Introduction: Sickle cell disease is the most common inherited disease in Brazil. Patients are known to suffer physical, emotional and social impairment and their quality of life may well be involved. Method: The quality of life of sickle cell disease patients treated in Hospital das Clínicas of the Universidade Federal de Goiás was evaluated. Sixty patients with ages ranging from 14 to 60 years old were interviewed. The WHOQOL-Bref (a quality of life validation instrument of the World Health Organization), and the ethnicalracial, and sociodemographic questionnaires were administered. A standard error of 5% (p-value ≤ 0.05) was considered acceptable. Results: The mean age of the participants was 27 years old, 53.3% of the patients were women, 71.7% were single and 51.7% had completed elementary school. The majority classified themselves as mulattos (46.7%) and the minority Blacks (11.7%). Only 6.7% considered themselves victims of racial discrimination because of their skin color but 33.3% considered themselves victims of discrimination due to sickle cell disease. The patients 48.3% reported an association between their disease and their skin color. The quality of life was considered bad by 6.7% and good by 70%. A total of 48.3% considered their lives to be satisfactory and 23.3% to be unsatisfactory. The scores obtained from the WHOQOL-Bref (from 0 to 100) were: 57.32 for physical, 66.03 for psychological, 69.86 for social and 52.76 for environmental domains. There were significant correlations of discrimination due to the disease with educational level and age with all the WHOQOL-Bref domains. Conclusion: Sickle cell disease significantly limits the quality of life of patients. Also, sickle cell disease, coinciding with the racial miscegenation, is losing its "black-related disease" character in Brazil.

No abstract

We propose to assess integrative seminar (IS) as a learning tool. In this descriptive quantitative study, 84 out of 107 (78%) first-year medical students from the Universidade Federal de Goiás responded to a self-administered semi-structured questionnaire. The item "Integration with the community" received the highest score (3.3-3.6), demonstrating that IS deepened the relationship between students and the community. The positive assessments of the items "Preparation of a written essay" (3.3-3.5), "IS as a learning resource" (3.2-3.5), "Oral presentation," and "Interpersonal relationships" (3.1-3.4 in both) suggest that teacher guidance and adequate time for preparation enable IS use as a teaching-learning strategy that stimulates communication development and interpersonal relationships. The low scores for the items "Tool Moodle" (2.6-2.7) and "Assessment of preparation and seminar presentation" (2.7-3.0) suggest that the evaluation process and feedback preparation by teachers did not meet students' expectations. IS was well accepted by students. The high score for "Integration with the community" demonstrates the importance of shared learning, because it facilitates problem recognition and feedback to the community. However, the low score for "Tool Moodle" points out that this learning instrument needs adjustments to virtual learning environments based on the community.

Aceruloplasminemia is a rare autosomal recessive disease in which a mutation leads to the absence or dysfunction of ceruloplasmin. Deficiency of this enzyme leads to the accumulation of iron in various organs; aceruloplasminemia is usually characterized by diabetes, retinal degeneration and neurological disorders. Diagnosis is suspected by the presence of elevated levels of ferritin, anemia, decreased serum copper and absence of ceruloplasmin in serum. Treatment of aceruloplasminemia is mainly based on the control of iron overload.

CONTEXT AND OBJECTIVE: Routine use of the script concordance test (SCT) is not common in Brazilian universities. This study aimed to analyze application of the SCT in the medical school of a Brazilian university. DESIGN AND SETTING: Quantitative, analytical and descriptive study in the medical school of a Brazilian university. METHODS: A total of 159/550 students participated. The test comprised ten clinical cases within internal medicine, with five items per case, rated on a five-point Likert scale. The test was scored in accordance with a marking key that had been validated by a reference panel. RESULTS:In the pre-clinical and clinical phases, the mean scores were 51.6% and 63.4% of the maximum possible scores, respectively. Comparison of the means of the responses among all the years showed that there were significant differences in 40% of the items. The panel marked all the possible answers in five items, while in one item, all the panelists marked a single answer. Cronbach's alpha was 0.64. The results indicated that the more senior students performed better. Construction of an SCT with discriminative questions was not easy. The low reliability index may have occurred due to: a) problems with the construction of the questions; b) limitations of the reference panel; and/or c) the scoring key. CONCLUSION:This instrument is very difficult to construct, apply and correct. These difficulties may make application of an SCT as an assessment method unfeasible in units with limited resources. RESUMO