisited: 59 transplants and challenges met</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100037&lng=es&nrm=iso&tlng=es BACKGROUND: A paediatric liver transplant programme was started at the Wits Donald Gordon Medical Centre, Johannesburg, South Africa (SA), in November 2005. We reported on the first 29 patients in 2012. Since then we have performed a further 30 transplants in 28 patients, having met the major challenge of donor shortage by introducing a living related donor programme and increasing the use of split liver grafts. OBJECTIVE:To review the Wits Donald Gordon Medical Centre paediatric liver transplant programme to date. We describe how the programme has evolved and specifically compare the outcomes of the first cohort with the most recent 28 patients METHODS: Case notes of all paediatric liver transplants performed between 14 November 2005 and 30 June 2014 were retrospectively reviewed. Data were analysed for age and weight at transplantation, indication and type of graft. Morbidity and mortality were documented, specifically biliary and vascular complications. Comparison was made between Era 1 (November 2005 - October 2012) and Era 2 (November 2012 - June 2014 RESULTS: A total of 59 transplants were performed in 57 patients. Age at transplantation ranged from 9 months to 213 months (mean 82.39 months) and weight ranged from 5 kg to 62 kg (mean 21 kg). A total of 23 whole livers, 10 reduced-size grafts, 14 split liver grafts and 12 living donor liver transplants (LDLTs) were performed. Eight patients were referred with fulminant hepatic failure (FHF), all in Era 2. Of these, three patients were successfully transplanted. Of the 57 patients, 45 are alive and well with actuarial 1-year patient and graft survival of 85% and 84% and 5-year patient and graft survival of 78% and 74%, respectively. Sixteen (25.42%) biliary complications occurred in 15 of our 59 transplants. Seven patients developed significant vascular complications. Comparing Era 1 with Era 2, mean age at transplant decreased from 100.86 months to 64.73 months, mean weight from 25.2 kg to 16.9 kg, and type of graft utilised changed with a trend away from the use of whole livers and reduced-sized grafts to split livers and segment 2,3 LDLT grafts CONCLUSION: Initially limited by a shortage of donor organs, we aggressively explored optimal utilisation, splitting liver grafts from deceased donors as often as possible and establishing an LDLT programme. This increased access to donor livers allowed us to include patients with FHF and to perform retransplantation in recipients with early graft failure. It remains to offer liver transplantation to the entire paediatric community in SA, in conjunction with the only other established paediatric liver transplant unit, at Red Cross War Memorial Children's Hospital in Cape Town <![CDATA[<b>Paediatric pancreatic trauma: a review of the literature and results of a multicentre survey on patient management</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100038&lng=es&nrm=iso&tlng=es BACKGROUND: It is generally accepted that paediatric solid organ injury should be treated conservatively, unless there is haemodynamic instability unresponsive to resuscitation. When it comes to pancreatic trauma, there is much debate about appropriate management OBJECTIVES: To review the literature and determine how pancreatic trauma is managed in South African (SA) tertiary institutions and compares with international standards METHOD: A survey was emailed to 45 paediatric surgical consultants working in various paediatric surgical units in SA, Italy, England and Australia. The questionnaire comprised two scenarios of isolated pancreatic trauma (grade III), the main difference between them being the time interval between initial injury and presentation. In the first scenario, the patient presented 6 hours post injury whereas in the second scenario, the patient presented 6 days post initial injury. The survey enquired about diagnosis and subsequent work-up (including preferred imaging techniques), supportive management (including nutrition), the various options of definitive intervention and follow-up procedure RESULTS: There were 21 responders from four different countries. In the first scenario, 10 surgeons would operate, 8 would treat conservatively and 3 would perform an endoscopic retrograde cholangiopancreatogram (ERCP) and stent. In the second scenario, 4 surgeons would operate, 13 would treat conservatively and 4 would undertake ERCP with stent. There was no difference in management between the SA surgeons and their international counterparts CONCLUSION: Management of blunt pancreatic trauma in SA is consistent with that reported in the literature. There is still controversy regarding the optimal management of pancreatic injury involving ducts. No absolute algorithm can be used to treat these patients. All patients should be treated individually and managed with an approach and techniques that are feasible <![CDATA[<b>Acute liver failure and transplantation in children</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100039&lng=es&nrm=iso&tlng=es Acute liver failure (ALF) was relatively easy to recognise in the days before liver transplantation became available as rescue therapy, because the diagnosis was based on end-stage disease manifestations such as profound coagulopathy, jaundice, encephalopathy and cerebral oedema (in a patient with no history of chronic liver disease). These criteria no longer help us in an era in which we struggle to define which patients are going to progress to this end-stage picture in the time necessary for evaluation and listing for life-saving transplantation. Ideally, identifying which patients will recover spontaneously or with appropriate treatment would relieve the justifiable concern that some patients receive a transplant when, given time, they would have recovered. Currently, the data to guide us in avoiding death without transplantation and unnecessary transplantation remain elusive. <![CDATA[<b>Liver tumours in children: current surgical management and role of transplantation</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100040&lng=es&nrm=iso&tlng=es This article reviews the current surgical management of liver tumours in children in the light of improved chemotherapy, surgical techniques and outcomes from transplantation. It is a principle of management that complete removal of a tumour must be achieved for cure. Neoadjuvant chemotherapy may downstage advanced local disease to enable safe curative tumour resection. When this is not achievable, transplant is indicated. Conventional indications for transplant are unresectable stages 3 and 4 tumours confined to the liver. With the realisation that lifelong immunosuppressive therapy has considerable adverse consequences, there has been a recent trend towards extreme and 'acrobatic' liver resection to avoid transplantation, but still obtain a cure. The current literature is reviewed in the light of these trends and our own experience. <![CDATA[<b>Controversies in choledochal malformation</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100041&lng=es&nrm=iso&tlng=es Choledochal malformations (some of which are choledochal cysts) may be characterised as an abnormal dilatation of the biliary tract in the absence of acute obstruction. Most appear to be of congenital origin, probably related to distal bile duct stenosis, and almost 15% can now be detected antenatally. Excision and biliary reconstruction using a Roux loop as an open operation is still the standard to compare against, although laparoscopic reconstruction is increasingly reported. This article discusses recent advances in the understanding of choledochal malformation aetiology and classification, together with the role of newer modalites of surgical treatment such as laparoscopic excision and biliary reconstruction. Although these are definitely feasible, care should be taken before dispensing with standard open techniques that have minimal complications and proven long-term benefit. <![CDATA[<b>Role of laparoscopy during surgery at the porta hepatis</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100042&lng=es&nrm=iso&tlng=es Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety, with comparable outcomes to open surgery and with the advantages of minimal scarring and less pain. In this article, we describe the latest laparoscopic techniques used at Juntendo University Hospital in Japan, for treating conditions affecting the porta hepatis, focusing on biliary atresia and choledochal cysts. We also summarise our postoperative management protocols and discuss preliminary outcomes. <![CDATA[<b>The role of interventional radiology in complications after paediatric liver transplantation</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100043&lng=es&nrm=iso&tlng=es Liver transplantation has become an established treatment in both adults and children for end-stage liver disease, acute hepatic failure and certain liver tumours. There is a significant risk of complications after all forms of liver transplantation. The interventional radiologist plays a critical role in the diagnosis and treatment of these complications. The use of image-guided, minimally invasive procedures reduces the need for surgical revision or retransplantation and improves graft and patient survival rate. This article reviews some of the most common vascular and non-vascular complications after paediatric liver transplantation, and the interventional radiology techniques used to diagnose and treat them. <![CDATA[<b>Paediatric liver transplantation for children treated at public health facilities in South Africa: Time for change</b>]]> http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742014001100044&lng=es&nrm=iso&tlng=es Paediatric liver transplantation (PLT) is the only therapeutic option for many children with end-stage chronic liver disease or irreversible fulminant hepatic failure, and is routinely considered as a therapy by paediatric gastroenterologists and surgeons working in developed countries. In South Africa (SA), a PLT programme has been available at Red Cross War Memorial Children's Hospital in Cape Town since November 1991, and another has rapidly developed at the Wits Donald Gordon Medical Centre in Johannesburg over the past decade. However, for most children with progressive chronic liver disease who are reliant on the services provided at state facilities in SA, PLT is not an option because of a lack of resources in a mismanaged public health system. This article briefly outlines the services offered at Chris Hani Baragwanath Academic Hospital - which is typical of state facilities in SA - and proposes that resources be allocated to establish an innovative, nationally funded centre that would enable greater numbers of children access to a PLT programme.