Ocorrência de Múltiplas Neoplasias em Paciente Portador de Leucemia Mieloide Crônica: Relato de Caso

Introdução: A leucemia mieloide crônica e um distúrbio mieloproliferativo clonal com uma anormalidade citogenética específica, resultante da translocação entre os cromossomos 9 e 22 com consequente produção de uma proteína com atividade tirosina quinase alterada. Tratamentos históricos com drogas como bussulfan, hidroxiureia e interferon passaram a ser pouco utilizados devido ao surgimento dos inibidores de tirosina quinase, cujo principal representante e o mesilato de imatinibe. Esse fármaco é a terapia de primeira linha, sendo bem tolerado pelos pacientes e com baixo risco de eventos adversos severos. Entretanto, com cerca de dez anos de uso, ainda há preocupação com efeitos colaterais em longo prazo, tais como o desenvolvimento de segunda neoplasia. Objetivo: Descrever a ocurrencia de múltiplas neoplasias em um portador de leucemia mieloide crônica. Método: Relata-se o caso de um paciente com leucemia mieloide crônica há 13 anos, tendo utilizado hidroxiureia e interferon como terapias prévias e em uso de mesilato de imatinibe há nove anos. Resultados: Há dois anos, o paciente apresentou dois nódulos em coxa esquerda que foram totalmente ressecados. Diagnosticou-se lipossarcoma mixoide e o paciente foi submetido à radioterapia. A tomografia computadorizada do abdômen de controle aos seis meses detectou nódulo espiculado na gordura mesenquimal adjacente ao jejuno/íleo. Feita laparotomia exploradora e ressecção, o anatomopatológico demonstrou fibromatose desmoide. Conclusão: O portador de tumor maligno tem risco aumentado de desenvolver uma segunda neoplasia, que pode dessa forma ocorrer nos portadores de leucemia mieloide crônica. Essa associação pode estar relacionada aos fármacos usados no tratamento da mesma

Script concordance test in medical schools in Brazil: possibilities and limitations

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

Emicizumab prophylaxis in people with hemophilia A and inhibitors: a systematic review and meta-analysis

ABSTRACT BACKGROUND: Until recently, the treatment of people with hemophilia A and inhibitors (PwHAi) was based on the use of bypassing agents (BPA). However, the advent of emicizumab as prophylaxis has demonstrated promising results. OBJECTIVES: We aimed to compare the bleeding endpoints between PwHAi on BPA and those on emicizumab prophylaxis. DESIGN AND SETTING: Systematic review of interventions and meta-analysis conducted at the Universidade Federal de Goiás, Goiânia, Goiás, Brazil. METHODS: The CENTRAL, MEDLINE, Scopus, and LILACS databases were searched on February 21, 2023. Two authors conducted the literature search, publication selection, and data extraction. The selected publications evaluated the bleeding endpoints between PwHAi on emicizumab prophylaxis and those on BPA prophylaxis. The risk of bias was evaluated according to the Joanna Briggs Institute criteria. A meta-analysis was performed to determine the annualized bleeding rate (ABR) for treated bleeds. RESULTS: Five publications (56 PwHAi) were selected from the 543 retrieved records. Overall, bleeding endpoints were lower during emicizumab prophylaxis than during BPA prophylaxis. All the publications had at least one risk of bias. The only common parameter for the meta-analysis was the ABR for treated bleeds. During emicizumab prophylaxis, the ABR for treated bleeds was lower than during BPA prophylaxis (standard mean difference: −1.58; 95% confidence interval −2.50, −0.66, P = 0.0008; I2 = 68.4%, P = 0.0031). CONCLUSION: Emicizumab was superior to BPA in bleeding prophylaxis in PwHAi. However, both the small population size and potential risk of bias should be considered when evaluating these results. SYSTEMATIC REVIEW REGISTRATION: CRD42021278726, https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=278726

Aceruloplasminemia: a rare disease - diagnosis and treatment of two cases

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

Development of clinical reasoning in an undergraduate medical program at a Brazilian university

ABSTRACT CONTEXT AND OBJECTIVE The cognitive processes relating to the development of clinical reasoning are only partially understood, which explains the difficulties in teaching this skill in medical courses. This study aimed to understand how clinical reasoning develops among undergraduate medical students. DESIGN AND SETTING Quantitative and qualitative exploratory descriptive study conducted at the medical school of Universidade Federal de Goiás. METHODS The focus group technique was used among 40 students who participated in five focus groups, with eight students from each year, from the first to fifth year of the medical school program. The material was subjected to content analysis in categories, and was subsequently quantified and subjected to descriptive statistical analysis and chi-square test for inferential statistics. RESULTS The content of the students' statements was divided into two categories: clinical reasoning - in the preclinical phase, clinical reasoning was based on knowledge of basic medical science and in the clinical phase, there was a change to pattern recognition; knowledge of basic medical science - 80.6% of the students recognized its use, but they stated that they only used it in difficult cases. CONCLUSION In the preclinical phase, in a medical school with a traditional curriculum, clinical reasoning depends on the knowledge acquired from basic medical science, while in the clinical phase, it becomes based on pattern recognition