Self-identity discrepancy theory: Exploring returning South African Cuban-trained medical students sense of belonging

Background: The Nelson Mandela-Fidel Castro Collaboration programme (NMFCMC) between South Africa and Cuba was established in 1996. South African students, undergo five years of medical training in Cuba and finish the final eighteen months of training in South Africa. These students experience academic difficulties on their return. Methods: All twelve NMFCMC students enrolled at the University of KwaZulu-Natal in 2015 participated in this study. Data were elicited using focus group interviews, narrative interviews, found photovoice and the Collage Life Story Elicitation Technique. Results: Challenges faced by participants resulted in identity discrepancy, which in turn promoted unfavourable attitudes, affect, psychological and physical behaviours towards participants’ belongingness. Conclusion: Returning NMFCMC students experienced difficulties in assimilation due to identity discrepancies and frustrated sense of belonging. Focusing on reinforcing positive aspects of identity, and interpersonal relationships through moderating the tendency of local teachers and students to emphasise the “otherness” of the NMFCMC student is crucial

Editorial

Health funding  the writing on the wall - A personal viewAcute intermittent porphyria an underdiagnosed cause of abdominal painBottled dange

Classifying snakebite in South Africa: Validating a scoring system

Objective. To develop and validate a scoring system for managing snakebites in South Africa (SA). Methods. We studied all snakebite admissions to a regional hospital in KwaZulu-Natal, SA. The primary outcome was an active treatment intervention (ATI) defined as antivenom treatment or any surgical procedure. The development cohort consisted of 879 patients with snakebite who presented to the Ngwelezane Hospital Emergency Department from December 2008 to December 2013. Factors predictive of ATI and the optimal cut-off score for predicting an ATI were identified. These factors were then used to develop a standard scoring system. The score was then tested prospectively for accuracy in a new validation cohort consisting of 100 patients admitted for snakebite to our unit from 1 December 2014 to 31 March 2015. Accuracy of the score was determined. Results. Of 879 snakebite admissions, 146 in the development cohort and 40 of 100 in the development validation cohort reached the primary endpoint of an ATI. Six risk predictors for ATI were identified from the development cohort: age 7 hours (OR 4.63), white cell count >10 � 109/L (OR 3.15), platelets 1.2 (OR 2.25). Each risk predictor was assigned a score of 1; receiver operating characteristic curve analysis returned a value of >4 out of 6 as the optimal cut-off for prediction of an ATI (area under the curve 0.804; 95% confidence interval 0.758-0.84). Testing of the score on the validation cohort produced a specificity of 96.6% and a sensitivity of 22.5%. The positive predictive value and negative predictive value were 81.8% and 65.2%, respectively. Conclusion. Our results show that the identified score is a useful adjunct to clinical assessment in managing snakebite. Its value is greatest when used in those patients who fall in the mild to moderate clinical category. Until our severity score has been validated (or modified) for use across SA, we propose to name it the Zululand Severity Score; a true SA Severity Score may follow

An overview of the cutaneous porphyrias

This is an overview of the cutaneous porphyrias. It is a narrative review based on the published literature and my personal experience; it is not based on a formal systematic search of the literature. The cutaneous porphyrias are a diverse group of conditions due to inherited or acquired enzyme defects in the porphyrin–haem biosynthetic pathway. All the cutaneous porphyrias can have (either as a consequence of the porphyria or as part of the cause of the porphyria) involvement of other organs as well as the skin. The single commonest cutaneous porphyria in most parts of the world is acquired porphyria cutanea tarda, which is usually due to chronic liver disease and liver iron overload. The next most common cutaneous porphyria, erythropoietic protoporphyria, is an inherited disorder in which the accumulation of bile-excreted protoporphyrin can cause gallstones and, rarely, liver disease. Some of the porphyrias that cause blistering (usually bullae) and fragility (clinically and histologically identical to porphyria cutanea tarda) can also be associated with acute neurovisceral porphyria attacks, particularly variegate porphyria and hereditary coproporphyria. Management of porphyria cutanea tarda mainly consists of visible-light photoprotection measures while awaiting the effects of treating the underlying liver disease (if possible) and treatments to reduce serum iron and porphyrin levels. In erythropoietic protoporphyria, the underlying cause can be resolved only with a bone marrow transplant (which is rarely justifiable in this condition), so management consists particularly of visible-light photoprotection and, in some countries, narrowband ultraviolet B phototherapy. Afamelanotide is a promising and newly available treatment for erythropoietic protoporphyria and has been approved in Europe since 2014

Variegate porphyria in South Africa, 1688 - 1996 - new developments in an old disease

Variegate porphyria, an autosomal dominant inherited trait resulting in decreased activity of protoporphyrinogen oxidase, the penuttimate haem biosynthetic enzyme, is characterised clinically by photosensitive skin disease and a propensity to acute neurovisceral crises. The disease has an exceptionally high frequency in South Africa,owing to a founder effect. The specific mutation in the protoporphynnogen oxidase gene sequence which represents this founder gene has been identified. Genetic diagnosis is therefore now possible in families in whom the gene defect is known. However, the exact nature and degree of activity of the porphyria can only be determined by detailed quantitative biochemical analysis of excreted porphyrins. The relative contributions of the acute attack and the skin disease to the total disease burden of patients with variegate porphyria is not static, and in South Africa there have been significant changes over the past 25 years, with fewer patients presenting with acute attacks, leaving a greater proportion to present with skin disease or to remain asymptomatic with the diagnosis being made in the laboratory. The most common precipitating cause of the acute attack of VP is administration of porphyrinogenic drugs. Specific suppression of haem synthesis with intravenous haem arginate is the most useful treatment of a moderate or severe acute attack. Although cutaneous lesions are limited to the sun-exposed areas, management of the skin disease of VP remains inadequate

Key Terms and Definitions in Acute Porphyrias: Results of an International Delphi Consensus Led by the European Porphyria Network

Background: Acute porphyrias are a group of rare inherited disorders causing acute neurovisceral attacks. Many terms used frequently in the literature and in clinical practice are ambiguous, which can lead to confusion in the way patients are managed, studied, and reported in clinical studies. Agreed definitions are a necessary first step in developing management guidelines and will facilitate communication of results of future clinical research. Methods: The Delphi method was used to generate consensus on key terms and definitions in acute porphyria. The process started with a brainstorming phase offered to all members of the European Porphyria Network followed by 2 Delphi rounds among international experts in the field of porphyria (the Acute Porphyria Expert Panel). A consensus of 75% or more was defined as the agreement threshold. Results: 63 respondents from 26 countries participated in the brainstorming phase, leading to the choice of 9 terms and definitions. 34 experts were invited to take part in the Delphi rounds. 7 of the initial 9 terms and definitions which entered the first Delphi round achieved the threshold for agreement. Following a second Delphi round, all 9 definitions achieved agreement. Conclusion: Agreement on the definitions for 9 important terms describing acute porphyrias represents a significant step forward for the porphyria community. It will facilitate more accurate comparison of outcomes among porphyria centres and in clinical trials and provide a strong framework for developing evidence based clinical guidelines.Background: Acute porphyrias are a group of rare inherited disorders causing acute neurovisceral attacks. Many terms used frequently in the literature and in clinical practice are ambiguous, which can lead to confusion in the way patients are managed, studied, and reported in clinical studies. Agreed definitions are a necessary first step in developing management guidelines and will facilitate communication of results of future clinical research. Methods: The Delphi method was used to generate consensus on key terms and definitions in acute porphyria. The process started with a brainstorming phase offered to all members of the European Porphyria Network followed by 2 Delphi rounds among international experts in the field of porphyria (the Acute Porphyria Expert Panel). A consensus of 75% or more was defined as the agreement threshold. Results: 63 respondents from 26 countries participated in the brainstorming phase, leading to the choice of 9 terms and definitions. 34 experts were invited to take part in the Delphi rounds. 7 of the initial 9 terms and definitions which entered the first Delphi round achieved the threshold for agreement. Following a second Delphi round, all 9 definitions achieved agreement. Conclusion: Agreement on the definitions for 9 important terms describing acute porphyrias represents a significant step forward for the porphyria community. It will facilitate more accurate comparison of outcomes among porphyria centres and in clinical trials and provide a strong framework for developing evidence based clinical guidelines