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1.
S Afr Fam Pract (2004) ; 66(1): e1-e6, 2024 Apr 19.
Artigo em Inglês | MEDLINE | ID: mdl-38708747

RESUMO

BACKGROUND:  The World Health Organization advocates the early, appropriate provision of palliative care (PC) to patients throughout the life course. Patient consultations to the emergency department (ED) have been recognised as opportunities to initiate or optimise their PC needs. This study aimed to assess the knowledge of and attitudes towards PC among doctors at emergency physician staffed EDs in KwaZulu-Natal, South Africa. METHODS:  A cross-sectional survey was conducted between November 2021 and February 2022 for doctors employed out at emergency physician staffed EDs in KwaZulu-Natal, South Africa, using the validated Palliative Care Attitude and Knowledge questionnaire. The variables assessed were the self-rated and basic knowledge and attitudes towards core domains of PC. Ordinal data were compared using the t-test or ANOVA as appropriate, using MedCalc® Statistical Software version 22.009. RESULTS:  Of the 39 participants, the scores for the knowledge questions showed that 15.3% participants had good knowledge, 53.8% had fair knowledge and 30.7% had poor knowledge. Participants had either favourable (58.8%) or an uncertain (41.0%) attitude towards PC. No correlation was seen between the knowledge and attitudes scores (Spearman's rho = 0.13, 95% CI -0.19 to 0.43, p = 0.43). CONCLUSION:  There appears to be a deficit in knowledge of PC among doctors in the ED and a need for in-service training in PC for emergency care physicians.Contribution: This study provides new knowledge around PC practices at EDs in KwaZulu-Natal, South Africa.


Assuntos
Atitude do Pessoal de Saúde , Serviço Hospitalar de Emergência , Conhecimentos, Atitudes e Prática em Saúde , Cuidados Paliativos , Médicos , Humanos , África do Sul , Estudos Transversais , Serviço Hospitalar de Emergência/estatística & dados numéricos , Feminino , Masculino , Adulto , Inquéritos e Questionários , Médicos/psicologia , Pessoa de Meia-Idade
2.
S Afr Fam Pract (2004) ; 63(1): e1-e5, 2021 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-33567838

RESUMO

BACKGROUND: Dermatological diseases are amongst the commonest reasons for consultation at primary care level. Yet, dermatology teaching in medical and nursing curricula is inconsistent and often insufficient to enable medical and nursing professionals to manage these conditions effectively. METHODS: We tested the knowledge of 100 doctors and 195 nurses who attended dermatology training sessions held in three health districts in the province of KwaZulu-Natal (KZN), South Africa, by using a quasi-experimental uncontrolled before-and-after study design. At the start of the session, participants were exposed to 15 slides representing common dermatological conditions; this was followed by a test. The participants then attended a series of short lectures followed by the same test. Pre- and post-intervention test scores were compared, and the results were analysed by professional status, health district and type of facility. RESULTS: The mean (standard deviation [SD]) pre-intervention test score was 40.6% (20.5%). Doctors scored significantly higher than nurses (p 0.0001). There were significant differences in performance by district (p 0.001) and type of facility (p 0.001). The mean (SD) post-intervention score improved to 68.7% (22.5%). CONCLUSION: Doctors and nurses working in the primary care sector appear to be insufficiently trained in the management of common dermatological conditions. A short period of in-service training resulted in an immediate, significant improvement in knowledge, although we did not study long-term retention beyond this. We recommend improved prequalification training in dermatology in medical and nursing schools and an expansion of continuing professional development as well as in-service training opportunities for primary care practitioners.


Assuntos
Dermatologia , Médicos , Pessoal de Saúde , Humanos , Atenção Primária à Saúde , África do Sul
3.
Artigo em Inglês | MEDLINE | ID: mdl-32998198

RESUMO

(1) Oesophageal squamous cell carcinoma is common in Africa and has a male preponderance. The gender-based differences in clinical presentation and risk factor exposure are poorly studied in the African context. Our aim was to compare males and females with this disease. We analyzed the differences in clinical features and risk factor exposure between males and females with oesophageal cancer. (2) Data from patients presenting to a tertiary hospital in South Africa with oesophageal squamous cell carcinoma were analyzed. Data collected included patient demographics, clinical presentation, pathology and risk factor exposure. (3) Three hundred and sixty three patients were included in the study. The male to female ratio was 1.4:1. The mean age was 66 years for females and 61 years for males (p < 0.0001). A significantly larger percentage of males were underweight compared to females (60% vs. 32%, p < 0.001). There were no differences between the genders with regards to performance status, dysphagia grade and duration and tumor length, location and degree of differentiation. There were significant differences between risk factor exposure between the two genders. Smoking and alcohol consumption was an association in more than 70% of males but in less than 10% of females There was no difference survival. (4) Female patients with oesophageal squamous cell carcinoma (OSCC) are older and have a higher body mass index (BMI) than their male counterparts. Traditionally purported risk factors of smoking and alcohol consumption are infrequent associations with OSCC in female patients and other environmental risk factors may be more relevant in this gender.


Assuntos
Neoplasias Esofágicas/epidemiologia , Carcinoma de Células Escamosas do Esôfago/epidemiologia , Idoso , Carcinoma de Células Escamosas/epidemiologia , Feminino , Humanos , Masculino , Fatores de Risco , Caracteres Sexuais , Fatores Sexuais , África do Sul/epidemiologia , Centros de Atenção Terciária
4.
Clin Chem ; 65(6): 737-738, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31138549

Assuntos
Exantema , Humanos
5.
South Afr J HIV Med ; 20(1): 847, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31061722

RESUMO

BACKGROUND: Preconception antiretroviral therapy (PCART) followed by sustained viral suppression is effective in preventing mother-to-child transmission of HIV. The rates of persistent and transient viraemia in such patients have not been prospectively assessed in South Africa. OBJECTIVES: We determined the prevalence of transient and persistent viraemia in HIV-positive women entering antenatal care on PCART and studied variables associated with viraemia. METHODS: We performed a prospective cross-sectional observational study of HIV-positive pregnant women presenting to a primary healthcare facility in KwaZulu-Natal. All had received at least 6 months of first-line PCART. Viral load (VL) was measured, patients were interviewed, adherence estimated using a visual analogue scale and adherence counselling provided. Viral load was repeated after 4 weeks where baseline VL exceeded 50 copies/mL. RESULTS: We enrolled 82 participants. Of them, 59 (72%) pregnancies were unplanned. Fifteen participants (18.3%) were viraemic at presentation with VL > 50 copies/mL. Of these, seven (8.5%) had viral suppression (VL < 50 copies/mL), and eight remained viraemic at the second visit. Adherence correlated significantly with viraemia at baseline. Level of knowledge correlated with adherence but not with lack of viral suppression at baseline. Socio-economic indicators did not correlate with viraemia. No instances of vertical transmission were observed at birth. CONCLUSIONS: Approximately 20% of women receiving PCART may demonstrate viraemia. Half of these may be transient. Poor adherence is associated with viraemia, and efforts to encourage and monitor adherence are essential. The rate of unplanned pregnancies is high, and antiretroviral therapy programmes should focus on family planning needs of women in the reproductive age group to prevent viral non-suppression prior to pregnancy. KEYWORDS: Preconception Antiretroviral Therapy; HIV; Viraemia; Antenatal Care; Adherence.

6.
SA J Radiol ; 22(2): 1377, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-31754519

RESUMO

BACKGROUND: The use of multi-parametric magnetic resonance imaging (MRI) in the evaluation of breast tuberculosis (BTB). OBJECTIVES: To evaluate the value of diffusion-weighted imaging (DWI), T2-weighted (T2W) and dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in differentiating breast cancer (BCA) from BTB. METHOD: We retrospectively studied images of 17 patients with BCA who had undergone pre-operative MRI and 6 patients with pathologically proven BTB who underwent DCE-MRI during January 2014 to January 2015. RESULTS: All patients were female, with the age range of BTB patients being 23-43 years and the BCA patients being 31-74 years. Breast cancer patients had a statistically significant lower mean apparent diffusion coefficient (ADC) value (1072.10 ± 365.14), compared to the BTB group (1690.77 ± 624.05, p = 0.006). The mean T2-weighted signal intensity (T2SI) was lower for the BCA group (521.56 ± 233.73) than the BTB group (787.74 ± 196.04, p = 0.020). An ADC mean cut-off value of 1558.79 yielded 66% sensitivity and 94% specificity, whilst the T2SI cut-off value of 790.20 yielded 83% sensitivity and 83% specificity for differentiating between BTB and BCA. The homogeneous internal enhancement for focal mass was seen in BCA patients only. CONCLUSION: Multi-parametric MRI incorporating the DWI, T2W and DCE-MRI may be a useful tool to differentiate BCA from BTB.

7.
SA j. radiol ; 22(2): 1-7, 2018. ilus
Artigo em Inglês | AIM (África) | ID: biblio-1271349

RESUMO

Background: The use of multi-parametric magnetic resonance imaging (MRI) in the evaluation of breast tuberculosis (BTB). Objectives: To evaluate the value of diffusion-weighted imaging (DWI), T2-weighted (T2W) and dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in differentiating breast cancer (BCA) from BTB. Method: We retrospectively studied images of 17 patients with BCA who had undergone pre-operative MRI and 6 patients with pathologically proven BTB who underwent DCE-MRI during January 2014 to January 2015. Results: All patients were female, with the age range of BTB patients being 23­43 years and the BCA patients being 31­74 years. Breast cancer patients had a statistically significant lower mean apparent diffusion coefficient (ADC) value (1072.10 ± 365.14), compared to the BTB group (1690.77 ± 624.05, p = 0.006). The mean T2-weighted signal intensity (T2SI) was lower for the BCA group (521.56 ± 233.73) than the BTB group (787.74 ± 196.04, p = 0.020). An ADC mean cut-off value of 1558.79 yielded 66% sensitivity and 94% specificity, whilst the T2SI cut-off value of 790.20 yielded 83% sensitivity and 83% specificity for differentiating between BTB and BCA. The homogeneous internal enhancement for focal mass was seen in BCA patients only. Conclusion: Multi-parametric MRI incorporating the DWI, T2W and DCE-MRI may be a useful tool to differentiate BCA from BTB


Assuntos
Neoplasias da Mama , Imagem de Difusão por Ressonância Magnética , África do Sul , Tuberculose
8.
Emerg Med J ; 33(7): 477-81, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27068867

RESUMO

BACKGROUND: Snakebites that have cytotoxic venom can cause significant soft tissue swelling. Assessing the site and degree of swelling using ultrasound as a non-invasive technique would be an important tool for instituting appropriate treatment. METHODS: Forty-two patients who presented to a referral hospital in South Africa with cytotoxic swelling of the limbs from snakebite were assessed using ultrasound. The envenomed limb of each patient was scanned at the point of maximal swelling and compared with the unaffected limb at the same site. Data were presented as an expansion coefficient defined as the ratio of the thickness of tissue structure (subcutaneous tissue or muscle compartment) in the envenomed limb to that in the unaffected limb. A p value of 0.05 was regarded as significant, and 95% CIs were expressed throughout. RESULTS: The majority of bites were in the upper limb (27/42). Twenty-five patients were children less than 12 years. Tissue expansion was noted in both the subcutaneous and muscle compartments of the envenomed limbs. The site of swelling was predominantly in the subcutaneous tissues, while swelling in muscle compartment was limited (the mean expansion coefficient for subcutaneous tissues was 2.0 (CI 1.7 to 2.3) vs 1.06 (CI 1.0 to 1.1), respectively). The difference between the groups was significant (p<001). One case, confirmed as compartment syndrome, showed marked swelling in the muscle group. CONCLUSIONS: Basic ultrasound techniques may be used to identify the site and degree of tissue swelling from cytotoxic envenomation. It is a non-invasive, painless procedure that can assist the clinician to assess the injured limb and may also be of benefit to monitor the progression of swelling.


Assuntos
Extremidade Inferior/diagnóstico por imagem , Mordeduras de Serpentes/diagnóstico por imagem , Ultrassonografia/métodos , Extremidade Superior/diagnóstico por imagem , Adolescente , Adulto , Animais , Criança , Edema/induzido quimicamente , Edema/diagnóstico por imagem , Humanos , Incidência , Escala de Gravidade do Ferimento , Mordeduras de Serpentes/epidemiologia , Serpentes , África do Sul/epidemiologia
9.
Wilderness Environ Med ; 27(1): 53-61, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26948554

RESUMO

OBJECTIVE: We propose a formula as a means to estimate the number and incidence of snakebites treated per annum in KwaZulu Natal (KZN), South Africa. METHODS: Using an unvalidated formula that includes an antivenom ratio, we crudely estimated the total number of snakebite presentations in KZN. Using antivenom supply data from the central pharmacy, we stratified a sample of 6 hospitals that were surveyed to establish an antivenom ratio, that is, the total number of patients receiving antivenom to the total number of snakebite presentations at hospitals. The antivenom ratio and the average number of antivenom vials for treated snakebites were incorporated into a formula to crudely estimate the number of snakebite presentations. This was then applied to all public hospitals and districts in the region. RESULTS: Seventy-eight percent of public hospitals were included. The mean antivenom ratio derived from the sample hospitals indicated that 12% (95% CI, 10-14%) of snakebite presentations received antivenom. We estimated an annual total of 1680 (95% CI, 1193-2357) snakebite presentations to hospitals. Two thirds of cases (1109 of 1680) were in the low-lying subtropical coastal region. Few cases were in the higher, cooler regions of KZN (87 of 1680) or the metropolitan city of Durban (93 of 1680). The overall incidence for KZN was 16/100,000. The estimated cost of snakebite in KZN was between $1,156,930 and $2,827,848. CONCLUSIONS: We propose an alternative method to estimate the annual number of snakebite presentations to hospitals.


Assuntos
Antivenenos/uso terapêutico , Hospitais Públicos , Mordeduras de Serpentes/epidemiologia , Hospitais Públicos/estatística & dados numéricos , Humanos , Incidência , Mordeduras de Serpentes/etiologia , África do Sul/epidemiologia
10.
Int J Dermatol ; 55(8): 875-81, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26547604

RESUMO

BACKGROUND: Alopecia has been shown to have a significant impact on quality of life (QoL), particularly in women. However, there are no data for African populations. This study was conducted to pilot an original questionnaire and a model-based methodology to measure QoL and its determinants in a sample of South African Black women of African ancestry with alopecia. METHODS: Fifty participants aged 21-79 years were randomly chosen from patients presenting to dermatologists with alopecia. We used an original questionnaire consisting of 24 items grouped into those assessing the respective impacts of subjective symptoms, objective signs, and relationship issues, measured on a four-level scale. These were then combined using component-based structural equation modeling to return a QoL index (QLI) and to rank the factors contributing to this. RESULTS: On a scale ranging from 0 (high QoL) to 100 (severely decreased QoL), we found a mean QLI of 67.7. The negative impact of alopecia on QoL was higher in younger patients than older patients. The factors with the highest impact were those relating to the subjective experience of alopecia and self-image (56.3%), followed by those relevant to relationships and interaction with other people (34.8%). The presence of objective symptoms and signs such as pruritus was of minor importance (8.9%). CONCLUSIONS: Although not a life-threatening condition, alopecia may seriously impair QoL, particularly by inducing anxiety and reducing self-esteem among African women. Healthcare practitioners should be mindful of this and intervene appropriately to mitigate these effects.


Assuntos
Alopecia/etnologia , Alopecia/psicologia , Qualidade de Vida , Autoimagem , Inquéritos e Questionários , Adulto , Idoso , Alopecia/diagnóstico , Ansiedade/etnologia , Ansiedade/fisiopatologia , Estudos Transversais , Feminino , Humanos , Relações Interpessoais , Pessoa de Meia-Idade , Projetos Piloto , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , África do Sul , População Urbana , Adulto Jovem
11.
S Afr Med J ; 105(10): 866-9, 2015 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-26428595

RESUMO

BACKGROUND: Breast tuberculosis (BTB) is uncommon, but not rare. Knowledge of the ways in which it can present can prevent unnecessary invasive procedures and delay in diagnosis. OBJECTIVES: To describe the clinical and radiological findings in patients with BTB, including evaluation of current treatment methods. METHODS: We retrospectively analysed 65 patients diagnosed with BTB at Addington and King Edward VIII hospitals, Durban, South Africa, between 2000 and 2013. Demographic, clinical and radiological findings and treatment outcomes were noted. RESULTS: A total of 11,092 patients underwent breast investigations between 2009 and 2013, with a prevalence of BTB for the period of 0.3% (30 patients). Of the 65 patients diagnosed between 2000 and 2013, 64 were female (98.5%) and one was male (1.5%). The age range was 23-69 years (mean 38.5). The most common mammographic pattern was density (39.4%) and the least common a mass (6.1%). Isolated axillary lymphadenitis was found in 12.1%. Abscess was the commonest ultrasound pattern (39.0%). Of the 47 patients with a known history of pulmonary tuberculosis (TB), 68.1% (n=32) did not have radiological evidence of previous or concurrent pulmonary TB, nor was there evidence of TB elsewhere. Of 47 patients with known HIV status, 34 were HIV-positive. Fine-needle aspiration cytology had sensitivity of only 28% compared with 94% for histology. Of those treated, 72.7% obtained full resolution following 9 months of TB treatment; 25.0% did not complete treatment, and 2.3% (n=1) died while on treatment. Follow-up data on relapse rates after treatment completion and disease resolution are scanty. CONCLUSION: Understanding and being aware of the various presentations of BTB make it possible to treat most patients successfully.

12.
Int J Dermatol ; 54(3): 279-85, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24738608

RESUMO

BACKGROUND: Precise knowledge of the prevalence and spectrum of skin diseases in a population allows for effective planning for provision of dermatology services and distribution of resources. There are no published data on the epidemiology of skin disorders in Durban, KwaZulu-Natal. OBJECTIVE: We investigated the prevalence of skin diseases in black African patients attending a predominantly black private healthcare facility and profiled the patients. METHODS: Clinical charts of all black African patients seen between January 2003 and December 2010 in a private practice in Durban were reviewed. The diseases seen were described and the prevalence calculated. RESULTS: A total of 6664 patient charts were reviewed. The five most common conditions were acne, eczemas, dyschromias, infections, and hair disorders. These data agree with reports from other parts of the world. LIMITATIONS: Selection bias was presented by a single private practice, thus data may not be fully representative of our population. CONCLUSION: Acne, eczemas, dyschromias, infections, and hair disorders are, in that order, the five most common disorders encountered.


Assuntos
População Negra/estatística & dados numéricos , Dermatopatias/etnologia , Acne Vulgar/etnologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Eczema/etnologia , Feminino , Doenças do Cabelo/etnologia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Transtornos da Pigmentação/etnologia , Prevalência , Dermatopatias Infecciosas/etnologia , África do Sul , Adulto Jovem
13.
BMC Med Educ ; 14: 249, 2014 Nov 28.
Artigo em Inglês | MEDLINE | ID: mdl-25431359

RESUMO

BACKGROUND: Written assessments fall into two classes: constructed-response or open-ended questions, such as the essay and a number of variants of the short-answer question, and selected-response or closed-ended questions; typically in the form of multiple-choice. It is widely believed that constructed response written questions test higher order cognitive processes in a manner that multiple-choice questions cannot, and consequently have higher validity. DISCUSSION: An extensive review of the literature suggests that in summative assessment neither premise is evidence-based. Well-structured open-ended and multiple-choice questions appear equivalent in their ability to assess higher cognitive functions, and performance in multiple-choice assessments may correlate more highly than the open-ended format with competence demonstrated in clinical practice following graduation. Studies of construct validity suggest that both formats measure essentially the same dimension, at least in mathematics, the physical sciences, biology and medicine. The persistence of the open-ended format in summative assessment may be due to the intuitive appeal of the belief that synthesising an answer to an open-ended question must be both more cognitively taxing and similar to actual experience than is selecting a correct response. I suggest that cognitive-constructivist learning theory would predict that a well-constructed context-rich multiple-choice item represents a complex problem-solving exercise which activates a sequence of cognitive processes which closely parallel those required in clinical practice, hence explaining the high validity of the multiple-choice format. SUMMARY: The evidence does not support the proposition that the open-ended assessment format is superior to the multiple-choice format, at least in exit-level summative assessment, in terms of either its ability to test higher-order cognitive functioning or its validity. This is explicable using a theory of mental models, which might predict that the multiple-choice format will have higher validity, a statement for which some empiric support exists. Given the superior reliability and cost-effectiveness of the multiple-choice format consideration should be given to phasing out open-ended format questions in summative assessment. Whether the same applies to non-exit-level assessment and formative assessment is a question which remains to be answered; particularly in terms of the educational effect of testing, an area which deserves intensive study.


Assuntos
Educação de Graduação em Medicina/métodos , Avaliação Educacional/métodos , Medicina Interna/educação , Redação , Competência Clínica , Medicina Baseada em Evidências , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Inquéritos e Questionários
14.
J Cosmet Dermatol ; 13(3): 236-41, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25196692

RESUMO

BACKGROUND: Skin-lightening products are commonly used by black communities in South Africa and worldwide. This practice has deep historical and cultural roots and is associated with adverse cutaneous effects. METHODS: A cross-sectional survey of 579 African and Indian women aged 18-70 years was conducted in two large public hospitals in Durban, South Africa. RESULTS: There were 292 Africans and 287 Indians included in the survey sample. Of these 32.3% had used skin-lightening products (60% of Africans and 40% of Indians). Most of those who had used skin lighteners (85 of Africans and 76% of Indians) claimed awareness of the adverse effects of the products, although this did not appear to inform knowledge of the product, how it was used, nor the decision to use the product. Most users (90%) expressed satisfaction with results achieved but 32% reported adverse events. CONCLUSION: Skin-lightening products are used by a third of African and Indian women in South Africa Cultural and historical perceptions equating a fairer skin with social advantage are pervasive and strongly reinforced by the media. There is a poor understanding of the risks associated with the use of these products. Public education campaigns are required to teach consumers about these risks and the importance of concomitant use of sunscreens with these products.


Assuntos
População Negra/psicologia , Cultura , Conhecimentos, Atitudes e Prática em Saúde , Percepção , Preparações Clareadoras de Pele , Adolescente , Adulto , Idoso , Comportamento do Consumidor , Estudos Transversais , Escolaridade , Feminino , Humanos , Pessoa de Meia-Idade , Medição de Risco , Preparações Clareadoras de Pele/efeitos adversos , África do Sul , População Branca/psicologia , Adulto Jovem
17.
S Afr Med J ; 102(6): 422-6, 2012 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-22668925

RESUMO

The porphyrias are a group of disorders resulting from defective haem biosynthesis. One form, variegate porphyria, is common in South Africa as a result of a founder effect. Over the past 50 years, the University of Cape Town Faculty of Health Sciences has built and maintained an international reputation for excellence in the field of porphyria. The porphyria group is respected for its research and for its accumulated experience in the management of these disorders. Equally important has been the comprehensive and holistic care offered to patients with porphyria, and to their families.


Assuntos
Porfirias/história , Universidades , Flavoproteínas/genética , História do Século XX , História do Século XXI , Hospitais Universitários , Humanos , Proteínas Mitocondriais/genética , Porfiria Variegada/genética , Porfirias/diagnóstico , Porfirias/terapia , Protoporfirinogênio Oxidase/genética , África do Sul
18.
J Clin Pathol ; 65(3): 200-5, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22049218

RESUMO

It has been suggested that King George III of Great Britain suffered from the haem biosynthetic disorder, variegate porphyria. This diagnosis is pervasive throughout the scientific and popular literature, and is often referred to as the 'Royal Malady.' The authors believe it inappropriate to view the case for porphyria purely in terms of symptoms, as has generally been the case in his presumptive acute porphyria diagnosis. Accordingly, this review provides a current description of the natural history and clinical presentation of the porphyrias, against which we measure the case for porphyria in George III and his relatives. The authors have critically assessed the prevalence of porphyria in a population, the expected patterns and frequency of inheritance, its penetrance and its expected natural history in affected individuals, and conclude that neither George nor his relatives had porphyria, based on four principal reasons. First, the rarity of the disease mandates a very low prior probability, and therefore implies a vanishingly low positive predictive value for any diagnostic indicator of low specificity, such as a historical reading of the symptoms. Second, penetrance of this autosomal dominant disorder is approximately 40%, and one may expect to have identified characteristic clinical features of porphyria in a large number of descendants without difficulty. Third, the symptoms of both George III and his relatives are highly atypical for porphyria and are more appropriately explained by other much commoner conditions. Finally, the natural history of the illnesses reported in this family is as atypical for variegate porphyria as are their symptoms.


Assuntos
Pessoas Famosas , Porfiria Variegada/diagnóstico , Progressão da Doença , Predisposição Genética para Doença , Hereditariedade , História do Século XIX , Humanos , Linhagem , Penetrância , Fenótipo , Porfiria Variegada/complicações , Porfiria Variegada/epidemiologia , Porfiria Variegada/genética , Porfiria Variegada/história , Prevalência
19.
J Gastroenterol Hepatol ; 27(2): 385-9, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21793904

RESUMO

BACKGROUND AND AIM: The thiopurines azathioprine and 6-mercaptopurine are effective in the management of patients with inflammatory bowel disease (IBD) in whom aminosalicylates, antibiotics and corticosteroids have failed to induce or maintain remission. Long-term use of these agents has been linked to a greatly increased risk of non-melanoma skin cancer and lymphatic cancer in organ transplant recipients. There is some evidence to suggest that IBD patients receiving thiopurines might be at increased risk of cancer. Our aim was to determine the incidence of cancer in a cohort of patients with IBD managed in our clinic, and to relate this to thiopurine exposure. METHODS: We conducted a retrospective study based on the clinical and pathology records of patients attending a specialist IBD clinic at Groote Schuur Hospital, Cape Town, South Africa between 1960 and 2007. RESULTS: We analyzed the records of 1084 patients. A total of 123 subjects (11.5%) had received thiopurine therapy. Cancer was identified in 51 patients (4.7%), including colorectal cancer (15 patients), melanoma (two patients), non-melanoma skin cancer (seven patients) and non-Hodgkin's lymphoma (five patients). A diagnosis of non-melanoma skin cancer was significantly associated with thiopurine exposure (odds ratio 5.0, 95% confidence interval 1.1-22.8). Six of seven non-melanoma skin cancers occurred in Caucasian patients, with a highly significant association with thiopurine use (odds ratio 12.4, 95% confidence interval 2.3-67.4). CONCLUSIONS: Patients with IBD who receive thiopurines are at increased risk of non-melanoma skin cancer. The risk is highest in Caucasian patients, and is negligible in other groups.


Assuntos
Anti-Inflamatórios/efeitos adversos , Azatioprina/efeitos adversos , Fármacos Gastrointestinais/efeitos adversos , Doenças Inflamatórias Intestinais/tratamento farmacológico , Mercaptopurina/efeitos adversos , Neoplasias Cutâneas/induzido quimicamente , Adulto , Feminino , Humanos , Incidência , Doenças Inflamatórias Intestinais/etnologia , Masculino , Pessoa de Meia-Idade , Razão de Chances , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Neoplasias Cutâneas/etnologia , Pigmentação da Pele , África do Sul/epidemiologia , Luz Solar/efeitos adversos , Fatores de Tempo , População Branca/estatística & dados numéricos , Adulto Jovem
20.
Case Rep Med ; 2011: 398571, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21966293

RESUMO

A 38-year-old HIV-positive female, recently started on antiretroviral therapy, presented in extremis. She had features suggestive of an HIV-associated cardiomyopathy complicated by the following problems: a four-day-old stroke, extensive deep venous thrombosis, and massive pulmonary embolism. She received intravenous streptokinase with rapid improvement, both haemodynamically and, unexpectedly, neurologically. Our case illustrates that a positive outcome is potentially possible where the two conditions coincide.

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